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INS Dictionary of Neuropsychology
and Clinical Neurosciences
INS Dictionary of
Neuropsychology and
Clinical Neurosciences
Second Edition

Edited by

David W. Loring, Ph.D.

Emory University

Major Contributors and Content Advisors

Stephen C. Bowden, Ph.D. Steven J. Loring, Ph.D.

University of Melbourne New Mexico State University

Felicia C. Goldstein, Ph.D. Susan Marino, Ph.D.

Emory University University of Minnesota

Chadwick M. Hales M.D., Ph.D. Kimford J. Meador, M.D.

Emory University Stanford University

Glenn J. Larrabee, Ph.D. Stephen E. Nadeau, M.D.

Private Practice University of Florida

Gregory P. Lee, Ph.D. Andrew C. Papanicolaou, Ph.D.

Georgia Regents University University of Tennessee

1
1
Oxford University Press is a department of the
University of Oxford. It furthers the University’s objective
of excellence in research, scholarship, and education
by publishing worldwide.
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in the UK and certain other countries.
Published in the United States of America by
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© 2015 by David W. Loring and International Neuropsychological Society

All rights reserved. No part of this publication may be reproduced,

stored in a retrieval system, or transmitted, in any form or by any means,
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Library of Congress Cataloging-in-Publication Data
INS dictionary of neuropsychology.
INS dictionary of neuropsychology and clinical neurosciences / edited by David W. Loring, Ph.D.,
Emory University ; major contributors and content advisors, Stephen C. Bowden, Ph.D.,
University of Melbourne [and nine others].—Second edition.
pages cm
Previous edition: INS dictionary of neuropsychology. New York : Oxford University Press, 1999.
ISBN 978-0-19-536645-7
1. Neuropsychology—Dictionaries. I. Loring, David W., editor. II. Bowden, Stephen,
1955- III. Title. IV. Title: Dictionary of neuropsychology and clinical neurosciences. V. Title:
International Neuropsychological Society dictionary of neuropsychology and clinical neurosciences.
QP360.I55 2015
612.8—dc23   2014042334

9 8 7 6 5 4 3 2 1
Printed in the United States of America
on acid-free paper
Foreword

The fact that the International Neuropsy- neurological diseases and disorders was not
chological Society (INS) has a dictionary at that same level in 1967.
that requires an update and therefore a With these modest beginnings, the field
second edition is testament to the growth rapidly advanced, but the modern field of
and importance of neuropsychology and neuropsychology—with its expanding meth-
the role that INS has played in the devel- ods, new terms, and research findings—needs
opment of this discipline. It also demon- a common base and a common language.
strates the changing landscape and breadth Elizabeth Warrington, the 36th INS Presi-
of the field. INS’s history and the emergence dent, highlighted this need when reviewing
of neuropsychology as a discipline can help the history of neuropsychological assess-
put into context the importance of this ment (Cipolotti & Warrington, 1995). In
second edition. INS began as an organiza- describing early 20th-century interests in
tion that focused on standardizing behavioral neurological syndromes like aphasia, alexia,
methods and research to make inferences and agraphia, acalulia, visual agnosia, and
about brain integrity and function. Remark- amnesia, Warrington concluded that “the
ably, neuropsychology began in an era when clinical and descriptive methods” that began
there were no noninvasive methods to di- the field “provided a poor standard of de-
rectly observe the living brain. Now, there scription of the cognitive impairments in
are enormous technological advances that these syndromes. They were . . . little more
have been applied to the study of the brain, than the bald statement of the clinical
which are integrated with neuropsycholog- opinion of the investigator” (Shallice, 1988).
ical methods that require the field to have There needed to be a science of human
a common lexicon, as outlined in the second brain–behavior relations along with rigorous
edition of the INS Dictionary of Neuropsy­ methods to evaluate these conditions and a
chology and Clinical Neurosciences. language to describe such findings. Begin-
INS was founded in 1967 and chartered ning in the 1940s, a broad spectrum of psy-
as an international and interdisciplinary chometric methods was applied to assessing
research organization to further the scien- abilities like intellectual and academic
tific study of brain–behavior relations, espe- functions, which in turn were applied to
cially in humans (Rourke & Murji, 2000). patients with neurological or psychiatric
It started with a handful of individuals and disorders. Initially, only the coarsest of as-
now has approximately 5,000 members. sociations could be made, but psychometric
However, in 1967, it was challenging to methods brought sound scientific princi-
study human brain–behavior relations. INS’s ples and rigor to evaluating aspects of cog-
first president, Karl H. Pribram, was a neu- nition, emotion, and behavior that could
rosurgeon who was best known for his work be replicated. Using a psychometric foun-
with nonhuman primates. Although animal dation, investigators began to standardize
work had the precision of experimentation, behavioral measures separate from the estab-
that same precision in assessing cognitive, lished intellectual and educational assess-
emotional, and behavioral changes in human ment techniques of the day. These novel

v
Foreword

methods were designed to specifically ad- the brain, all of which had major problems
dress explicit aspects of cognition, emotion, with morbidity and mortality. None of these
and behavior. The foundations for a neu- procedures gave a direct image of the brain,
ropsychological discipline were being laid only shadows and outlines. Neurosurgery,
down, and the marriage of “neuroscience” of course, provided direct observation of
with “psychology” had formed. Yet it was the brain but with obvious limitations. In
not until 1963 that the term “clinical neu- fact, because of the limitations of some of
ropsychology” would first be used in the these methods, understanding brain func-
published literature by Hallgrim Kløve, tion through behavior and neuropsycho-
who would become INS’s 18th president logical techniques became that much more
(Kløve, 1963). important. Neuropsychology could not use
During this formative era, the terminology these methods to advance the field.
of the field was dominated by clinical neu- Starting in 1973, this all began to change
rology and psychology. Neuropsychology’s with the first publication of what at that
lexicon was tied to these two disciplines time was referred to as “computerized trans-
as well. Because most neuropsychological verse axial scanning tomography,” which
measures were paper-and-pencil based at was later shortened to computed axial to-
that time, there was minimal technological mography (CAT) and then, eventually, just
sophistication that had to be mastered. In computed tomography (CT). For the first
these formative days, often the neurological time, a direct image of the brain could be
or neuropsychiatric condition being exam- obtained. However, in the early days of CT
ined typically had obvious impairments, scanning, the images of the brain were prim-
such as a hemiplegia or hemisensory deficit. itive, and there were only limited methods
This began to change with technological for any kind of image analysis. This all
advances such as electroencephalography changed with rapid improvements in CT
(EEG), which was used to establish side technology and the development of mag-
and location of potential seizure discharges netic resonance imaging (MRI) and other
and presumed localization of abnormal neuroimaging modalities. Now, there are
brain function. As introduced by Kløve in incredible and diverse methods for study-
1963, neuropsychology began to use other ing and quantifying brain structure and
technologies in addition to EEG and, there- function, and these have become integrated
fore, the necessary lexicon of the field had with neuropsychological methods for the
to expand. Being well-versed in traditional study of brain–behavior relations. As this
psychological assessment methods, statistics, Foreword is being written, a National Li-
and neurological nomenclature remained brary of Medicine search, using the terms
essential, but, with an expansion of the “cognition” and “MRI” where neuropsy-
disorders studied and the associated tech- chological methods are used to assess cog-
nologies applied—in conjunction with im- nition, indicates that since, 1984, there have
proved neuropsychological techniques—the been approximately 14,000 publications!
neuropsychologist needed an even broader Currently, essentially all neurological and
scope of terms. neuropsychiatric disorders are characterized
In 1967, when INS was founded, only by their influence on not just the medical
invasive methods like ventriculography, state of the patient, but also by their cog-
pneumoencephalography, direct puncture nitive, emotional, and neurobehavioral se-
arteriography, or radioisotope scintillation quelae using neuropsychological techniques.
scanning were available to directly study Incredible advances in examining neural

vi
 Foreword

connections and networks, so-called connec­ niques and information has only enhanced
tomics, and the human connectome project our conceptual models of brain–behavior
along with genomics, all have major impli- relations. During INS’s nearly 50 years of
cations for the field of neuropsychology. existence, these unprecedented advance-
As Julia Hannay, the 30th INS president, ments link the molecular basis of neural
pointed out in the Foreword of the first function to neural systems that subserve
edition of the INS Dictionary, as a relatively cognition and behavior. The “neuro” in
new society applying new methods to study neuropsychology embraces all of neurosci-
brain–behavior relations, terminology will ence and the addition of clinical neurosciences
change. As such, a dictionary for the field to the title of this dictionary reinforces the
becomes an essential part of its matura- interrelationship of neuropsychology within
tiona benchmark. New evidence supports the broader field of neuroscience. This update
or changes the way in which certain brain is critical to the advancement of neuropsy-
functions may be considered to influence chology, providing common terminology,
cognition, behavior, and emotion or how background, contemporary thinking, and
they should be assessed. An up-to-date a standard reference for the field.
common language becomes critical for the
field to advance. This is particularly true Erin D. Bigler, Ph.D.
for graduate and postgraduate education. President
In this second edition, there is a change in International Neuropsychological Society
December, 2014
the title. In addition to “neuropsychology,”
“clinical neurosciences” has been added.
Louis Costa, 6th INS president, foresaw
this important encirclement that all of References
neuroscience would bring to the field of Cipolotti, L., & Warrington, E. K. (1995). Neuro­
neuropsychology. In 1983, he wrote the first psychological assessment. Journal of Neurology,
article for the inaugural journal affiliated Neurosurgery, and Psychiatry, 58(6): 655–664.
with INS, titled “Clinical Neuropsychology: Costa, L. (1983). Clinical neuropsychology: A dis­
cipline in evolution. Journal of Clinical Neuro­
A Discipline in Evolution” (Costa, 1983).
psychology, 5(1): 1–11.
At that point, neuroscience as a general Delis, D. C. et al. (1998). Integrating clinical assess­
field was just beginning its major surge in ment with cognitive neuroscience: Con­struct
development. Another National Library of validation of the California Verbal Learning
Medicine search shows that, in 1983, the Test. Journal of Consulting and Clinical Psychology,
term “neuropsychology” showed up in only 56(1): 123–130.
Kløve, H. (1963). Clinical neuropsychology. The
23 publications; in 2014, there were more Medical Clinics of North America, 47: 1647–
than 1,200. During that same era, the term 1658.
“clinical neuroscience” went from two hits Rourke, B. P., & Murji, S. (2000). A history of
in 1983 to more than 5,000 in 2014. Linking the International Neuropsychological Society:
“cognitive” with “neuroscience” does not The early years (1965–1985). Journal of the
Interna­tional Neuropsychological Society, 6(4):
show up until 1988, in a publication by
491–509.
Edith Kaplan, 11th INS president (Delis Shallice, T. (1988). From neuropsychology to mental
et al., 1998). The exponential growth of neu- structure. New York, NY: Cambridge University
roscience following an explosion of tech- Press.

vii
Preface to the Second Edition

The interval between the publication of the biases, and thus there are many dictionary
first edition of the INS Dictionary of Neuro­ entries reflecting clinical research and design
psychology and this second edition, renamed because these topics will have increasing
the INS Dictionary of Neuropsychology and relevance for the development and imple-
Clinical Neurosciences, has witnessed phe- mentation of evidence-based medicine/
nomenal change in our understanding of evidence-based neuropsychology.
brain–behavior relationships in normal brain By and large, medications that are used
and clinical diseases, as well as in the avail- to treat the various diseases frequently seen
ability of information from a variety of in clinical neuropsychological evaluations
Web-based resources. are not defined in this dictionary. How-
The widespread availability of medical ever, in keeping with the general theme of
and psychological information on the In- providing a neuropsychological perspective
ternet poses special challenges for concep- whenever possible, some medications are
tualizing how a dictionary can become a included based on neuropsychological rele-
unique resource because a variety of mate- vance or based on differential response com-
rials for any dictionary entry is only a few pared to similar medications in the same
clicks or taps away. Although there is nothing drug class.
wrong with this approach to understanding References for many entries are now in-
new or unfamiliar terms, particularly when cluded. Although the goal of this dictionary
the links are to reputable websites, there is was never intended to be encyclopedic in
no overarching consistency in the content nature, citations allow interested readers to
of material discovered. This dictionary, how- seek out original source material if interested
ever, is designed as much to provide “top- or, when appropriate, to easily find author-
down” guidance to identify relevant topics itative information for selected entries. The
for students of clinical neurosciences as to inclusion of references also permits proper
provide definitions for neuropsychological recognition of original descriptions of im-
terms. Thus, relevance is achieved not only portant brain-based concepts because psy-
by specific definition content, but also by chology and neurology are strongly influ-
the choice of the specific items included. enced by historical context. Although I did
Unlike Web-based entries that tend to be not strive to include all possible assessment
used as needed on an individual item basis, measures that have been used to charac-
my hope is that this dictionary will serve, terize cognition or behavior in neuropsy-
in part, as a textbook that can be read, or at chology, I did try to include a broad sampling
least perused, to identify new concepts or of tests and questionnaires to illustrate the
terms to facilitate greater understanding of large number of assessment approaches that
the many terms encountered in neuropsy- exist outside commonly employed clinical
chology, in related fields of medicine, and tests.
in both clinical and basic research into This dictionary has adopted the conven-
brain–behavior relationships. The choice of tion of not using the possessive when de-
specific item content reflects my personal scribing diseases, syndromes, or regions that

ix
Preface to the Second Edition

are eponyms. Although this may be jarring also common for me to ask my Emory col-
for some readers’ eyes and ears, it reflects leagues for their opinions of initial drafts of
movement away from their use in the med- definitions, and I would like to extend my
ical and professional literature, at least in appreciation to Albert Anderson, Donald
North America. Bliwise, Bruce Crosson, Dan Drane, Stewart
I have been fortunate to always have Factor, Marla Gearing, Robert Gross, William
worked in diverse and stimulating academic Hu, Mahlon DeLong, David Rye, and Camille
and clinical environments, beginning at Vaughan for their assistance in clarifying
Baylor College of Medicine, University of content. Through the generosity and efforts
Texas Medical Branch, followed by posi- of multiple dictionary contributors and
tions at the Medical College of Georgia, item writers, my hope is that this dictionary
Georgetown University, and the University reflects a concise but balanced presen­tation
of Florida prior to arriving in Atlanta at of topics relevant both for students begin-
Emory University. Many persons with ning their careers and for more seasoned
whom I have worked over the years agreed professionals.
to develop entries for this dictionary, and
they are formally recognized in the contrib- David W. Loring, Ph.D.
utor list. While developing entries, it was December, 2014

x
Preface to the First Edition

Neuropsychology is a diverse discipline specific entries were based upon consultation

with varied practice and application; this with the editorial board, and at times, there
dictionary is intended to reflect that diver- was no rational basis for making a choice
sity. To some, there will be too many tests, other than simply including the informa-
to others, too much peripheral neurology, tion that was first entered into the dic-
to some, too much historical context. One tionary. Thus, this dictionary should not
advantage of a dictionary, however, is that be considered definitive in these areas, al-
it is by definition athematic. Unlike texts though in all cases, there was a credible
that tend to be read in sections, chapters, source for the information contained. Unless
or even in their entirety, dictionary entries a person’s name is associated with a clinical
that are not of interest can be easily ig- syndrome, biographical information is gen-
nored and skipped. However, it is hoped erally limited to individuals who are no
that this dictionary will be a valuable re- longer alive.
source to many. As Samuel Johnson ob- In addition to standard editorial deci-
served, “Dictionaries are like watches; the sions regarding entries, we have chosen to
worst is better than none, and the best be brief in descriptions of approaches to
cannot be expected to be quite true.” detect symptom exaggeration. Although we
Many medical abbreviations are included. realize that much of this kind of informa-
Nonmedical students experience a consider- tion is already in circulation, we prefer that
able disadvantage compared to their med- this volume not be a primary source of in-
ical school counterparts because, unlike formation used to subvert the validity of
medical training, in which there are clinical clinical neuropsychological testing.
rotations during the junior and senior year I thank all contributors to this volume
in which many of the abbreviations can be and acknowledge the influence of Daniel
learned from a junior or senior resident, no E. Sheer, Ph.D., the founding director of
comparable training is generally present for the University of Houston Clinical Neu-
neuropsychologists. ropsychology program, on this product; I
Although we have tried to be as objective dedicate my portion of this work to his
as possible when making specific entries, and memory. Persons who repeatedly reviewed
have approached the definition writing with the entire manuscript are noted in the con-
a certain degree of expertise, this was not tributor list as editorial consultants to re-
always the case for the etymologies or for flect their significant contributions over this
the brief biographies. Since we are not extend period. I thank my MCG colleagues
­experts in classic Greek or Roman lan- whose many hallway consultations have
guages nor in the history of medicine and significantly improved the quality of many
psychology, we have relied on a variety of dictionary entries, as well those who re-
sources for this material. Unfortunately, sponded to NPSYCH queries with informa-
there were occasional discrepancies in dif- tion that could not be identified elsewhere.
ferent source material regarding word ori- Thomas R. Swift, M.D., MCG Department
gins or specific dates, and the choice of of Neurology Chair, continues to provide

xi
Preface to the First Edition

the multidisciplinary environment that for his helpful comments and for advice,
allows scholarly activity to be performed but also for his patience and tolerance.
despite increasing pressures for clinical
productivity. Finally, I thank Jeffrey W. David W. Loring, Ph.D.
House, Oxford University Press, not only July, 1998

xii
Item Writers and Reviewers

Peter Arnett, Ph.D. Neil R. Lava, M.D.

Penn State University Emory University

Erin D. Bigler, Ph.D. Emila Lojek, Ph.D.

Brigham Young University University of Warsaw

Justin J. Boseck, Ph.D. Sherrill R. Loring, M.D.

Trinity Health Shepherd Center

Dawn Bowers, Ph.D. Bernice Marcopulos, Ph.D.

University of Florida James Madison University

Leslie Breting, Ph.D. Fadi Nahab, M.D.

NorthShore University HealthSystem Emory University

Adam M. Brickman, Ph.D. Margaret O’Connor, Ph.D.

Columbia University Beth Israel Deaconess Medical Center

Morris Cohen, Ed.D. David C. Osmon, Ph.D.

Georgia Regents University University of Wisconsin-Milwaukee

Christopher D’Amato, Ph.D. Kati Pagulayan, Ph.D.

VA Hudson Valley Health Care VA Puget Sound Health Care System

Kathy Y. Haaland, Ph.D. Patrick Riordan, Ph.D.

University of New Mexico Edward Hines Jr. VA Hospital

David E. Hartman, Ph.D. Lynn A. Schaefer, Ph.D.

Private Practice Nassau University Medical Center

Bruce Hermann, Ph.D. Guy Vingerhoets, Ph.D.

University of Wisconsin Ghent University

Laura Hokkanen, Ph.D. Ann D. Watts, Ph.D.

University of Helsinki Entabeni Hospital

Reema Jayaker, M.A. Barbara A. Wilson, Ph.D.

Georgia State University MRC Applied Psychology Unit

Roy Kessels, Ph.D.

Radboud University Nijmegen

xiii
INS Dictionary of Neuropsychology
and Clinical Neurosciences
 A

Aβ—β-amyloid protein A 40– to 42– AAC See Augmentative or alternative com-

amino acid peptide that is produced from munication.
the amyloid precursor protein (APP) Aachen Aphasia Test A linguistically ori-
during normal cell metabolism via the ented, standardized aphasia battery in
action of two proteases, β-secretase and German. Spontaneous speech is rated on
γ-secretase. This peptide is a main compo- six dimensions: communicative behavior,
nent of amyloid plaques, a characteristic articulation and prosody, automatized
pathological finding in brains of Alzheimer *lan­guage, semantic structure, phonolog-
disease patients and is thought to be a ical structure, and syntactic structure. Per-
major contributor to disease development formance on five subtests is also rated on
(see also Amyloid hypothesis). Abnormal *token test, repetition, written language,
accumulations of Aβ can also sometimes *confrontation naming, and comprehen-
be found in blood vessel walls, termed am- sion. With the exception of the token test,
yloid angiopathy. In Alzheimer disease, which has a dichotomous scoring, responses
changes in amyloid can be detected in *ce- on the subtests are rated on a 4-point scale.
rebrospinal fluid and visualized with brain The Aachen Aphasia Test is commonly
*positron emission tomography (PET) employed to test for *aphasia classification,
amyloid imaging. Possible therapeutic strat­ patient description, and treatment evalu-
egies for the pharmacological manage- ation effects in German-speaking countries.
ment of Alzheimer disease have attempted Huber, W., Poeck, K., & Willmes, K. (1983).
to reduce Aβ synthesis by targeting en- Der Aachener Aphasie Test. Göttingen, Germany:
zymes and/or increasing *clearance of Aβ Hogrefe.
with immunotherapies. (Amyl- + -oid; ABA design (reversal design) An ex-
from Latin amylum, starch; from Greek perimental design to assess intervention
amulon, starch, the finest flour, from neuter effects in individual participants. In the
of amulos, “not ground in a mill,” a-, not + first A phase, baseline target behaviors
mulé, mill) are measured. An intervention is applied
A Quick Test of Cognitive Speed (AQT) during the B condition while the same
A standardized screening measure for *de- behaviors are assessed. The second A phase
mentia based on cognitive speed through (i.e., reversal) measures the effect of with-
the rapid naming of colors and familiar drawing the intervention and is used to
shapes, and then naming both the color demonstrate that the intervention does
and the shape (e.g., red circle, yellow not simply reflect practice by allowing
square). The AQT reportedly takes 3–5 performance to return to or approach
minutes to administer, has no *ceiling baseline levels. An ABAB design is a var-
or *floor effect, and performance is in- iant that studies the treatment effect on
dependent of education or culture. Orig- two occasions and has the clinical advan-
inally called the Alzheimer’s Quick Test. tage of facilitating treatment beyond the
Wiig, E. H., Nielsen, N. P., Minthon, L., & experimental requirements of the study.
Warkentin, S. (2002). AQT: A quick test of Abasia Inability to walk. See Astasia-
cognitive speed. San Antonio, TX: Pearson. abasia. (Greek a, not; basis, a step)

1
ABCs Absence seizure

ABCs Airway, breathing, and circulation & Shaywitz, S. E. (2002). Classification of

(pulse). learning disabilities: An evidence-based
evaluation. In R. Bradley, L. Danielson, &
Abducens nerve Cranial nerve VI. The
D. P. Hallahan (Eds.), Identification of learning
abducens nerve controls the lateral rectus disabilities: Research to practice (pp. 185–250).
muscle, which moves the eyes laterally and Mahwah, NJ: Lawrence Erlbaum.
away from midline (abducts the eye). It
emerges anteriorly between the pons and Abiotrophy A programmed loss of neurons
*medulla to innervate the *ipsilateral lat- in neurodegenerative diseases reflecting a
eral rectus muscle and, due to its rela- genetic abnormality (e.g., *Huntington
tively long length, is susceptible to injury disease). Neural loss may begin at any age
from a variety of causes including skull from infancy to old age. The term is more
fracture, intracavernous carotid artery an- commonly used with diseases with later
eurysms, or mass lesions exerting pressure presentation onset. (Greek a-, without;
on the brainstem. (Latin abductus: ab-, bios, life; trophe, nourishment)
away + dūcere, to lead) Ablation Surgical removal or destruction
Abduction Movement away from the mid­ of tissue which, in neurosurgical applica-
line, the opposite of adduction. (Latin tions, is performed to eliminate or reduce
abductus: ab-, away + dūcere, to lead) abnormal brain function. Anterior tem-
Ability–achievement discrepancy An poral *lobectomy, which is used to treat
approach to identifying the presence of a medically refractory *epilepsy, is an ex-
specific *learning disability based on the ample of a surgical ablative procedure.
magnitude of discrepancy between *IQ *Deep brain stimulation in *Parkinson
and formal achievement test scores (e.g., disease, which is designed to interrupt ex-
reading, writing, arithmetic). A specific citatory motor pathways, is an example of
learning disability was said to be present ablation by electrical lesioning. Transient
following “a severe discrepancy between pharmacological ablation may also be
achievement and intellectual ability” in achieved using medications; as used to
an appropriate academic area (34 C.F.R. identify cerebral *language and *memory
§ 300.541, November 29, 1976) (repealed). dominance during the *Wada test. (Latin
This approach to identifying learning dis- ablatus, past participle of auferre, to carry
abilities has been criticized because it fails away)
to identify children with unique educa- Abscess, brain An *encapsulated collec-
tional needs (e.g., poor readers across IQ tion of pus in the brain associated with
levels benefit from similar reading inter- localized infection that most commonly
ventions). Also, by delaying intervention results from suppuration elsewhere in
until grades 3–4 when predetermined dis- the body; it can develop either from a
crepancy criteria frequently emerge, local source (e.g., brain trauma) or from
learning-disabled children not only fall a distant site by hematogenous spread.
further behind their peers, but treatment The most common identified source is
interventions are also more difficult to im- lung infection. Brain abscesses typically
plement. A major criticism of the ability– present with deficits similar to other
achievement discrepancy is that it does space-occupying lesions but are often
not establish a group of students with difficult to treat with antibiotics because
unique needs. the encapsulation wall acts as a barrier,
Fletcher, J. M., Lyon, G. R., Barnes, M., and surgical drainage is often necessary.
Stuebing, K. K., Francis, D. J., Olson, R. K., Absence seizure See Seizure.

2
Absolute risk reduction (ARR)Acalculia

Absolute risk reduction (ARR) The nouns are presented; the task is to decide
magnitude of decreased risk of an ad- whether each noun is abstract or con-
verse outcome associated with treatment crete. This approach has been used to
intervention relative to a different treat- investigate dual coding of information
ment or to no treatment. Because ARR in semantic *memory, in which different
is expressed as a proportion of the total neural processing of abstract and concrete
population, absolute risk reduction may nouns has been suggested. Abstract words
be a better indication of risk reduction tend to be encoded into the semantic
when the risk is low than the use of *rel- system linguistically based on verbal in-
ative risk reduction. Calculated as the formation, and concrete words tend to
difference between the *control event rate be encoded using both verbal (linguistic)
(CER) minus the *experimental event and multimodal (nonverbal, imagistic)
rate (EER), it is the inverse of number systems.
needed to treat (NNT). Also called risk Abulia Lack of initiative or drive, accom-
difference. See also Relative risk reduction. panied by a lack of spontaneity in speech,
Absolutism (cultural absolutism) The thought, and initiative. Abulia may be as-
position that human behaviors are sim- sociated with psychosis and neurological
ilar across cultures and that the cultural disease. In the latter, it is associated with
environment plays a minor role in shap- disruptions of frontal-subcortical circuitry
ing behavior. In neuropsychology, cultural such as bilateral lesions in the medial
absolutism implies that similar assessment *frontal lobe or orbital frontal lobe from
methods may be used regardless of a tumor, severe traumatic brain injury, or
­patient’s cultural background and that degenerative processes such as *Pick dis-
neuropsychological constructs are largely ease. Abulia is less frequently seen with
invariant. lesions of the *caudate nuclei, *midbrain,
Abstract attitude Capacity for abstrac- or *thalamus. Social abulia is inactivity
tion characteristic of normal brain func- from an inability to select a course of
tion; the ability to generalize from the action, although a wish to participate may
particular. Loss of abstract attitude was be present. Also spelled aboulia. (Greek a-,
used to describe the inability to see beyond without; boulesis, will)
the most simple or pedestrian concepts. ACA See Anterior cerebral artery.
Kurt Goldstein (American neurologist Academic achievement Knowledge and
born in Germany, 1878–1965) considered skills that are acquired during formal ed-
loss of abstract attitude to be the car- ucation. In school and neuropsycholog-
dinal feature produced by *frontal lobe ical contexts, achievement is characterized
damage and psychosis, although Gold- by proficiency in specific academic con-
stein’s focal lesion studies showed that tent areas that are assessed formally on
the sign could be observed with damage measures such as reading, writing, and
in many locations. The term is not in arithmetic. See also Ability–achievement
active use now but is still frequently en- discrepancy.
countered in older literature. Abstraction Acalculia Acquired disturbance of com-
is now interpreted as an example of in- putational ability that is commonly as-
ductive reasoning or *fluid intelligence. sociated with both *alexia and *agraphia
See Cattell-Horn-Carroll model. for numbers. Both oral and written cal-
Abstract/concrete task A measure of culation are commonly impaired. Acal-
concreteness in which abstract or concrete culia subtypes include primary acalculia

3
Accessory nerve (spinal accessory nerve) Acetylcholine (ACh)

(anarithmetria) and spatial acalculia. eye adduction, and eye lenses becoming
Milder forms of acalculia may affect divi- more convex. The *afferent (sensory)
sion and multiplication more than addi- input for visual accommodation occurs
tion and subtraction. The lesion classically through the *optic nerve (*cranial nerve
involves the left angular *gyrus (Brodmann II), and the *efferent (motor) output
areas 39 and 40), although computational occurs via the oculomotor nerve (cranial
impairment may be associated with aphasia nerve III). (Latin accommodāre, to make
resulting from any perisylvian lesion. Acal- fit; ad- + commodus, fit, “conforming with
culia is one of the constellation of symp- the measure”)
toms comprising *Gerstmann syndrome. Acculturation The process of *learning
(Greek a-, without; Latin calculare, to the style of the dominant and prevailing
reckon, from calculus, a pebble) culture, including the experiences and
• Anarithmetria See Primary acalculia, changes that groups and individuals un-
below. dergo, through interaction with other cul-
• Primary acalculia Acquired calcula- tures. Acculturation occurs in the context
tion impairment that, according to of continuous contact of two or more
Henri Hécaen (French neurologist, groups from different cultures for ex-
1912–1983), cannot be explained by tended periods of time.
alexia or agraphia for numbers or by Acetylcholine (ACh) An excitatory *neu-
spatial disorganization for numbers. rotransmitter that originates in the pedun-
In practice, however, the term often culopontine and laterodorsal tegmental
refers to calculation difficulty regard- nuclei in the *brainstem and in the *nu-
less of other neuropsychological defi- cleus basalis of Meynert in the basal *fore-
cits. Also known as anarithmetria. brain. *Cholinergic neurons act centrally
• Spatial acalculia Impaired ability to more in neuromodulation than in neu-
perform written calculations due to rotransmission, and the main functions
failure to properly process spatial as- of central acetylcholine are facilitation
pects of written problems. In contrast of attention, *learning, and *memory. De-
to primary acalculia, spatial acalculia creased ACh has been demonstrated in
is more frequently associated with *Alzheimer disease, which is the rationale
right hemisphere lesions (e.g., manip- for anticholinesterase therapy. ACh also
ulating numerals across columns). plays a prominent role in *involuntary
Accessory nerve (spinal accessory movement disorders, which are often
nerve) *Cranial nerve XI. Motor nerve managed by decreasing caudate ACh.
innervating the trapezius and sternoclei- Because decreased ACh is associated with
domastoid muscles and controlling head anterograde *memory impairment, how-
turning and tilting and shoulder shrug. ever, anticholinergic therapy for move-
Accessory nerve injury is typically asso- ment disorders may be associated with
ciated with shoulder impairment, and negative cognitive side effects. In the
trauma or surgery are common causes of peripheral nervous system, ACh is the
injury. (Medieval Latin accessor, helper, principal neurotransmitter at the neu-
accessory, subordinate; from Latin accessus, romuscular junction, preganglionic au-
access) tonomic synapses, and postganglionic
Accommodation (visual) Reflexive ad- parasympathetic synapses. *Myasthenia
justments of the eyes to view a near object gravis is characterized by a defect in pe-
that includes constriction of the pupils, ripheral ACh transmission.

4
Acetylcholinesterase inhibitors Acrodynia (Pink disease)

Acetylcholinesterase inhibitors See (Greek a‑, without; chroma, color; opsis,

Cholinesterase inhibitors. sight, vision, from optikos, denoting
Acetylsalicylic acid (ASA) Aspirin. ASA sight)
is a nonsteroidal anti-inflammatory, an- ACoA See Anterior communicating artery.
algesic, and antipyretic drug that is also Acoustic agraphia See Agraphia.
used in the treatment of cardiovascular Acoustic neuroma A benign, slow-growing
disease and ischemic (i.e., nonhemor- tumor arising from *cranial nerve VIII.
rhagic) *stroke because it decreases blood The tumor may displace and compress
viscosity. the *pons and lateral *medulla and ob-
Achievement tests Measures designed struct *cerebrospinal fluid (CSF) circu-
to assess school-based *learning and com- lation. Symptoms include disturbance of
petence for specific skills such as reading, hearing, headache, tinnitus, disturbance
spelling, or arithmetic. Achievement test of balance, and unsteadiness of gait.
performance is often contrasted with per- (Greek akoustikos, denoting sound; neuron,
formance on *IQ tests, which are consid- nerve; -oma, suffix meaning morbid growth,
ered measures of learning potential. See tumor)
also Ability–achievement discrepancy. Acquired immune deficiency syndrome
Achilles tendon reflex A *deep tendon (AIDS) The late clinical expression of
reflex in which a twitch-like contraction infection with *human immunodefi-
of the calf muscles and plantar flexion at ciency virus (HIV), recognized as a dis-
the ankle is elicited by sharply tapping tinct disease in 1981. AIDS is associated
the Achilles tendon above the heel. This with reduction in CD4+ T-lymphocytes.
reflex may be abolished by lesions of the According to the *Centers for Disease
first sacral root (S1) or peripheral neu- Control and Prevention (CDC), AIDS
ropathy. Also called ankle jerk. is operationally defined as fewer than
Achromatopsia Loss of color vision fol- 200 CD4+ T-lymphocytes per μL, or a
lowing *temporal-*occipital lobe damage. CD4+ T-lymphocyte percentage of less
Color loss is usually restricted to one than 14% of the total lymphocyte count
*hemifield (hemiachromatopsia), although with an accompanying AIDS-defining
full-field achromatopsia may occur. Clini- condition. AIDS may affect the nervous
cally, patients may complain of washed-out system at any level, resulting in AIDS
colors or reduced brightness or vibrancy of *dementia, cerebral *lymphoma, intra-
colors. In severe cases, color perception cranial infectious processes such as *cy-
may be completely lacking and patients tomegalovirus (CMV), *encephalitis,
may complain of seeing everything in gray. toxoplasmosis, *meningitis, vacuolar
Achromatopsia differs from *color agnosia, myelopathy, neuromuscular transmis-
in which verbal color association is im- sion defects, peripheral neuropathy, or
paired because color discrimination is also myopathy. See also AIDS dementia;
affected. Achromatopsia is often accom- HAND (HIV-associated neurocognitive
panied by *prosopagnosia, topograph- impairment).
agnosia, and superior *homonymous field Acrodynia (Pink disease) A rare early
defects, or *alexia with a left brain/right childhood toxic neuropathy associated
*hemianopia. The etiology of achromatop- with mercury exposure involving severe
sia is typically vascular, from posterior hand and foot pain and pink discolora-
cerebral artery occlusion or coagulapa- tion; often accompanied by irritability
thy affecting lingual and fusiform gyri. and *light sensitivity. Acrodynia was

5
ACTH Acute confusional state (delirium, encephalopathy)

prevalent in the first half of the 20th stages of the disease. Subtypes of action
century and was attributed to mercury tremor include *postural tremor, which
commonly found in teething powders. is seen when the patient maintains a
Mercury in teething powders was largely position against gravity, such as when
abolished in the 1950s. (Greek akros, top- holding arms outstretched; kinetic
most, extreme + -dynia, pain) tremor, which is present during move-
ACTH See Adrenocorticotropic hormone. ment of a body part, such as moving
Action imitation task A task requiring wrists up and down; and *intention tremor,
the imitation of specific actions. This task which is present during a purposeful
has demonstrated that known gestures are movement toward a target and is com-
reproduced using a *semantic, indirect monly tested clinically by having the pa-
route, whereas novel gestures are repro- tient touch the examiner’s moving finger,
duced using a sublexical, direct route. alternating with touching his or her own
Tessari, A., Canessa, N., Ukmar, M., & Rumiati, nose.
R. I. (2007). Neuropsychological evidence Activities of daily living (ADLs) Skills
for a strategic control of multiple routes in necessary for independent or semi-inde-
imitation. Brain, 130(4), 1111–1126. doi: 10.1093/
brain/awm003
pendent living. Physical self-maintenance
activities of daily living (or “personal
Action potential Rapid, transient, all- ADLs”) include toileting, feeding, dress­
or-none nerve impulse initiated at the ing, grooming, physical ambulation, and
axon hillock. The action potential is the bathing. Instrumental activities of daily
electrical signal by which the brain re- living are more complex everyday behav-
ceives, analyzes, and conveys informa- iors and include the abilities to use the
tion. The action potential in patients telephone, shop, prepare food, perform
with *multiple sclerosis decreases as the house­keeping tasks such as laundry and
*myelin sheath degenerates with disease handling finances, and take medications
progression. responsibly. The first ADL scale with
Action Programme Test A subtest from widespread acceptance was published in
the Behavioral Assessment of the *Dysex- 1963.
ecutive Syndrome (BADS) test designed Katz, S., Ford, A. B., Moskowitz, R. W., Jackson,
to assess ability to devise and implement B. A., & Jaffe, M. W. (1963). Studies of illness
in the aged. The index of ADL: A stan­
a solution to a problem following certain
dardized measure of biological and psy­
rules. The task is to get the cork out of chosocial function. Journal of the American
the tube using the objects in front of the Medical Association, 185, 914–919. doi:
participant but without lifting up the 10.1001/jama.1963.03060120024016
stand, the tube, or the beaker, and without Activity limitations Difficulties an in-
touching the lid. dividual may have in executing specific
Action tremor A rhythmic tremor that tasks or actions (e.g., stairs, standing or
occurs during any type of movement of sitting for several hours at a time, grasping
the affected body part and that is often small objects, or carrying items such as
worse at the end of motor activity. Action groceries). Activity limitations are often
tremor occurs with fine movements such used to characterize *disability.
as writing or lifting a cup for drinking. Acute confusional state (delirium, en-
*Parkinson disease patients often have a cephalopathy) Altered mental status
combined *resting tremor and a faster that develops rapidly and is character-
action tremor, particularly in the later ized by altered *consciousness, impaired

6
Acute disseminated encephalomyelitis (ADEM) Adenoma

*cognition, limited course, and identifi- Addenbrooke’s Cognitive Examination–

able etiologies. Elderly patients are at risk Revised (ACE-R); Addenbrooke’s Cog-
for developing acute confusional states nitive Examination III (ACE-III) A cog-
from metabolic abnormalities or infec- nitive screening battery assessing five
tions, and hospitalization increases the domains: orientation/attention, *memory,
risk for *confusional state development. verbal *fluency, *language, and visuo-
Patients behaviorally may be either hy- spatial function. The ACE-R incorpo-
poactive, characterized by being with- rates portions of the *Mini-Mental State
drawn, quiet, and sleepy, or hyperactive, Examination (MMSE) and takes between
characterized by heightened arousal 12 and 20 minutes to administer and score
­including restlessness, agitation, or ag- in a clinical setting. Three versions are
gression. available for repeated assessments. Named
Acute disseminated encephalomyelitis after Addenbrooke’s Hospital in Cam-
(ADEM) An acute demyelinating dis- bridge, UK, the ACE-III was introduced
ease that may follow a viral syndrome to overcome limitations associated with
or vaccination or have no identifiable the ACE-R including repetition (“no ifs,
predisposing cause. Symptoms develop ands, or buts”), spelling “world” back-
rapidly, beginning with *encephalitis- ward, and poor sensitivity of comprehen-
like symptoms such as fever, fatigue, sion items. Unlike the ACE-R, the MMSE
headache, nausea and vomiting, and, score cannot be derived. The ACE-III
in the most severe cases, *seizures and was developed in Sydney, Australia.
*coma. Because ADEM affects myelin, Mioshi, E., Dawson, K., Mitchell, J., Arnold, R.,
neurological symptoms often include & Hodges, J. R. (2006). The Addenbrooke’s
Cognitive Examination Revised (ACE-R):
optic nerve inflammation, weakness,
A brief cognitive test battery for dementia
and difficulty coordinating voluntary screening. International Journal of Geriatric
muscle movements. ADEM is occa- Psychiatry, 21(11), 1078–1085. doi: 10.1002/
sionally misdiagnosed as a severe ini- gps.1610
tial *multiple sclerosis (MS) attack Hsieh, S., Schubert, S., Hoon, C., Mioshi, E.,
­because its symptoms and *magnetic & Hodges, J. R. (2013). Validation of the
Addenbrooke’s Cognitive Examination III
resonance imaging (MRI) scan may be in frontotemporal dementia and Alzheimer’s
similar. disease. Dementia and Geriatric Cognitive
AD See Alzheimer disease. Disorders, 36(3–4), 242–250. doi: 10.1159/
Adaptive behavior Conceptual, social, 00035167; ACE-III download: http://www.
and practical skills necessary to func- neura.edu.au/frontier/research/test-
downloads
tion effectively in everyday life. Adaptive
behaviors are commonly assessed in
­ Adduction Movement toward the body.
*learning disability evaluations by using Opposite of abduction. (Latin adducer,
questionnaires and observation and are to bring to; ad-, toward + dūcere, to lead)
typically characterized in three areas: ADEM See Acute disseminated encephalo-
practical skills that include activities of myelitis.
daily living, conceptual skills that reflect Adenoma A benign epithelial tumor in
cognitive abilities, and social skills that which tumor cells arise from glandular
reflect interpersonal skills and social structures, the most common of which
problem-solving. is the *pituitary. (Greek aden, signifying
ADD See Attention-deficit/hyperactivity of a gland or glands; -oma, suffix meaning
disorder. morbid growth, tumor)

7
ADHD Adult Behavior Checklist (ABCL)

ADHD See Attention-deficit/hyperactivity Adrenergic neurons Neurons that se-

disorder. crete *epinephrine or *norepinephrine.
Adiadochokinesia Deficit of rapidly al- Neurons containing norepinephrine (also
ternating movements. This is commonly called noradrenaline) are located in the
tested by having patients slap the palm *locus coeruleus and lateral tegmental
and then the back of their hand in rapidly area in the *brainstem and project to the
alternating fashion on their opposite spinal cord, cerebellum, brainstem, thal-
palm or thigh. Adiadochokinesia results amus, and entire *forebrain. The ascending
from cerebellar lesions but may also be norepinephrine system is important in
seen with cerebral lesions, thus implicat- modulating attention, sleep-wake cycle,
ing cerebellar-cortical pathways. Also called and *mood. Epinephrine (adrenaline) is
*dysdiadochokinesia. (Greek a-, without; found in a small number of brainstem
diadoche, working in an orderly succes- neurons and may play a role in blood
sion; kinesis, movement) pressure control.
Adipsia Absence of thirst, characterized Adrenocorticotropic hormone (ACTH)
by an absence of drinking. The associ- A hormone secreted by the *pituitary
ated lesion for adipsia is located in the gland; it is the major regulator of ad-
lateral *hypothalamus. (Latin a-, without; renal activity. ACTH levels follow a
dipsia, thirst) *circadian rhythm, with elevated levels
ADL See Activities of daily living. in the early morning and depressed
ADME Absorption, distribution, metab- levels in the late afternoon and early eve-
olism, elimination, which characterize ning. ACTH is increased in Addison
drug levels in an organism and the ki- disease, congenital adrenal hyperplasia,
netics of drug exposure. ACTH-secreting tumors, stress, surgery,
Adrenal glands Triangular-shaped duct- *electroconvulsive therapy (ECT), and
less endocrine glands that sit above each hypoglycemia and is decreased in panhy-
kidney and release hormones in response popituitarism and adrenal cancer. Also
to stress through the synthesis of *cor- called *corticotropin.
ticosteroids (e.g., *cortisol) and *cate- Adrenoleukodystrophy A genetic dis-
cholamines (e.g., *epinephrine (adren- ease of childhood transmitted as a sex-
aline) and norepinephrine). The adrenal linked recessive trait marked by diffuse
glands are subdivided into two broad abnormality of the cerebral *white matter
regions: the adrenal cortex and the ad- and adrenal atrophy. It is characterized
renal medulla. (Latin ad, to; ren, kidney by cognitive impairment that progresses
+ -al ) to *dementia and by *aphasia, *apraxia,
• Adrenal cortex The outer layer of *dysarthria, and impaired vision. An adult-
the adrenal gland. It is stimulated onset variant exists in which the disease
from the pituitary gland by *adreno- progresses more slowly, although it too
corticotropic hormone (ACTH) to is associated with deterioration of brain
produce steroids. The adrenal cortex function. (adrenal + Greek leukos, white;
regulates metabolism, *blood pressure, dys-, hard, ill, bad; trophe, nourishment,
and sexual behavior. from trephein, to nourish)
• Adrenal medulla The central por- Adult Behavior Checklist (ABCL) A
tion of the adrenal gland. The adrenal checklist of 118 rating items for the pres-
medulla secretes *epinephrine and *nor­ ence of problem behaviors of adults be-
epinephrine. tween 18 and 59 years of age in the domains

8
Adult Memory and Information Processing Battery (AMIPB) Advanced Clinical Solutions

of aggressive behavior, rule-breaking and, unlike many memory tests, con-

behavior, intrusive thoughts, thought
­ tains alternative test versions. When up-
problems, attention problems, anxious/ dated, it was renamed the Brain Injury
depressed mood, withdrawn behavior, Rehabilitation Trust Memory and In-
and somatic complaints. It was developed formation Processing Battery (BIRT).
as an upward extension of the *Child Coughlan, A. K., & Hollows, S. E. (1985). The
Behavior Checklist. The scales are com- Adult Memory and Information Processing
bined into summary measures of exter- Battery (AMIPB). Leeds: AK Coughlan, St.
James’s University Hospital.
nalizing problems and internalizing
problems. Like the CBCL, information Adult polyglucosan body disease
is obtained from an informant who knows (APBD) A rare, progressive metabolic
the individual well, such as a spouse, dis­ease affecting both *upper and *lower
partner, family member, or friend. motor neurons that results from accu-
Achenbach T. M., & Rescorla L. A. (2003). mulation of polyglucosan bodies prima-
Manual for the ASEBA adult forms & profiles. rily in the militated nerve fibers. Patients
Burlington, VT: University of Vermont, have difficulty walking from a combina-
Research Center for Children, Youth, &
Families.
tion of decreased sensation in the legs and
muscle weakness. Altered cognitive func-
Adult Memory and Information Proc- tion is present in approximately half the
essing Battery (AMIPB) A *memory patients developing APBD. The interval
assessment battery consisting of six sub- between onset of symptoms and death
tests: information processing parts A and generally ranges from 3 to 21 years.
B (number cancellation, motor speed Advance medical directive A legal doc-
cancellation); immediate and delayed ument that details one’s wishes concerning
story recall containing a story similar to life-sustaining treatments (“living will”)
the *Logical Memory test; a 15-word list or assigns someone to make such deci-
*learning task similar to the *Rey Audi- sions if one becomes incapacitated and
tory Verbal Learning Test consisting of is unable to provide such guidance (e.g.,
five *learning trials, an interference trial durable medical power of attorney). The
of a new word list, and a memory trial right to refuse life-sustaining treatment
of the original list; a complex design test was first established in case law by the
similar to the Rey-Osterrieth Complex New Jersey Supreme Court in the case
Figure (copy, immediate and delayed recall of Karen Ann Quinlan (1976); the court’s
trials); and a design learning test (five ruling recognized a constitutional right
learning trials, interference, and postint- to refuse unwanted interventions, in-
erference recall) that is a nonverbal ana- cluding life-sustaining treatment, and
logue to the word-list learning subtests. opined that when patients are unable to
The AMIPB is popular in the UK, but it exercise that right, family members may
is not offered by a formal test publisher forego life support on behalf of incom-
and consequently is not well known. It petent loved ones, based on the patient’s
is designed to take 45–60 minutes to ad- wishes and best interests.
minister and contains alternative test Advanced Clinical Solutions A formal
versions. The standardization study is clinical scoring supplement to the
based on 184 adults between 18 and 75 *Wechsler Adult Intelligence Scale–4th
years of age. The test is designed to take Edition and Wechsler Memory Scale–4th
45 minutes to an hour to administer Edition that generates demographically

9
Advanced Progressive Matrices Ageusia

adjusted scores, performance validity Gunn pupil is often present with *mul-
measures from stand-alone and embed- tiple sclerosis or other demyelinating
ded measures, an estimate of premorbid disease (named for Robert Marcus Gunn,
function based on reading of words with Scottish ophthalmologist, 1850–1909).
atypical *grapheme-to-*phoneme rela- Afterdischarge Abnormal pattern of cell
tionships, reliable change scores for re- discharges that may appear at and above
peated assessments, and a measure of social a specific electrical threshold during elec-
*cognition. trical cortical stimulation mapping. Af-
Advanced Progressive Matrices See terdischarges are thought to result from
Raven Progressive Matrices. synchronization of preexisting local field
Adverse event (AE) In investigational potentials through temporary inactiva-
drug trials, any untoward, noxious, or tion of inhibitory *interneurons.
unintended event experienced by a re- Kalamangalam, G. P., Tandon, N., & Slater, J. D.
search participant in a clinical trial, re- (2014). Dynamic mechanisms underlying
gardless of whether the occurrence is afterdischarge: A human subdural recording
study. Clinical Neurophysiology, 125(7), 1324–
thought to be related to the investiga- 1338. doi: 10.1016/j.clinph.2013.11.027
tional drug.
Aesthesiometer An instrument to mea­ Agenesis of the corpus callosum A
sure tactile sensitivity; it employs a com- congenital condition in which the *corpus
pass-like device with nylon filaments of callosum fails to develop. Patients typi-
increasing thickness to assess the thresh­ cally present with intellectual *disability
old or minimum spatial separation for and *epilepsy, and it has been described
two-point perception. (Greek aisthesis, sen- as an isolated condition or in combina-
sation; metron, measure) tion with other brain abnormalities, in-
Affect Observed behaviors that reflect un- cluding *Chiari malformation, Dandy-
derlying emotional tone or feeling state. Walker syndrome, Andermann syndrome,
This contrasts with *mood, which is a schizencephaly, and *holoprosenceph-
more sustained and pervasive subjective aly. Girls may have a gender-specific con-
emotional experience. Affect is revealed by dition called Aicardi syndrome. However,
facial expressions, tone of voice, speech agenesis of the *corpus callosum has also
content, and motor behaviors. Disturbed been observed in healthy individuals.
affect may be described as blunted, flat, The agenesis may not be complete, and
inappropriate, *labile, restricted, or con- partial agenesis tends to involve the pos-
stricted. (Latin affectus, state of mind, terior corpus callosum. Also called cal-
from afficio, to have influence on) losal agenesis. (Greek a-, without; genesis,
Afferent Neural conduction toward the beginning)
*central nervous system characteristic Ageusia Loss of taste sense. Ageusia is
of sensory or receptor neurons. (Latin common in Bell palsy over the anterior
afferens, past participle of afferre, ad, to; two-thirds of one side of the tongue; it
ferre, to bear) also may result from *head injury if the
Afferent pupillary defect (Marcus Gunn first (olfactory) cranial nerve is severed.
pupil) Pupillary dilation associated Drugs are also a common cause of ageu-
with a prechiasmic optic pathway lesion sia; when it associated with medica-
(e.g., optic neuritis) elicited by shining a tions, bitter taste is the first to be
light in the damaged eye after first shin- ­affected and is the last to come back.
ing it in the normal eye. A *Marcus The taste of sweet is affected later and is the

10
AgitationAgonist

first to return. (Greek a‑, without; gueu- *efficacy, producing a full response while
sis, taste) occupying a relatively low proportion of
Agitation A state characterized by ex- receptors. A partial agonist has lower ef-
treme restlessness and heightened arousal. ficacy than a full agonist. It produces
It may involve wringing of the hands, submaximal activation even when occu-
pacing, and poor emotional control, in- pying the total receptor population;
cluding increased irritability and crying therefore, it cannot produce the max-
or laughing without apparent cause. Ag- imal response irrespective of the concen-
itation may be associated with psychiatric tration applied. An inverse agonist produces
disease, such as schizophrenia, or with an effect opposite to that of an agonist
neurological conditions. In neurological yet binds to the same receptor binding
patients, agitation is common following site as an agonist. (Latin agonista, com-
acute lesions and with diffuse processes petitor; from Greek agonistes, contention,
that are at least moderately severe (e.g., from agon, contest)
*traumatic brain injury, *Alzheimer dis- Lissauer, H. (1890). Ein Fall von Seelenblindheit
ease). (Latin agitatio, past participle of nebst einem Beitrage zur Theorie derselben.
agitare, to put in motion, from agere, to Archiv fur Psychiatrie und Nervenkrankheiten,
21, 222–270.
move)
Agnosia Impaired recognition of previ- • Anosognosia Originally used to de-
ously meaningful stimuli that cannot be scribe unawareness of *hemiplegia
attributed to primary sensory defects, following nondominant (right) hemi-
attentional disturbance, or a naming sphere injury as part of the acute *ne-
­disorder. Agnosia was described by Hans- glect syndrome, anosognosia is now
Lukas Teuber (American neuropsychol- used more broadly for commonly oc-
ogist born in Germany, 1916–1977) as a curring unawareness of cognitive, lin-
“normal percept stripped of its meaning.” guistic, sensory, and motor deficits after
It can theoretically be present in each focal injures (e.g., *Wernicke aphasia)
sensory modality, although clinically the or conditions affecting the brain more
most common agnosias are visual and diffusely (e.g., *traumatic brain injury
auditory. In his early descriptions of ag- and *dementia). Anosognosia consist-
nosia, Henirich Lissauer (German neu- ing of denial of blindness is a cardinal
rologist, 1861–1891) hypothesized that feature of Anton syndrome. The term was
visual recognition could be disrupted introduced by Joseph François Felix
either during visual perception (apper- Babinski (French neurologist, 1857–
ceptive agnosia) or during the process of 1932). (Greek, a‑, without; nosos, disease;
associating the precept with meaning gnosis, knowledge)
(associative agnosia). Although studies • Apperceptive agnosia Form of visual
have demonstrated that Lissauer’s de- agnosia in which the deficit is presumed
scription is not fully consistent with ag- to lie in the production of a stable per-
nosia (e.g., associative agnosia may have cept arising from impaired visual per-
some perceptual difficulty), it continues ception. Patients have relatively intact
to accurately characterize stages at which acuity and other elementary visual
the deficits are most pronounced. (Greek functions but have impaired shape
a-, without; gnosis, knowledge) and object recognition. Recognition
Agonist A drug that binds to and acti- improves if the stimulus is in motion.
vates a receptor. A full agonist has high Associated lesions are typically diffuse.

11
AgonistAgonist

(Latin ad, to; percipere, to perceive; a-, perceptual-discriminative impairments

without; gnosis, knowledge) associated with right hemisphere le-
• Associative agnosia Visual agnosia sions or semantic-associative impair-
arising from disruption at the post- ment associated with posterior left
perceptual stage of visual processing hemisphere damage and aphasia have
in which meaning is attributed. Per- been described.
ception is intact, as demonstrated by • Autotopagnosia Disturbed body
the ability to draw the object, but it schema involving an inability to iden-
cannot be associated with semantic tify the parts of one’s body, either to
information for identification. Object verbal command or by imitation. Gross
recognition is demonstrated through autotopagnosia is rare and is not ob-
other sensory modalities. Prosopag- served in isolation. Limited autotop-
nosia (see below) is a specialized form agnosia includes impaired left-right
of associative agnosia. discrimination and finger agnosia (see
• Astereognosis Impaired ability to below). (Greek autos, self, topos; a place,
discriminate objects based on the gnosis, knowledge)
physical characteristics of size, weight, • Color agnosia Inability to recognize
shape, density, or textural cues. Aste- colors despite intact color discrimina-
reognosis is classically associated with tion ability. The impairment is dis-
postcentral lesions. The term has been tinct from *color anomia, which is an
operationally defined in many ways, inability to provide color names with
the most common being the loss of normal color perception, and from
tactile object recognition in the ab- *achromatopsia, which is an impair-
sence of anesthesia. Some have defined ment in color perception. Associated
astereognosis as any disturbance in lesions are typically in the left or bilat-
object recognition, perception, or dis- eral occipital-temporal areas. The term
crimination. Astereognosis is usually “color agnosia” has been applied to dif-
unilateral and, in this respect, differs ferent phenomena in the literature,
from tactile agnosia, which is a higher however, and it should be employed
order impairment of tactile recognition cautiously given the lack of a standard
that is present bilaterally. No study has definition and usage.
demonstrated the existence of tactile • Finger agnosia Bilateral loss of the
agnosia in the absence of anesthesia. ability to name or identify the fingers.
(Greek a-, without; stereos, solid; gnosis, The concept of finger agnosia is linked
knowledge) to that of the *Gerstmann syndrome,
• Auditory agnosia Impaired ability to in which specific disorders of body
recognize sounds despite normal audi- schema are postulated to occur following
tory function. Two types of auditory left *inferior parietal lobe/angular *gyrus
agnosia have been described: auditory lesions. Failure on tests of finger rec-
sound agnosia and pure word deafness. ognition and finger localization may
• Auditory sound agnosia Inability to depend on the specific demands of the
recognize meaningful nonspeech au- tasks employed (e.g., aphasic misnam-
ditory stimuli such as environmental ing, sensory deficit, spatial *disorienta-
sounds (e.g., train whistle). It occurs tion, attentional disturbance). Finger
more frequently than pure word agnosia may be considered a minor form
deafness. Subtypes consisting of either of autotopagnosia (see above).

12
AgonistAgraphia

• Mirror agnosia Condition described tegrate the meaning of elements of a

in patients with right hemisphere picture was interpreted by Gestalt
strokes and left *visual neglect in psychologists as a failure of “simulta-
which, when looking at reflected ob- neous synthesis,” although a defect in
jects from the left (neglected) *visual ocular scanning has been suggested as
field from a mirror positioned in the a more parsimonious explanation for
good right visual field, they are unable this phenomenon. (Latin simul, at the
to locate objects. Patients reach toward same time; Greek a-, without; gnosis,
the virtual object in the mirror or grope knowledge)
behind the mirror attempting to grab • Tactile agnosia Inability to recognize
the reflection but do not reach for the objects by touch in the absence of an-
real object on the left despite being esthesia. Patients can describe object
aware that they are looking into a qualities such as shape or texture but
mirror. cannot recognize them by touch (see
Ramachandran, V. S., Altschuler, E. L., & Astereognosis). Tactile agnosia is a uni-
Hillyer, S. (1997). Mirror agnosia. Proceedings lateral disorder affecting only a single
Biological Sciences of the Royal Society of hand. Associated lesions are generally
London, 264(1382), 645–647. doi: 10.1098/
rspb.1997.0091
in the inferior *parietal lobe (Brod-
mann areas 39 and 40) and may in-
• Prosopagnosia Inability to recognize clude the posterior *insula.
familiar faces that is unrelated to a pri- • Visual agnosia Impaired ability to
mary visual disturbance. Familiar per- recognize visual information. See also
sons are recognized from their speech, Apperceptive visual agnosia, Associative
articles of clothing, or gait. The recog- agnosia.
nition difficulty also often includes Agrammatism A disturbance of the
familiar buildings, landscapes, and au- production or comprehension of gram-
tomobiles, and a patient may not recog- matical structures such as *function words
nize his or her own face. Color agnosia (e.g., articles, prepositions, auxiliary
(see above) or *achromatopsia is fre- verbs) and word endings (e.g., -ed, -ing).
quently associated with prosopag- Although *language does not adhere to
nosia. A left upper *quadrantanopsia conventional rules of *grammar, meaning
is commonly present. Lesions are typ- is generally preserved due to the pres-
ically bilateral infarctions in the territory ence of substantive words with high in-
of the posterior cerebral artery involv- formation content. Agrammatism is
ing the inferior occipital-temporal also called telegraphic speech (or tele-
junction or inferior parietal-occipital grammatism) due to the absence of syn-
regions. Reports of prosopagnosia as- tactical modifiers and the relative preser-
sociated with unilateral lesions have vation of nouns, verbs, or substantive
been published, however, with lesions words, a pattern that was characteristic
typically being in the right hemisphere. of telegrams for which charges were
(Greek prosopon, face; Greek a-, without; based on the number of words trans-
gnosis, knowledge) mitted. (Greek a‑, without; gramma,
• Simultanagnosia Impaired recogni- letter, from graphein, to write)
tion of the meaning of a whole pic- Agraphia An acquired difficulty in
ture or object, with preserved ability writing or spelling. Agraphia is com-
to describe its parts. The failure to in- monly associated with *aphasia and

13
AgraphiaAgraphia

*alexia, although it may occur without • Phonological agraphia A syndrome

other linguistic impairments. When seen that reflects an impairment of the
in isolation, agraphia typically results from nonlexical spelling route; that is, an
lesions of superior *parietal lobe or the impairment of spelling via sound-to-
second frontal *gyrus of the language- letter correspondences. The syndrome
dominant hemisphere. Specific agraphia is characterized by markedly impaired
subtypes (e.g., apraxic, constructional, spelling of nonwords and unfamiliar
surface, and deep) have been distinguished words but relatively spared spelling of
depending on the motor mechanisms real words. In many cases, some im-
(writing disorder) and various linguistic pairment of the lexical spelling route
features (spelling disorders) involved. is evident so that effects of word class,
(Greek a-, without; graphein, to write) frequency, and imagery are noted (as
Spelling Disorders described in deep agraphia); however,
• Deep agraphia A syndrome that is semantic errors are not associated
similar to phonological agraphia in with phonological agraphia.
that there is an impairment of the • Semantic agraphia Loss of the
nonlexical spelling route so that non- ability to incorporate meaning into
words and unfamiliar words are mis- spelling and writing. Semantically in-
spelled. There is also damage to the correct but correctly spelled dictated
lexical spelling route, however, thus *homophones (e.g., “doe” for “dough”)
resulting in semantic errors in writing. may be written. Irregular words and
Spelling is strongly influenced by nonwords are written correctly, how-
word class (*content words are easier ever, thus demonstrating intact lexical-
to spell than *function words), fre- and phoneme-to-*grapheme con­
quency (high-frequency words are easier version.
to spell than low-frequency words), and • Surface agraphia Spelling disorder
imagery/concreteness (high-imagery in which sublexical spelling-to-sound
words are easier to spell than low-imag- correspondences are used to assemble
ery words). the spelling of the word. Words may
• Jargon agraphia Agraphia character- be phonetically plausible but mis-
ized by senseless combinations of let- spelled. The reverse phenomenon is
ters or words. phonological agraphia.
• Lexical agraphia A selective impair- • Verbal agraphia Inability to com-
ment of the lexical (or whole-word) bine single letters into words despite
spelling route that results in an over- the ability to write individual letters.
reliance on spelling by sound-to-letter Writing Disorders
correspondences. The disorder is char- • Afferent agraphia Writing impair-
acterized by impaired ability to spell ment associated with impaired sensory
orthographically irregular or ambig- feedback involving stroke or *grapheme
uous words, with preserved ability duplication or omission.
to spell orthographically regular words • Allographic agraphia Writing im-
and nonwords. Spelling errors typi- pairment associated with poor written
cally include phonologically plausible production characterized by frequent
attempts (e.g., “manshun” for “man- omission errors with well-formed letter
sion”). Also called surface agraphia production. Although well-formed,
(see below). the letter production may ­reflect the

14
Agyria Akinetopsia (motion blindness)

wrong letter. Copying and oral spelling difficulty performing appropriate motor
are spared. sequences, but instead is a deficit in the
• Apraxic agraphia Writing impair- initiation of movement (i.e., a disorder of
ment characterized by poor letter for- motor intention). *Parkinsonian aki-
mation. Although copying is slavishly nesia is associated with decreased arm
produced, oral spelling may be spared. swing while walking and with a “masked
It results from impaired ability to pro- face” (amimia or reptilian stare). More
gram the necessary motor movements complete akinesia is seen with bilateral
for writing. supplementary motor area and anterior
• Peripheral agraphia Writing impair- *cingulate gyrus lesions. (Greek a-,
ment characterized by distortion of without; kinesis, movement)
written production reflecting motor/ Akinetic mutism A state associated with
sensory system deficits. decreased limb movements and absent
• Spatial agraphia Writing impair- speech with preserved *eye movements.
ment due to spatial deficits that affect Akinetic mutism is associated with large
nonlinguistic aspects of writing. The medial *frontal lobe or basal *forebrain
term has been applied to a wide va- tumors or ruptured aneurysms of the
riety of phenomena such as writing anterior cerebral artery. It may also occur
on a slant; uneven spacing of letters, after thalamic injury. In contrast to
words, and blank spaces; ignoring the *locked-in syndrome, in which commu-
left side of a blank page when writing; nication can be established with eye
writing over other words; and dupli- movements, *cognition is not easily dem-
cating strokes. onstrated in akinetic mutism because
Agyria See Lissencephaly. patients do not respond to requests or
AIDS See Acquired immune deficiency other stimuli.
syndrome. Akinetopsia (motion blindness) The
AIDS dementia See Dementia. inability to perceive motion despite
Akathisia (restless leg syndrome) Inner intact object perception. The pure dis-
restlessness and continual leg movement order is considered extremely rare and
associated with *parkinsonism character- may be associated with damage to lateral
ized by tingling, creepy-crawly, or aching occipitoparietal structures. Transitory aki-
sensation in the legs and sometimes netopsia can be induced by *transcranial
arms, as well as motor restlessness that magnetic stimulation (TMS) to area V5,
tends to occur at rest (especially around thought to be essential for perception of
the patient’s usual bedtime). Patients feel coherent motion. Affected individuals
compelled to walk or to pace, which pro- perceive flowing liquids as frozen, and
vides temporary relief. Akathisia may moving objects appear to them to leap
occasionally occur following medication unpredictably from one location to an-
reduction in the treatment of parkin- other. Akinetopsia is associated with bi-
sonism. It is also associated with neuro- lateral visual association cortex lesions
leptic therapy and may decrease over time involving posterior portions of the *in-
after the dosage is reduced. (Greek a‑, ferior temporal gyrus. Akinetopsia has
without; kathısma, seat) also been described in posterior cortical
Akinesia Decreased movement that atrophy and visual variant *Alzheimer
occurs in the absence of *paralysis. Unlike disease. (Greek a-, without; kinesiss
*apraxia, akinesia is not associated with motion; oran, to see)

15
Alcohol withdrawal syndrome Alexia/dyslexia, acquired

Alcohol withdrawal syndrome A syn- slower, lengthier course (named for

drome of agitation, *disorientation, and W. Stewart Alexander, 20th-century
*hallucinations that may be seen in alco- New Zealand pathologist).
holics following alcohol withdrawal. Alexia/dyslexia, acquired Loss or im-
Symptoms usually begin 1–3 days after pairment of the ability to read caused by
the last drink and may last as long as 1 cerebral injury or illness (for develop-
week. Withdrawal seizures are a compli- mental reading disorders see Dyslexia).
cation occurring within the first 48 Acquired dyslexias are typically observed
hours of cessation. *Delirium tremens as manifestations of aphasia, occurring
(DT) is the most severe manifestation of with other *language deficits such as
alcohol withdrawal, which occurs in ap- agraphia and impaired comprehension
proximately 5–20% of patients experi- and expression. Cases of relatively pure
encing detoxification. *Benzodiazepines alexia occur and have contributed to
are commonly used to treat alcohol theories of language processing and the
withdrawal. cerebral localization of language. Ac-
Alcoholic cerebellar degeneration quired dyslexias are categorized based
Cerebellar impairment associated with on clinical presentation, psycholin-
chronic alcoholism, now thought to be guistic features, neuroanatomical sub-
caused by *thiamine deficiency, and pro- strate, or historical convention. (Greek
ducing a wide-based stance and gait, a‑, without; lexis, word, from legein, to
varying degrees of truncal instability, speak, to say)
and *ataxia of the legs. *Nystagmus and • Alexia with agraphia Loss of the
*dysarthria may not be present. Patholog- ability to read and write frequently as-
ical features consist of severe degenera- sociated with left *parietal lobe (angular
tion of the cerebellum, most prominent *gyrus) lesions. Informally referred to
in the anterior and superior *vermis. In by some as “acquired illiteracy.” It was
most cases, alcoholic cerebellar degener- first described in 1891.
ation develops subacutely over several Dejerine, J. J. (1891). Sur un cas de cécité
weeks or months but may improve with verbale avec agraphie, suivi d’autopsie.
cessation of alcohol intake and with thi- Comptes Rendus Hebdomadaire des Séances
et Mémoires de la Société de Biologie, 43,
amine therapy. 197–201.
Harper, C. (2009). The neuropathology of
alcohol-related brain damage. Alcohol and • Alexia without agraphia An ac-
Alcoholism, 44(2), 136–140. doi: 10.1093/ quired inability to read without a cor-
alcalc/agn102 responding deficit in writing. Patients
Alcoholic dementia See Wernicke-­ are unable to read their own written
Korsakoff syndrome. words. It is generally associated with
Alcoholic Korsakoff syndrome See Kor- large ischemic lesions in the posterior
sakoff psychosis/syndrome. cerebral artery territory of the *lan-
Alexander disease A neurodegenera- guage-dominant hemisphere that af-
tive *leukodystrophy of childhood re- fects both the left visual cortex and
sulting from a genetic disorder affecting the posterior *corpus callosum. Visual
the *midbrain and *cerebellum. Most information cannot be received by the
children with the infantile form do not damaged left occipital cortex. Because
survive past 6 years of age. Juvenile and of the corpus callosum lesion, visual
adult-onset forms of the disorder have a input to the right posterior cortex

16
Alexia/dyslexia, acquired Alexia/dyslexia, acquired

cannot be transmitted to the left peri- • Literal alexia Reading disorder char-
sylvian language areas (e.g., angular acterized by an inability to recognize
*gyrus) involved in decoding written written letters.
language. Although written language • Neglect dyslexia Reading disorder
cannot be decoded, it can be generated characterized by failure to identify the
because language and motor areas are initial or left-most portion of a string
unaffected. This syndrome was origi- of letters or words in text. Omissions
nally described in 1892. Also called and substitutions that preserve the
pure alexia, *Dejerine syndrome. overall length of the target are common
Dejerine, J. J. (1892). Contribution à l’étude errors. Errors can also be influenced
anatomoclinique et cliniques des différentes by the lexical information contained
variétés de cécité verbale. Comptes Rendus in the stimulus, suggesting a root at-
Hebdomadaire des Séances et Mémoires de la
Société de Biologie, 44, 61–90.
tentional disturbance at a relatively
high level of representation. Although
• Aphasic alexia A reading disorder
seen most commonly in patients with
associated with aphasia in which read-
left neglect, a few cases with right-
ing comprehension and oral reading
sided neglect have also been described.
are both impaired.
• Peripheral dyslexia Reading disorder
• Attentional dyslexia Reading dis-
caused by visual processing deficits in
order characterized by gross distur-
which visual inputs cannot be associ-
bance in reading multiple words or
ated with the stored representations of
text. Single-word reading is relatively
written words. They include attentional
preserved. Associated with distur-
and neglect dyslexias and alexia without
bances of visual attention.
agraphia. Term suggested by Shallice
• Central dyslexias Acquired reading
and Warrington (1980). Contrast with
disorders that affect processes by
central dyslexias.
which word forms activate meaning
Shallice, T., & Warrington, E. K. (1980). Single
or speech production mechanisms and multiple component central dyslexic
(“higher” language processes). They syndromes. In M. Coltheart, K. Patterson, &
include deep, phonological, and surface J. C. Marshall (Eds.), Deep dyslexia. London:
dyslexias, and alexia with agraphia. Routledge.
Contrast with peripheral dyslexias. • Phonological alexia Reading dis-
• Deep dyslexia Reading disorder af- order resulting from a selective impair-
fecting both the lexical (whole-word) ment in the decoding of print to sound.
and nonlexical (reading by letter-to- Because words cannot be sounded out,
sound correspondences) reading routes. they must be read as whole units. Thus,
Deep dyslexia is characterized by se- unfamiliar or nonsense words cannot
mantic paralexias (e.g., reading “lib- be read, but recognition and pronun-
erty” for “freedom”) and the effects of ciation of familiar words is intact or
word class (*content words read better only mildly impaired. Reading errors
than *function words), frequency tend to involve visually similar words
(high-frequency words read better than (e.g., father for further). This may be
low-frequency words), and imagery an isolated *language impairment or
(high-imagery words read better than may be associated with *fluent aphasia.
low-imagery words). Nonword reading Anatomic correlations have been in-
is severely impaired. consistent, although most cases include

17
AlexithymiaAllesthesia

damage to superior temporal cortex Todd’s initial description and the experi-
and angular *gyrus of the left hemi- ence of the protagonist in Lewis Carol’s
sphere. Contrast with surface dyslexia. Alice’s Adventures in Wonderland (1865).
• Pure alexia See Alexia without Common etiologies include viral infec-
agraphia. tions, particularly *Epstein-Barr virus,
• Semantic alexia Term describing and *migraine.
surface alexia when it develops in *de- Todd, J. (1955). The syndrome of Alice in
mentia. The term semantic alexia is Wonderland. Canadian Medical Association
used because stored representations of Journal, 73(9), 701–704.
unusually pronounced words that re- Alien hand syndrome Condition in
flect semantic knowledge are pre- which one hand, typically the left, either
sumedly lost. acts under its own volitional control or is
• Surface alexia Reading disorder in perceived by the patient to be “foreign,”
which words are read and understood “alien,” or “uncooperative.” Alien hand
only inasmuch as they can be “sounded syndrome may result from large medial
out.” Thus, in a pattern directly oppo- *frontal lobe lesions, *commissurotomy
site to that of phonological dyslexia, (also called corpus callosotomy) or other
nonsense words are read accurately, as lesions of the *corpus callosum, or corti-
are words with phonetically regular cobasal degeneration. In commissurot-
spelling. Irregularly spelled words, omy patients, corpus callosotomy alone
however, are misread as regularly spelled without additional cerebral involvement
ones (regularization errors; e.g., “busy” is considered insufficient to cause this
read as “buzzy”). A variety of cerebral syndrome. The behavior of the alien
lesions have been implicated; most cases hand following *callosotomy may dem-
have included injury to left temporal- onstrate hemispheric specialization, with
parietal cortex. an alien left hand correcting incorrect
Alexithymia Difficulty in recognizing right-hand response on verbal neuropsy-
and describing one’s emotions. Persons chological tasks.
with alexithymia may also have diffi- Loring, D. W., Meador, K. J., & Lee, G. P.
culty distinguishing between feelings (1989). Differential-handed response to verbal
and the bodily sensations that accom- and visual spatial stimuli: Evidence of
pany states of emotional arousal. (Greek specialized hemispheric processing following
callosotomy. Neuropsychologia, 27(6), 811–827.
aleksomai, to repulse; thymos, affect)
Alice-in-Wonderland syndrome A con- Allele An alternate form of a gene that is
dition originally characterized by Todd located on a specific locus on a specific
(1955) as self-experienced paroxysmal chromosome. Because each individual
image distortions of body size, body inherits two copies of each chromosome
mass, body shape, or body position in (one from each parent), two alleles for
space that is often associated with deper- each gene are inherited. There can be
sonalization and derealization. The term multiple alleles for a particular trait. If
Alice-in-Wonderland syndrome has sub- both alleles are the same, the organism is
sequently been applied more broadly to homozygous at that locus. If the alleles
visual distortions, including *micropsia are different, the organism is heterozy-
(objects appear smaller) and *macropsia gous at that locus.
(objects appear bigger) without self-­ Allesthesia A condition in which the
perceived body distortion, which differs from sensation of touch *contralateral to the

18
Allocentric Allographic store/allographic errors

lesion is referred to the analogous location Allochiria Sensation of touch *contra-

on the (opposite) side *ipsilateral to the lateral to the lesion that is referred to the
lesion. This phenomenon, however, is analogous location on the (opposite)
more accurately termed allochiria. A more side *ipsilateral to the lesion. Allochiria
literal interpretation of allesthesia based was first described with tactile stimuli
on the term’s Greek origins is the sensa- but may also occur with visual, auditory,
tion of touch being experienced at a point or olfactory stimuli. It is typically associ-
remote from the point touched. Allesthe- ated with right hemisphere lesions and
sia is typically associated with right hemi- *hemispatial neglect. (Greek àllos, other;
sphere lesions as part of the *neglect syn- cheir, hand)
drome. (Greek allaché, elsewhere; aisthésis, Obersteiner, H. (1881). On allochiria: A
perception) peculiar sensory disorder. Brain, 4(2), 153–
Meador, K. J., Allen, M. E., Adams, R. J., & 163. doi: 10.1093/brain/4.2.153
Loring, D. W. (1991). Allochiria vs. Allocortex Phylogenetically older *cere-
allesthesia. Is there a misperception? Archives
of Neurology, 48(5), 546–549. doi: 10.1001/
bral cortex consisting of three cytoarchitec-
archneur.1991.00530170110029 tural layers. Allocortex includes paleocortex
(olfactory cortex) and archicortex (*limbic
Allocentric *Encoding spatial informa-
system; primarily *hippocampus and den-
tion relative to the location of other ob-
tate *gyrus within the *temporal lobe)
jects rather than in relation to one’s own
and contrasts with the neocortex.
body. Left allocentric neglect is associ-
Allodynia Painful sensation in response to
ated with lesions to the right posterior
normally nonpainful stimuli such as wear­
superior temporal sulcus, angular, and
ing clothing or in response to the wind.
middle temporal/inferior temporal and
Allodynia is a common component of
middle occipital gyri. Contrasts with
central poststroke pain and may vary as a
*egocentric. (Greek àllos, other)
function of lesion location within the thal-
Chechlacz, M., Rotshtein, P., Roberts, K. L.,
Bickerton, W. L., Lau, J. K., & Humphreys, amus (i.e., cold allodynia is common with
G. W. (2012). The prognosis of allocentric dorsal lesions; movement allodynia is
and egocentric neglect: Evidence from more common with rostral-dorsal area le-
clinical scans. PLoS ONE, 7(11), e47821. doi: sions). (Greek àllos, other + -odine, pain)
10.1371/journal.pone.0047821 Allographic store/allographic errors
Allocentric navigation A cognitive strat­ The theoretical representation of the ap-
egy for solving complex navigation tasks propriate letter form (e.g., case: upper
requiring enduring representations of the vs. lower, or font: cursive vs. print) in-
locations of multiple objects in relation to volved in writing. Allographic agraphia
each other. This “object-to-object” strategy errors are reflected by an inability to
relies more on a cognitive spatial map spell in some selected modalities (case or
than on visible cues, thus providing an font) but not in others. Some patients
advantage in large and unfamiliar envi- exhibit a disturbance in their ability to
ronments. The use of allocentric strategies control the case of words, manifested in
during navigation activates regions of their writing—in mIXeD CasES. Others
the *hippocampus and parahippocampus. may be selectively unable to write in one
Deterioration of allocentric processing is specific font or case but not another. Al-
thought to contribute more generally to lographic agraphia has been reported
navigation decline in normal aging and in patients with *dementia or *encepha-
*dementia. litis. (Greek àllos, other; graphein, to write,

19
Allokinesia Alzheimer disease (AD)

a representation, from graphein, to write, ALS See Amyotrophic lateral sclerosis.

draw) Alternate forms See Parallel tests.
Allokinesia Limb movement that occurs Alternating sequences test A test of
*contralateral to the side of intended *frontal lobe function involving the
movement. Allokinesia is typically asso- changing from one action to the next in
ciated with right hemisphere lesions and an alternating fashion. See Luria figures,
seen as part of the *neglect syndrome. Fist-edge-palm test.
(Greek àllos, other; kinesis, movement) Alveus A layer of *white matter on the
Allosteric modulator A drug that binds to ventricular surface of the *hippocampus
a receptor at a site distinct from the active that primarily conveys *efferents from
site, thus inducing a conformational change the *hippocampus to other cortical
in the receptor that alters the affinity of the structures. (Latin alvus, belly, hollow)
receptor for the endogenous ligand. Posi- Alzheimer disease (AD) A neurodegen-
tive allosteric modulators increase the af- erative disease with characteristic pa-
finity whereas negative allosteric modulators thology of neurofibrillary tangles and
decrease the affinity of the ligand for the senile amyloid plaques. AD is estimated
receptor. (Greek àllos, other; steros, solid) to affect 1.2–5.0% of the population
Alpha The first letter of the Greek alpha- older than age 65, and its prevalence rises
bet, α 1. The probability of type I error in steeply with each decade (e.g., 30% by
making a decision about the tenability of age 80–90). The clinical diagnosis is
a null hypothesis (e.g., p < .05 when α = based on history, exam findings, and
.05). 2. The *electroencephalogram (EEG) other supportive studies, with patients
frequency band of 8–13 Hz that occurs demonstrating a progressive loss of cog-
maximally over posterior brain regions. nitive ability that interferes with social
Also called the α rhythm. 3. A measure of a functioning. Neuropathological analysis
test’s reliability (*coefficient alpha) that re- is necessary for a definite diagnosis, al-
flects the *internal consistency of an item. though biomarkers are increasingly being
Alpha coma A *coma accompanied by used, including *cerebrospinal fluid (CSF)
an *electroencephalographic (EEG) pat- assays of Aß1-42 and phosphorylated
tern containing primarily alpha (α) oscil- tau, amyloid *positron emission tomog-
lations in the 8–13 Hz *frequency range. raphy (PET) imaging, and volumetric
Alpha coma is most commonly observed *magnetic resonance imaging (MRI). Neu-
in anoxic *encephalopathy, toxic *en- ropsychological assessment may be im-
cephalopathy, or locked-in state. portant for identifying cognitive changes
Alpha-synuclein A presynaptic neuronal in the early disease stages, as in *mild
protein that, when aggregated, is linked cognitive impairment (MCI), or for iden-
genetically and neuropathologically with tifying other areas of neuropsychological
neurodegenerative diseases such as *Par- difficulty that may not be easily recog-
kinson disease, *dementia with *Lewy nized with a bedside examination. About
bodies, and multisystem atrophy. The 5% of early-onset AD (i.e., before age 60)
demonstration of α-synuclein pathology is caused by autosomal dominant muta-
as a major constituent of Lewy bodies tions in one of three genes (APP, PS1,
has provided the rationale for developing PS2). There are also AD susceptibility
medications to treat Parkinson disease, genes, such as the apolipoprotein E4
by reducing α-synuclein gene expression allele (APOE4). Patients without a family
or blocking its aggregation. history of AD have approximately a 15%

20
Alzheimer Disease Assessment Scale (ADAS) Ammon horn

lifetime risk of the disease (named for summary, for example, to document
Alois Alzheimer, German neuropatholo- that the discharge was neither ordered
gist, 1864–1915). nor approved by the attending physi-
Alzheimer Disease Assessment Scale cian. 2. American Medical Association.
(ADAS) A combined *mental status Amacrine Amacrine cells are *interneu-
examination and *dementia rating scale rons in the retina that synapse onto ret-
for *Alzheimer disease. The scale has two inal ganglion cells. Located in the inner
parts, one for cognitive (ADAS-Cog) plexiform layer of the retina, which is the
and the other for noncognitive function. second synaptic retinal layer in which bi-
The cognitive functions sampled include polar cells and retinal ganglion cells form
*memory, *language, and praxis, and the synapses.
noncognitive functions include *mood Amantadine An antiviral medication
and behavioral changes. The ADAS-Cog that has neurological application as ad-
is a commonly employed instrument in junctive therapy for the treatment of
pharmaceutical research. *Parkinson disease with effects on *resting
Rosen, W. G., Mohs, R. C., & Davis, K. L. tremor, *rigidity, and *akinesia. Amanta-
(1984). A new rating scale for Alzheimer dine, due to it mild stimulant effect, is
disease. American Journal of Psychiatry, 141, occasionally used to treat fatigue in *mul-
1356–1364.
tiple sclerosis, but with limited *efficacy.
Alzheimer’s Disease Neuroimaging Initi- Amaurosis fugax Transient monocular
ative (ADNI) Ongoing, longitudinal blindness resulting from a *transient is-
multicenter study to validate clinical and chemic attack (TIA) of the ophthalmic
cognitive data, serum plasma, *magnetic artery. It is described by patients as a
resonance imaging (MRI) brain scans, blanket of gray coming down slowly over
*positron emission tomography (PET) one eye. Amaurosis fugax episodes indi-
scans using the Pittsburgh Compound-B cate underlying atherosclerotic disease
to detect β-amyloid, and *cerebrospinal with an increased risk for *stroke and
fluid (CSF) biomarkers in older adults myocardial infarction. (Greek mauros,
with normal *cognition, *mild cognitive black; amaurosis, making something
impairment (MCI), or *Alzheimer disease black; Latin fugare, to cause to flee, chase)
(AD). More recently, ADNI included re- Ambidextrous Having the ability to use
cruitment of patients with early MCI either left or right hand for performing
(eMCI; *memory complaints but no ob- manual tasks. (Greek amphi, both; Latin
jective findings) and Amyvid scans to dexter, the right hand)
detect β-amyloid plaques. Standardized Ambient cistern The *cerebrospinal
protocols across sites allow investigators to fluid–filled subarachnoid cistern that par-
share data to identify preclinical changes tially encircles the posterior-lateral portion
and predictors of conversion from normal of the *midbrain. It is lateral extension of
aging to MCI and AD. The ultimate objec- the superior or quadrigeminal cistern.
tive is to validate biomarkers to monitor Amimia Inability to express ideas through
disease progression and clinical treatment gestures. This term is occasionally used to
*efficacy (http://adni.loni.ucla.edu). described the loss of facial expressiveness
Alzheimer’s Quick Test (AQT) See A associated with *parkinsonism. (Greek
Quick Test of Cognitive Speed. a-, without; mimos, a mimic)
AMA 1. Against medical advice. This no- Ammon horn Portion of the *hippo-
tation is made in a patient’s discharge campus that is a common site for scar

21
Amnestic syndrome (amnesia) Amyloid angiopathy (cerebral amyloid angiopathy)

tissue formation (i.e., sclerosis or *glio- tactile *agnosia but also visual-spatial dif-
sis), which often gives rise to *temporal ficulties, *neglect, and *dressing apraxia.
lobe seizures. Ammon is an Egyptian This is an old heuristic concept that is not
god who was depicted as a ram, and this currently used. (Greek a-, without, morphē,
area is so named because of its resem- form, synthesis, composition)
blance to a ram’s horn. AMPA receptor (alpha-amino-3-hy-
Amnestic syndrome (amnesia) Severe droxyl-5-methyl-4-isoxazole-propio-
impairment in the ability to acquire and nate) One of two types of *glutamate
retain new information with otherwise receptors. Synapse structure and AMPA
preserved cognitive function. Common receptor function are altered in many neu-
lesion sites include the *hippocampus, rological diseases. AMPA receptors allow
hippocampal projections to the *fornix cytotoxic levels of calcium into neurons,
and septum, and medial thalamic nuclei. leading to motor neuron death in *amy-
Conditions associated with amnesia in- otrophic lateral sclerosis. In some forms
clude *Korsakoff syndrome, *herpes sim- of *epilepsy, overactivation of AMPA
plex encephalitis, posterior cerebral artery receptors leads to neuron damage. In
*stroke, anoxia, trauma, and *transient *ischemia, overactivation of AMPA re-
global amnesia (TGA). Memories that ceptors from oxygen deprivation leads
were encoded well before onset of the to *excitotoxicity. *Alzheimer disease is
amnestic syndrome are relatively well pre- characterized by decreased AMPA acti-
served. In a dimensional model of *cogni- vation and synapse loss.
tion (e.g., the *Cattell-Horn-Carroll Amusia Acquired deficit of music proc-
model), a clinical amnesia can be thought essing. Following right hemisphere le-
of as an extreme variant of the underlying sions, amusia may involve impairment
construct of long-term retrieval. *Func- of melody recognition or difficulty in
tional amnesia, which is a psychiatric dis- identifying musical elements such as
order, usually involves failure to remember rhythm. In left hemisphere lesions, music
autobiographical material, such as one’s reading and music performance may be
name. The ability to learn new informa- poor without impairment of either music
tion, however, is generally unaffected. recognition or the ability to judge music
(Greek mnéme, memory; closely related quality. (Greek a‑, without; Mousa, muse
to Greek amnestia, forgiving (forgetting) a of song)
wrong, amnesty) Amygdala Almond-shaped collection
Amobarbital An intermediate-acting of nuclei in the anterior-*medial tem-
*barbiturate used either as a sedative or poral lobe, just beneath the *uncus, that
to control seizures. It is the most common plays a role in *memory and emotional
agent used in the *Wada test to induce control. The amygdala receives inputs
hemispheric *anesthesia. from all senses as well as visceral input,
Amorphognosia Inability to identify an with important output pathways to
object by *proprioception due to impaired *hippocampus, *entorhinal cortex, and
perception of its physical attributes that *dorsomedial nucleus of the thalamus.
cannot be explained on the basis of con- (Greek amygdale, almond)
current *anesthesia. (Greek a-, without; Amyloid angiopathy (cerebral amyloid
morphē, form, gnosis, knowledge) angiopathy) Amyloid deposition in
Amorphosynthesis Impairment of sen- cerebral and meningeal vessel walls seen
sory integration that involves not only in patients with *dementia, particularly

22
Amyloid hypothesis (of Alzheimer disease) Anagram letters

*Alzheimer disease (AD), but also in mosome 21, best known as the precursor
many individuals without dementia. Amy- molecule that generates β-amyloid pro-
loid angiopathy is associated with hemor- tein (Aβ). Mutations in the APP gene are
rhagic *strokes and is also seen in cognitive associated with early-onset familial forms
impairment and ischemic infarctions. Al- of *Alzheimer disease (AD). Duplica-
though generally unrecognized during tions of APP, as seen in trisomy 21 (*Down
life, the presence of at least some amyloid syndrome), also lead to AD.
angiopathy is often found at autopsy. Amyotrophic lateral sclerosis (ALS) A
Estimates of the prevalence of amyloid progressive motor neuron disease that
angiopathy from autopsy series range ­affects neurons in the *cerebral cortex,
from 10% to 50% in the general elderly *brainstem, and spinal cord. ALS is not
population, to 80% in patients with path- generally seen before 45 years of age. The
ological features of AD. (Amyl- + -oid; disease ends fatally from respiratory com-
from Latin amylum, starch; from Greek plication, usually within 2–4 years of onset.
amulon, starch, the finest flour, from Also called Lou Gehrig’s disease after the
neuter of amulos, “not ground in a mill,” famous New York Yankee baseball player
a-, not + mule, mill; Greek angeion, vessel, who developed the disease when he was
capsule; pathos, disease) 37 years old. Also known as motor neuron
Amyloid hypothesis (of Alzheimer dis- disease, ALS is associated with frontotem-
ease) The hypothesis that the ab- poral cognitive changes in some patients;
normal accumulation of Aβ peptide into these range from mild abnormalities only
amyloid plaques is a critical pathophysi- recognized with formal neuropsychological
ological feature giving rise to a cascade of testing to profound frontotemporal *de-
events that eventually leads to the devel- mentia (FTD). (Greek a-, without; mys,
opment of *Alzheimer disease (AD). The muscle; trephein, to nourish; Latin latus,
hypothesis is bolstered by findings that side; Greek skleros, hard)
genetic mutations in *amyloid precursor Amyvid scan A *positron emission to-
protein (APP), presenilin-1 (PS1), and mography (PET)-based technology used
presenilin 2 (PS2) all lead to autosomal to estimate β-amyloid neuritic plaque
dominant forms of early-onset AD by density in the brain. A radioactive agent
increasing the production of Aβ. These (Amyvid or Florbetapir F18 injection)
findings have provided the theoretical tags amyloid plaques. A positive scan in-
rationale for AD therapeutic develop- dicates the presence of moderate to fre-
ment using agents to reduce Aβ produc- quent plaques but does not establish a
tion or Aβ aggregation. Although the diagnosis of *Alzheimer disease (AD)
abnormal deposition of Aβ is a major because *plaques can be seen in other
feature in the AD brain, the failure of neurological disorders as well as in older
many phase III clinical trials targeting adults with cognitive impairment. The
Aβ has suggested that alternative mecha- scan results are not intended to predict
nisms are also likely contributing. In ad- the development of *dementia or to
dition, the amyloid hypothesis does not monitor therapy responsiveness.
clearly explain how and why tau is ab- ANA Antinuclear antibody. A positive
normally phosphorylated and aggregated ANA suggests collagen vascular disease
in the human AD brain. such as *systemic lupus erythematosus.
Amyloid precursor protein (APP) A Anagram letters Individual movable
transmembrane protein located on chro- letters that are used to test spelling or

23
AnalgesiaAneurysm

orthographic competence without writing. Andermann syndrome An autosomal

(French anagramme, from Late Latin recessive disorder seen primarily in the
anagramma, from Greek ana, back, and French-Canadian population in north-
gramma, from graphein, to write) eastern Quebec and characterized by
Analgesia An absence of pain in re- agenesis of the *corpus callosum, intel-
sponse to painful stimulation. (Greek lectual *disability, sensory-motor neu-
an-, without; algesis, pain) ropathy, and dysmorphic facial features.
Analogy theory Theory proposing that Andermann, E., Andermann, F., Melanee, D.,
the pronunciation of new words and non- Karate, G., & Carpenter S. (1971). Three
words is based on analogies with known familial midline malformation syndromes of
the central nervous system. In J. Grouchy
words. See also Rule-based reading. (Ed.), Proceedings of the Fourth International
Anaphora A linguistic term for a word Congress of Human Genetics. Amsterdam, the
that gains its meaning from another Netherlands: Excerpt Medical International
term in the same or previous sentence. Congress Series; 233, 15.
Anaphoric words maintain cohesion in Anencephaly (aprosencephaly) In
discourse and carry a reference-tracking utero neurodevelopmental defect char-
role. For instance, in the phrase “Mary acterized by a rudimentary *brainstem
did not come to school. She is ill,” the without cerebral and cerebellar hemi-
pronoun she refers to Mary in the pre- spheres. Anencephaly results from a
vious sentence. Compare with *deixis. neural tube defect occurring when the
Anaplasia Loss of tissue differentiation *cephalic end of the *neural tube fails to
that is characteristic of most *malignant close, usually within the first month of
tumors. Well-differentiated tumors re- pregnancy. Most neonates with anen-
semble their tissue of origin, whereas cephaly do not survive birth. (Greek
poorly differentiated or undifferentiated an-, without; enkephalos, brain)
(anaplastic) tumor cells appear primitive Anesthesia Impaired tactile stimulus
and lack specialization along any partic- detection that may result from damage
ular cell line. (Greek ana, again; plathein, to the anterior *parietal lobe, spinal injury,
to mold) or peripheral causes. Anesthesia induced
Anarithmetria See Acalculia. by pharmacological depression of neural
Anarthria Speech impairment due to function is used to minimize or elimi-
bulbar paralysis. This term was originally nate pain associated with medical or
used by Pierre Marie (French neurolo- surgical procedures. (Greek an-, without;
gist, 1853–1940) and was synonymous aisthesis, sensation)
with “pure *motor aphasia” or aphemia. Aneurysm Arterial bulge resulting from
(Greek an‑, without; arthrosis, artic­ a weakened vessel wall. Most aneurysms
ulation) are located in the subarachnoid space
Anastomosis The anatomical connec- and pose a risk of *hemorrhage. A dis-
tion of one set of blood vessels to another. secting aneurysm consists of layers of
Normal brain anastomoses include the the artery separating. (Greek aneurysma,
*Circle of Willis, although pathological from aneurynein, to widen; from ana,
anastomoses (e.g., *fistulas) also occur, throughout; eurunein, to dilate, widen,
and these require interventions such as from eurus, wide)
*embolization or resection. (Greek anas- • Berry aneurysm A sac-like or berry-
tomosis, from anastomoo, to furnish with shaped aneurysm that grows outward
a mouth) from an arterial wall. Intracranial

24
Angiogram Angular gyrus

a­ neurysms are commonly seen at bi- trast medium; this procedure carries a risk
furcations of cerebral vessels (e.g., at of *stroke and reaction to the contrast
the *internal carotid artery–posterior medium. Cerebral angiograms involve
communicating artery junction, the an- catheters inserted through the femoral
terior cerebral artery–anterior commu- artery. *Magnetic resonance angiography
nicating artery junction, or the *middle is a less invasive technique that typically
cerebral artery bifurcation). Ruptured does not require *contrast agents. (Greek
berry aneurysms are the most common angeion, a case, vessel, capsule; graphein,
cause of nontraumatic *subarachnoid to write, draw)
hemorrhage. Because of the risk for Angioma A congenital *vascular mal-
bleeding or rupture, berry aneurysms formation involving blood vessel prolif-
are usually treated surgically. Also called eration that resembles a tumor. Most
saccular aneurysm. angiomas are asymptomatic, and, when
• Fusiform aneurysm A long segment present, symptoms are usually due to *ar-
of arterial dilation. Fusiform aneu- teriovenous malformation (AVM) or cav-
rysms typically develop secondary to ernous angiomas. (Greek angeion, a case,
*atherosclerosis and pose a very low vessel, capsule)
risk of bleeding. Angiotensin-converting enzyme (ACE
• Giant aneurysm An aneurysm that inhibitor) Class of drugs used to treat
has expanded to greater than 25 mm *hypertension, heart failure, and renal
in diameter. Giant aneurysms often disease. ACE inhibitors restrict the ac-
present with *mass effect rather than tivity of angiotensin-converting *enzyme
rupture. (ACE), which is responsible for produc-
• Mycotic aneurysm Dilatation of an ing angiotensin II that causes narrowing
artery secondary to any infectious of blood vessels (vasoconstriction) and
process, usually due to the lodgment release of aldosterone, a substance that
of an infected embolus or from local causes kidneys to retain sodium and
spread of an infection (e.g., *depressed fluid. ACE inhibitors expand blood ves-
skull fracture with local infection). sels, thereby lowering *blood pressure.
Although mycotic refers to a fungal in- Angiotensin receptor blockers (ARBs)
fection, the term was originally ap- A class of antihypertensive medications
plied to describe infectious aneurysms that target the renin angiotensin aldos-
without reference to fungal agents and terone system by blocking the effect of
is retained for historic continuity. angiotensin 2 (Ang II), a mediator of
• Saccular aneurysm See Berry aneu- neurovascular injury.
rysm (P. 24). Angular gyrus Convolution of the infe-
Angiogram Approach to image the cer- rior *parietal lobe, arching over the poste-
vical and cerebrovascular arterial tree. rior end of the superior temporal sulcus
With conventional angiograms, there is and continuous with the *middle temporal
less than a 1% risk of complication when gyrus; Brodmann area 39. Angular *gyrus
performed by interventional neuroradi- lesions in the dominant hemisphere may
ologists. However, conventional angiog- produce various combinations of *Gerst-
raphy permits selective vessel injections. mann syndrome signs, which include
Angiography Radiological technique for agraphia, acalculia, finger agnosia, and
imaging vessels. Angiography may refer right-left *disorientation. *Alexia may
to the invasive procedure using a con- result from angular *gyrus lesions.

25
AnhedoniaAnosmia

Anhedonia The inability to experience Ankle-foot orthosis A brace worn on

pleasure from events that are typically the lower leg and foot to support the
considered enjoyable. Anhedonia is a ankle and correct foot drop.
cardinal feature of affective disorders. Ankle jerk See Achilles tendon reflex.
(Greek an‑, without; hedone, pleasure) Anoetic consciousness One of the
Animal naming (animal fluency) A three *levels of consciousness proposed
generative verbal *fluency task requiring by Endel Tulving (Estonian-Canadian
the generation of as many different ani- cognitive psychologist, b. 1927); other
mals as possible within a 60-second levels include noetic and autonoetic con-
period. Like other generative *verbal flu- sciousness. Anoetic consciousness is a state
ency tests, different versions of animal that is temporally and spatially bound to
naming may employ slightly different the current time but that contains un-
instructions and definitions to charac- thinking and nonreflective forms of expe-
terize which responses are considered to rience, some of which may be affectively
be separate and distinct scorable answers intense without being “known.”
meriting credit. Although all generative Anomia (dysnomia) The impaired ability
fluency tasks can be affected by disrupted to name objects or retrieve words. Anomia
frontal networks, semantic fluency tasks refers to a pathological word-finding dif-
such as animal naming are impacted by ficulty rather than to normal word-find-
anterior temporal lesions, with selective ing difficulties or vocabulary limitations.
impairments described in *Alzheimer Anomia has been historically associated
disease (AD), semantic *dementia, and with lesions of the temporal-parietal
*temporal lobe epilepsy. Consequently, junction but may also be present in sub-
animal naming is more sensitive than cortical lesions, particularly thalamic le-
generative fluency to letter prompts (e.g., sions. Anomia is present to varying de-
Controlled Oral Word Association, aka grees with all forms of *aphasia, is
FAS) to impaired semantic *memory common in *dementia of moderate se-
access in AD, and this performance dis- verity, and, by itself, has little localizing
crepancy may at times have diagnostic significance. Anomia is often assessed
utility for dementia classification. through visual object *confrontation
Animal Sorting A card-sorting task de- naming, although the term is also used to
veloped for use in pediatric neuropsy- characterize word-finding difficulty in
chology that measures concept formation, spontaneous speech. These two types of
ability to apply concepts, and set shifting. word-finding difficulty, however, are dis-
The child is shown eight cards and is sociable. Although the terms anomia and
asked to look for ways that the cards are dysnomia are often used interchangeably,
the same and different. The child is asked dysnomia implies a less marked naming
to generate different ways that the cards impairment than anomia. (Greek a-,
can be sorted into two groups of four. without; onoma, name)
Animal sorting is a *NEPSY-II subtest. Anosmia Impairment in the ability to
Anisocoria Unequal pupil sizes. Although smell. Anosmia is associated with facial
aniscoria may be seen in healthy individu- bone injuries, olfactory nerve degenera-
als, it clinically reflects neurological dis- tion, head trauma, or *frontal lobe tumors.
ease including tumor, *seizure, *meningit Patients with anosmia often state that
is/*encephalitis, and *stroke. (Greek an-, their sense of taste is also diminished, al-
not; iso-, equal; kórē, pupil of the eye) though this is an artifact of the way we

26
Anosodiaphoria  Anterior cerebral artery (ACA)

derive food characteristics from odor. In nuclei of the thalamus and serve a motor
anosmic patients, the ability to detect and control function.
recognize sweet, sour, salt, and bitter is Antagonist A drug that attenuates the
preserved. (Greek a‑, without; osmé, sense effect of an agonist. Antagonists are clas-
of smell) sified as either competitive or noncompet-
Anosodiaphoria Lack of concern for se- itive, each of which can be reversible or
rious neurological impairments without irreversible. A competitive antagonist binds
denying their existence. *La belle indiffer- to the same site as the agonist but does
ence, which also describes a similar absence not activate it, thus blocking the ago-
of concern for sensory or motor deficits, is nist’s action. A noncompetitive antagonist
commonly used in the context of psycho- binds to an allosteric (nonagonist) site
genic impairment (e.g., conversion symp- on the receptor to prevent activation of
tomatology). (Greek a-, without; nósos, the receptor. A reversible antagonist binds
disease; diaphorein, to disperse, dissipate noncovalently to the receptor and there-
by perspiration: dia‑, in different direc- fore can be “washed out.” An irreversible
tions; phorein, from pherein, to carry or antagonist binds covalently to the re-
bear; adiaphoría, lack of interest) ceptor and cannot be displaced by either
Anosognosia See Agnosia. competing ligands or washing.
Anoxia Complete or nearly complete lack Antagonistic pleiotropy A theory of
of oxygen supply to tissue. The most gene expression in which both beneficial
common cause of cerebral anoxia is car- and detrimental traits are conferred. The
diac arrest. Its greatest effect is on the *hip- antagonistic pleiotropy theory of aging
pocampus (Sommer sector), resulting in postulates that some genes that are bene-
anterograde *memory impairment. Anoxia ficial at earlier ages are harmful at later
may also affect the primary visual and ages. A gene that increases survival to re-
visual association cortices, resulting in productive age will be favored by natural
central visual disturbances (e.g., *cor- selection if it decreases the chances of
tical blindness, visual agnosia). Hypoxia dying prior to age 20. Harmful late-act-
refers to lack of oxygen that is not as com- ing genes can remain in a population if
plete. (Greek an-, without; + oxymoron) they have a beneficial effect early in life
Ansa lenticularis A fiber tract that is part (e.g., increasing fitness or increasing re-
of the projection system from the *globus productive success). Natural selection will
pallidus to the *thalamus. The ansa len- frequently maximize vigor in youth at
ticularis has fewer axons than the other the expense of vigor later on and thereby
pallidal-thalamic projection, the *lentic- produce a declining vigor (aging) during
ular fasciculus; together, these form the adult life. (Greek anti-, against + ago-
thalamic *fasciculus. An alternate nomen- nizesthai, to struggle, from agon, contest;
clature for these pathways was developed ple(i)on, more + -trope, change)
by Auguste Henri Forel (Swiss neurolo- Antalgic gait Gait that is used to avoid
gist, 1848–1931), who described the pain on weight-bearing structures such
Haubenfelder fields (H fields) of the sub- as ankle, hip, or knee. A limp is charac-
thalamic tegmentum, which are abbrevi- teristic due to a shortened step associ-
ated as the H1 field of Forel and the H2 ated with the injured side. (Greek anti-,
field of Forel. These basal ganglia output against; algos, pain)
pathways primarily project to the ventral Anterior cerebral artery (ACA) See
anterior (VA) and ventral lateral (VL) Arteries, cerebral.

27
Anterior commissure Anterior perforated substance

Anterior commissure An interhemi- ventral motor neurons. Part of the

spheric fiber bundle near the most ante- medial motor system (along with the
rior portion of the third ventricle that vestibulospinal, reticulospinal, and tecto-
connects olfactory structures and lateral spinal tracts), the anterior corticospinal
parts of the *temporal lobe to each other. tract plays an important role in control
*Commissurotomy as a treatment for of bilateral axial and girdle muscles vital
intractable *epilepsy may, but does not for postural control, proximal move-
always, include transection of the ante- ments, gait, and balance. Selective lesions
rior commissure in addition to the *corpus of the anterior corticospinal tracts rarely
callosum. present with any persistent motor defi-
Anterior communicating artery (ACoA) cits because these tracts provide bilateral
See Arteries, cerebral. innervation to the axial muscles.
Anterior communicating artery syn- Anterior fossa That portion of the cra-
drome Syndrome of dense anterograde nial vault on which the *frontal lobes rest.
amnesia, *disorientation, and *confabu- Anterior horn (of spinal cord) The an-
lation combined with disturbances of terior (ventral) *gray matter portion of
­attention and behavior, including *dis- the spinal cord that contains cell bodies
tractibility, *perseveration, and *utiliza- of α motor neurons, whose axons enter
tion behavior. Motor, sensory, and linguistic the ventral spinal nerve roots and end
functions remain intact. It is associated on skeletal muscle. One of the three gen-
with rupture of anterior communicating eral divisions of the spinal cord gray
artery aneurysms. matter, the others being the posterior
Alexander, M. P., & Freedman, M. (1984). horn and the intermediate gray matter.
Amnesia after anterior communicating Anterior horn cells mediate movement.
artery aneurysm rupture. Neurology, 34(6), Anterior inferior cerebellar artery
752–757. doi: 10.1212/WNL.34.6.752
(AICA) A long branch of the basilar
Anterior cord syndrome Deficits asso- artery arising just above the union of the
ciated with spinal cord injury involving two vertebral arteries on the ventral sur-
damage to the anterior two-thirds of the face of the brainstem. It supplies ante-
spinal cord; often due to occlusion of rior regions of the inferior surface of the
the anterior spinal artery and resulting cerebellum (including the *flocculus)
in bilateral paralysis, bladder dysfunc- and parts of the *caudal *pons.
tion, and loss of pain sensation below Anterior nucleus A thalamic relay nu-
the level of the lesion. cleus located dorsally and anteriorly
Anterior corticospinal tract A motor within the thalamus. *Afferent inputs to
outflow tract that contains approxi- the nucleus are from the mammillary body
mately 15% of the motor fibers traveling and other limbic structures, and *efferent
from cortex to the spinal cortex. These outputs travel to the *cingulate gyrus.
fibers do not cross in the *medulla, and Part of the *Papez circuit.
this tract is smaller than the *lateral cor- Anterior perforated substance The
ticospinal tract. Some of the anterior inferior surface of the *forebrain be-
*corticospinal tract fibers eventually tween the orbital gyri and the *hypo-
cross in the anterior white *commissure thalamus. This is the region where many
of the spinal cord and some end ipsilat- branches of the *lenticulostriate arteries
erally (primarily in the cervical and tho- and other small penetrating branches
racic segments of the spinal cord) on enter the brain.

28
Anterograde amnesiaAntihistamines

Anterograde amnesia Inability to ac- Anticoagulation therapy Drug therapy

quire and retain new information regard- to decrease blood viscosity/coagulation
less of the type of material presented often initiated following ischemic (i.e.,
(i.e., verbal or nonverbal) or the sensory nonhemorrhagic) *stroke or *transient
modality in which it is presented (i.e., ischemic attacks. The aim is to prevent
auditory, visual). The term amnesia is thrombogenesis and recurrent *embo-
typically used to describe significant dif- lism. The treatment may be administered
ficulty *learning new information in the on an acute or chronic basis, although
absence of generalized cognitive impair- different agents are used for the two
ment, as is the case in dementia. See also modes.
Amnestic syndrome. Anticonvulsant drugs Older term for
Anterolateral system A collection of *antiepilepsy drugs, although it is still
ascending sensory fiber tracts in the often encountered in contemporary use.
spinal cord and *brainstem that mediate This term has lost favor because many
pain, temperature, and crude touch (tac- *seizure types (e.g., absence seizures) are
tile pressure sensation). The *spinotha- unaccompanied by *convulsions. See
lamic tract is the best characterized of these Antiepilepsy drugs.
tracts, and, along with the spinolimbic Antidepressant drugs Medications used
tract, it carries pain and temperature sen- to treat *depression. The most common
sations to the *forebrain. Other tracts class of antidepressants is *selective sero-
within the anterolateral system (e.g., tonin reuptake inhibitors (SSRIs). Another
spinoreticular and spinomesencephalic common antidepressant class is serotonin
tracts) terminate at higher spinal levels and norepinephrine reuptake inhibitors
or brainstem sites, such as the reticular (SNRIs), which inhibit the reuptake of
formation. norepinephrine in addition to serotonin.
Antianxiety drugs Medications de- Atypical antidepressants are so classified
signed to reduce anxiety. Also called anx- because they do not fit into other catego-
iolytics. *Benzodiazepines are the most ries. *Tricyclic and tetracyclic antidepres-
commonly prescribed class of antianxi- sants and *monoamine oxidase inhibitors
ety agents. are older medications that are not com-
Anticholinergic delirium Acute *confu- monly used as first-line therapy because
sional state associated with anticholinergic of their multiple side effects.
therapy, commonly seen in the elderly and Antiepilepsy drugs (AEDs) Medications
during hospital admission. Many medi- used to control seizures. AEDs may have
cations have primary or secondary anti- cognitive side effects, primarily on psy-
cholinergic effects, including those used chomotor speed and rate of *informa-
to manage respiratory disorders, urge tion processing, and the risk of cognitive
incontinence, and *Parkinson disease. impairment is generally increased with
Unintended anticholinergic adverse ef- polydrug therapy. AEDs are generally
fects frequently occur with medications selected based on *epilepsy syndrome
prescribed with other mechanisms of and *seizure frequency.
action in mind, such as antihistamines Antihistamines A class of drugs that re-
(e.g., diphenhydramine), antidepressants duces histamine action. Antihistamines
(*tricyclic antidepressants and some ser- have anticholinergic effects that may cause
otonin reuptake inhibitors), and anti- sedation, although the sedating effects of
psychotics. newer antihistamines are significantly less

29
Antipsychotic drugs Aphasia

than those of older antihistamines. Older without pallium (cortex). (Latin pallo;
antihistamines that cross the *blood–brain cloak, robe)
barrier (e.g., diphenhydramine) are also Kretschmer, E. (1940). Das apallische
more likely to have sedative effects. In Syndrom. Zeitschrift für gesamte Neurologie
patients with *dementia, anticholinergic und Psychiatrie, 169, 576–579.
effects may exacerbate recent *memory Apathy A reduction of goal-directed be-
difficulty or *confusion. havior. The neural bases of apathy rely on
Antipsychotic drugs Medications used lesions or dysfunctions of those brain
to manage psychotic disorders. There structures that generate and control goal-
are several chemical classes of antipsy- directed behavior, notably the *frontal
chotic agents that vary widely in po- lobes, the *basal ganglia, and the frontal-
tency and side effects (phenothiazines, basal ganglia circuits. (Greek apatheia,
thioxanthenes, debenzapines, butyro- from apathēs, without feeling; a-, without
phenones, and indolones). Also called + pathos, feeling)
neuroleptic drugs. Apgar score A measure of neonatal
Antisaccade A voluntary eye movement status usually assessed at 1, 5, and 10 min-
in the direction opposite to the side where utes after birth. The Apgar score is based
a stimulus is presented. The task requires on heart rate, respiratory effort, muscle
the ability to suppress reflexive prosaccade tone, skin color, and response to stimula-
tendencies to look at the flashed stimulus tion, with resulting Apgar score ranging
and to generate a voluntary saccade to the from 0 to 10. Lower scores reflect poorer
*contralateral side. Poor antisaccade ability status with poorer prognosis (named for
is associated with impaired *frontal lobe Virginia Apgar, American pediatrician/
function, which controls *saccadic *eye anesthesiologist, 1909–1974).
movements. (Greek anti-, against; French Aphagia Decreased eating, typically asso-
saccade, jerk, jolt) ciated with hypothalamic lesions. Apha-
Hallett, P. E. (1978). Primary and secondary gia may also refer to swallowing difficulty,
saccades to goals defined by instructions. which in turn leads to decreased eating.
Vision Research, 18(10), 1279–1296. doi:
(Greek a-, without; phagein, to eat).
http://dx.doi.org/10.1016/0042-6989(78)
90218-3 Aphasia Acquired disorder of symbolic
*language processing. Aphasia is character-
Anton syndrome Denial of blindness, ized by a combination of naming, *fluency,
usually seen with bilateral *occipital comprehension, and repetition deficits that
lobe lesions causing *cortical blindness. are accompanied by reading and writing
*Confabulation may be present. Anton impairments. Characteristics of the lan-
syndrome is a specialized form of anosog- guage impairment include paraphasias,
nosia (named for Gabriel Anton, Aus- *circumlocution, or *conduite d’approche.
trian neurologist, 1858–1933). Different aphasia subtypes are classified
Anxiolytic drugs See Antianxiety drugs. according to the relative impairments of
Apallic syndrome This term is more different language domains, and different
commonly used in Europe and Asia than names for the same constellation of defi-
in North America to refer to *unrespon- cits are often used. In addition, aphasia is
sive wakefulness syndrome (previously commonly characterized by the mech-
termed *vegetative state). Term intro- anism of injury or by its relationship to
duced by Ernst Kretschmer (German underlying brain process. Aphasia does
psychiatrist, 1888–1964) in 1940, meaning not include disorders of articulation, such

30
AphasiaAphasia

as *dysarthria. Because most aphasia pa- often with *circumlocutions, few para-
tients have disturbed language function phasias, good comprehension, and
rather than a complete absence of lan- normal repetition; it is often the ulti-
guage, these disorders are more accurately mate evolutionary stage of reproduc-
classified as dysphasias. By tradition, how- tion conduction aphasia (see below).
ever, the term aphasia is used. Impairment may be sufficient to reduce
The classic aphasia subtypes usually *fluency. Anomia is the most common
result from acute injury, which is usu- and disabling feature of all perisylvian
ally vascular in etiology. Although slowly aphasias, but anomic aphasia refers to
growing tumors may cause language dis- pure anomia without other aphasic
turbance, they produce much less impair- deficits. Also called nominal aphasia or
ment than does *stroke, given the brain amnesic aphasia. Lesions causing anomic
area and volume of tissue involved. Aphasia aphasia are classically in the posterior
may also be observed in a variety of other superior *temporal lobe/supramarginal
conditions, such as *traumatic brain *gyrus region, but because semantic
injury. The quality of the language im- impairments can contribute to anomia,
pairments characteristically changes over anomic aphasia may be observed fol-
the months following acute injury or as lowing lesions almost anywhere in the
a function of disease progression. (Greek left hemisphere.
an‑, without, none; phemi to speak; • Anterior aphasia Nonfluent aphasia;
phasis, utterance) Broca aphasia. The term is used to
Aphasia Syndromes contrast with posterior aphasia. Its
• Adynamic aphasia Aphasia subtype name is derived for characteristic le-
proposed by Aleksandr Luria (Soviet sions anterior to the *central sulcus.
neuropsychologist, 1902–1977), char- • Apraxia of speech An articulatory dis-
acterized by nonfluent spontaneous order characterized by damage to the
output with preserved picture descrip- neural networks responsible for con-
tion, repetition, and comprehension. verting phonemes, which are discrete
Adynamic refers to difficulty in speech language elements, into continuous
initiation. It is often considered a form motor programs involving the oral, lin-
of transcortical motor aphasia (see gual, pharyngeal, and diaphragmatic
below), although it is better viewed as muscles. Speech is characterized by ef-
a disorder of volitional concept en- fortful production of severely distorted
gagement. phonemic sounds—sometimes referred
• Amnesic aphasia Aphasia subtype to as phonetic disintegration. Uni-
used in several early classification sys- formly observed with large anterior le-
tems that is characterized by difficulty sions, typically in the context of Broca
producing major lexical items; synony- aphasia (see below). Often associated
mous with nominal (anomic) aphasia. with at least some phonemic parapha-
“Amnesic” aphasia implies that word- sias, representing either genuine pho-
finding difficulty results from words nemic exchanges (aphasic errors) or de-
being “forgotten.” formation of phonemic articulation
• Anomic aphasia Aphasia subtype that leads to blurring of the articulatory
characterized by difficulty producing boundary between phonemes; for ex-
nouns and verbs in spontaneous lan- ample, a shift in voice onset time such
guage or naming to confrontation, that /p/ and /b/ are not reliably distinct.

31
AphasiaAphasia

• Broca aphasia Nonfluent aphasia 330–357, 398–407. Translated as “Remarks on

characterized by effortful, often agram- the set of the faculty of articulate language,
followed by an observation of aphemia.”
matic speech production with poor
Springfield, IL: Charles C. Thomas, 1960,
repetition and relatively preserved com- pp. 49–72.
prehension of single words and short
phrases (although comprehension of • Conduction aphasia Classically, fluent
syntax may be impaired, e.g., *Token aphasia (see below) with severely im-
Test). Naming is generally impaired, paired nonword repetition but relatively
although prompting with either a con- preserved language comprehension and
text or phonemic cueing may facilitate speech output characterized by frequent
performance. The sine qua non is sim- phonemic (literal) paraphasias and
plification of syntax (sentence structure). word-finding difficulty. Patients with
In English, grammatic morphology conduction aphasia have difficulty read-
(e.g., articles, pronouns, prepositions, ing aloud due to paraphasic errors but
auxiliary verbs, and tense markers) is may have relatively good comprehen-
typically omitted, but in richly in- sion. Now classified into two subtypes:
flected languages, grammatic mor- reproduction conduction aphasia and
phemes are usually present but often repetition conduction aphasia. Repro-
affected by paragrammatic errors. Read- duction conduction aphasia is synony-
ing comprehension is poor for sen- mous with the classic type. Repetition
tences that require processing of gram- conduction aphasia is characterized by
matical words. Right *hemiplegia is complete absence of phonological func-
commonly present because the large tion (hence no phonemic paraphasias),
lesions causing Broca aphasia typically relatively preserved ability to repeat
stem from occlusion of the tip of the words but no capacity at all for non-
*internal carotid artery or proximal word repetition, and markedly impaired
*middle cerebral artery, thus causing auditory-verbal short-term *memory.
severe damage to descending *cortico- These are best viewed as extremes along
spinal fibers. The lesion responsible for a continuum. Ludwig Lichtheim
aphasia typically results from infarc- (German physician, 1845–1925) pre-
tion of the upper (anterior) division of dicted the existence of this syndrome
the *middle cerebral artery. It charac- prior to seeing any clinical cases (see
teristically involves the frontal oper- Wernicke-Lichtheim model). He further
culum, insula, and adjacent peri- and postulated the lesion to involve the ar-
parasylvian frontal and temporal re- cuate *fasciculus beneath the supra-
gions. Global aphasia (see below) fol- marginal *gyrus, which disconnects the
lowing stroke commonly resolves into comprehension area of the *temporal
Broca aphasia, likely reflecting, in sub- lobe from the language output area of
stantial part, right hemisphere lan- the *frontal lobe. Thus, the conduction
guage function (named for Pierre Paul of language information between these
Broca, French surgeon and anthropol- areas is disrupted. Imaging studies have
ogist, 1824–1884). demonstrated that conduction aphasia
is associated with lesions of the posterior
Broca, P. (1861). Remarques sur le siège de la
faculté du language articulé; Suivies d’une perisylvian language areas, often rather
observation d’aphémie (perte de la parole). modest in the case of reproduction con-
Bulletins de la Société Anatomique (Paris), 6, duction aphasia but typically quite large

32
AphasiaAphasia

in the case of repetition conduction features. This term is used informally

aphasia. Damage to the arcuate *fascic- and has no localization or heuristic
ulus probably contributes, but in un- value.
clear ways that probably differ from • Mixed transcortical aphasia Rare
person to person. aphasia subtype in which repetition is
• Expressive aphasia Aphasia subtype relatively intact despite nonfluent spon-
in which nonfluent output is the prom- taneous language, poor comprehen-
inent feature; generally arises from ante- sion, and inability to name, read, or
rior lesions. The term is not commonly write. There may be *echolalia. Norman
used because some degree of expressive Geschwind (American neurologist,
difficulty is characteristic of most apha- 1926–1984) used the term “isolation of
sias. The term is used to contrast with the speech area” because the pathology
receptive aphasia. spares perisylvian language areas but
• Fluent aphasia Aphasia characterized involves the surrounding vascular end
by relatively normal prosodic variation zones.
and articulation with frequent para- Geschwind, N., Quadfasel, F. A., & Segarra, J.
phasias. Fluent aphasia includes Wer- (1968). Isolation of the speech area.
nicke aphasia, conduction aphasia, Neuropsychologia, 30, 327–340.
anomic aphasia, and transcortical sen- • Motor aphasia Nonfluent aphasia;
sory aphasia, all described within this Broca aphasia. The term is no longer
entry. The term contrasts with nonflu- in wide use and was used to contrast
ent aphasia. with sensory aphasia.
• Global aphasia Aphasia subtype that • Nominal aphasia Anomic aphasia.
involves nearly complete loss of all core • Nonfluent aphasia Aphasia that is
linguistic functions, including sponta- characterized by effortful speech pro-
neous language, naming to confronta- duction lacking normal prosody. Non-
tion, comprehension, repetition, read- fluent aphasia includes Broca aphasia,
ing, and writing. Large lesions involving global aphasia, transcortical motor aphasia,
both Broca area and *Wernicke area, as and mixed transcortical aphasia, all
well as other perisylvian language re- described within this entry. The term is
gions, are generally found. used to contrast with fluent aphasia
• Jargon aphasia Aphasia subtype char- (see above).
acterized by fluent paraphasic speech • Posterior aphasia Fluent aphasia;
that may be incomprehensible and filled Wernicke aphasia. The term is used to
with neologisms. Speech contains nu- contrast with anterior, or Broca aphasia.
merous semantic, verbal (real words This term is no longer in wide use. Its
phonologically but not clearly seman- name is derived from the character-
tically related to the target), and pho- istic location of lesions posterior to
nemic (literal) paraphasias. Paraphasias the *central sulcus.
are often complex and incomprehen- • Pure motor aphasia Nonfluent speech
sible (abstruse neologisms). Jargon with largely preserved language func-
aphasia represents a subtype of Wer- tioning, demonstrated by the ability
nicke aphasia (see below). to write. It is synonymous with aphe-
• Mixed aphasia Aphasia that does mia. Neither term is in wide use.
not fit into classic nosology, or aphasia • Pure word deafness A rare disorder
that has both expressive and receptive characterized by severely reduced

33
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 FOREIGN BODIES.
Foreign bodies become lodged on the internal surface of the
eyelids, in the folds of the conjunctiva, in the thickness of the cornea,
and sometimes, though rarely, in the anterior chamber, the lens, or
the vitreous humour. They include particles of grit or dust, the awns
and glumes of grain, etc.
The eyes are half closed and the conjunctiva is swollen, whilst the
eye weeps and the animals dread the light.
Diagnosis. This is somewhat difficult, for the parts rapidly
become very sensitive, and the animals violently resist examination.
When the foreign body penetrates the anterior chamber or the lens,
it produces suppuration or traumatic cataract.
Before anything can be done it is often necessary to render the
parts anæsthetic by instilling a few drops of cocaine solution into the
eye.
The foreign body may then be discovered by close observation. If
the pain is very intense, and great resistance is offered to opening the
eye, the practitioner may confine himself to passing a soft camel-hair
brush saturated with cocaine solution over the surface of the eye and
into the conjunctival sacs. The brush loosens, and often removes, the
offending body. In the absence of a camel-hair brush, the little finger,
covered with a piece of fine linen, may be used.
Where the parts cannot be touched owing to the resistance of the
animal, lukewarm solutions of antiseptics such as boric acid may be
occasionally injected into the eye by means of a syringe, but care
must be taken to prevent the animal injuring itself against the
syringe by sudden movements.
 CONJUNCTIVITIS AND KERATITIS.
Inflammation of the conjunctiva and inflammation of the cornea
almost always occur together, and reciprocally induce one another
when of a certain degree of intensity. They may be simple, that is to
say, produced by simple causes, or they may be specific, and of a
contagious character.
Simple inflammation is caused by the action of cold, draughts,
dust, or mechanical injuries. Specific inflammations, the nature of
which is still little understood, occur in the ox and goat. They are
very contagious, and may successively attack all the animals of a
herd.
The symptoms of acute and specific inflammation differ very
little. They comprise congestion, lachrymation, chemosis, a certain
amount of suppuration, and sometimes superficial ulceration of the
cornea. The patients suffer very acute pain, avoid the light, present
all the symptoms of photophobia, and are affected with spasm of the
orbicularis muscle.
In simple cases these symptoms frequently disappear, provided
the byres are kept clean and astringent eye-washes are applied.
In contagious keratitis, however, the cornea may suppurate and
even become perforated after a few weeks.
Treatment. The chief object of treatment under any
circumstances must be to insure the most perfect cleanliness both of
the globe of the eye and the conjunctival sacs.
The eye must, therefore, be irrigated with lukewarm water, the
stream being injected beneath the lids. Each irrigation is followed by
the use of an anodyne and astringent eye-wash containing borate of
soda or sulphate of zinc, combined if necessary with cocaine.

Distilled water 100 parts.

Borate of soda 4 „
Hydrochlorate of cocaine 1 „
 But saturated solution of boric acid is simple, and no less effective.
In contagious keratitis the eye lotion may contain 2 to 3 per cent.
of nitrate of silver, the excess of silver being neutralised by washing
out with a weak solution of common salt. After three or four
applications this should be changed for a saturated solution of boric
acid.
 VERMINOUS CONJUNCTIVITIS.
This form of conjunctivitis, described by Rodes in 1819, is due to
the presence of the Filaria lachrymalis, which varies in length
between ⅜ of an inch and 1 inch.
Like ophthalmia, the disease attacks cattle reared in wet localities.
Symptoms. The symptoms are those of acute conjunctivitis, and
consist first of lachrymation, then of injection of the blood-vessels of
the conjunctiva, together with swelling of the eyelids and
photophobia. The animals keep the eye closed, and display extreme
sensitiveness. Here again cocaine proves of value.
Examination is rather difficult, the worms being sometimes
displaced towards the cornea or membrana nictitans, although more
commonly they remain hidden in the folds of the mucous membrane
towards the point where the membrana nictitans is inserted. It is,
therefore, necessary to thoroughly expose the folds of the mucous
membrane in order to discover them.
In time conjunctivitis becomes complicated with diffuse
ulceration, keratitis, and sometimes with ophthalmia and
suppuration of the eye.
Diagnosis. The diagnosis is rather troublesome, and cocaine is of
great assistance.
Prognosis. This is rather grave.
Treatment. The object of treatment is the complete removal of
the parasites. This can sometimes be attained by means of the finger,
a pair of forceps, or a very clean feather.
Treatment is completed by injecting an antiseptic and anti-
parasitic eye lotion for several days, lest some of the worms should
remain hidden in the folds of the mucous membrane. One per cent.
creolin or 1 in 2,000 sublimate solution may be used.
If in some exceptional case it is difficult to remove the parasites,
they may be got rid of by injections or free irrigation. The stream of
liquid, pointed in different directions, distends the mucous
membrane and washes away the foreign bodies on its surface.
 VERMINOUS OPHTHALMIA OF THE OX.

This ophthalmia is due to the presence of a small worm, ⅔ of an

inch to 1¼ inches in length, which has been regarded as the larval
form of the Filaria cervina of the serous cavities.
It is very common in animals which are kept permanently in low-
lying meadows particularly in some parts of France, as for instance
in Normandy, in the departments of the Sarthe and the Mayenne.
Not infrequently it occurs as an epizootic, and is then regarded as a
contagious ophthalmia. Verminous ophthalmia occurs chiefly during
the spring and autumn.
Symptoms. The disease is accompanied by lachrymation, signs of
conjunctivitis, and fear of light. Very soon the media of the eye
become turbid, the sclerotic and cornea are injected, and finally
exhibit marked opalescence.
On examination the eye appears extremely sensitive; in fact, it can
scarcely be touched unless cocaine solution is previously applied.
The parasites, two or three as a rule, but in exceptional cases from
five to seven in number, are seen rolled up within the anterior
chamber of the eye. A week after the beginning of the attack,
however, they begin to move about, and are then found close behind
the cornea, upon the lens, or suspended in the aqueous humour.
The irritation produced sets up inflammation of Descemet’s
membrane and the cornea, together with iritis, and, secondly,
keratitis and changes in the lens.
Unless treatment is adopted verminous ophthalmia inevitably
ends in cataract.
Diagnosis. Diagnosis is always uncertain on account of the
difficulty of examination. When the cornea is very opaque
examination necessarily gives a negative result.
The prognosis is grave.
Treatment. Eye lotions containing tincture of aloes, creolin,
corrosive sublimate, etc., have been suggested, but are practically
useless, because they can have no action on a parasite enclosed
within the globe of the eye. The most logical treatment consists in
aseptic puncture of the anterior chamber of the eye towards its lower
border with a cataract needle.
The escaping liquid carries with it the parasites, and recovery is
then only a matter of time, provided the wound does not become
inflamed. The great danger consists in inflammation and
suppuration of the eye. This, however, can be avoided by antisepsis
and by applying a surgical wool dressing, which can be left in place
for a few days.
 SECTION X.
INFECTIOUS DISEASES.
 COW-POX—VACCINIA.
The name cow-pox, or vaccinia, is employed to describe a special
disease which in animals of the bovine species is characterised by the
development of pustules at points where the skin is fine, and more
particularly the mammary region.
It can be conveyed both to man and the domestic animals.
This disease has been known from time immemorial, and it would
appear that first of all in the East and later in England it was a
general belief that its attacks rendered human beings proof against
small-pox. Medical men, it must be admitted, long regarded this
belief as a popular delusion, as is proved by their continuing to
practise inoculation with true small-pox material.
Jenner in 1770 was the first to declare the truth of this popular
opinion, and by his wise foresight to confer on humanity one of the
most beneficent discoveries ever made, although the weight of
modern opinion is in favour of the identity of cow-pox and human
variola. Having observed that milkmaids who happened to have
small cuts or sores about the hands sometimes contracted the
disease in a mild form, and that they did not afterwards suffer from
small-pox, he was struck with the advantages consequent on such a
discovery, and having proved the possibility of inoculating human
beings artificially, he immediately formulated the principles of
vaccination. A child eight years of age was vaccinated with cow-pox,
and afterwards inoculated with pus from a small-pox patient. It
contracted vaccinia in consequence of the first inoculation, but
entirely resisted the attempt to inoculate it with small-pox.
Vaccination had been discovered.
Jenner furthermore proved that cow-pox was transmissible from
cow to cow and from man to man, but it seemed to him that the
original disease was to be sought elsewhere, and that the pustular
affection originated primarily with the horse. The horse is sometimes
the subject of a pustular disease called horse-pox; this disease when
inoculated in man confers immunity against small-pox, just as does
cow-pox, and Jenner believed that the disease did not attack cows
unless they had been accidentally inoculated through the medium of
the people about the farm. Unfortunately, he named the pustular
disease of the horse which he had studied “sore heels,” and for a long
time all those who busied themselves with the question of vaccine
confounded “sore heels” with a number of different diseases,
although as early as 1802 Loy had experimentally proved that so-
called “grease” (in reality horse-pox) was transmissible by
inoculation to the cow, in which it produced cow-pox.
Loy’s “grease” and Jenner’s “sore heels” only represent forms of
horse-pox, but for more than fifty years the origin of vaccine was
sought in grease, lymphangitis, and other diseases which attack the
extremities of horses’ limbs. Pételard (1845–1868) rediscovered and
redescribed horse-pox and proved its transmissibility to man;
Lafosse and U. Leblanc discovered it in an epizooty which broke out
at Rieumes; and Bouley in 1862 furnished a synthetical description
of it under the designation of horse-pox. He shows that horse-pox is
always a pustular disease, but that it may sometimes appear in the
form of a discrete eruption around the lips and nostrils, sometimes
of an eruption limited to the pasterns or extremities of the limbs
when inoculation has been effected in this region, sometimes of
lymphangitis, and sometimes of a more or less confluent and
generalised eruption.
Symptoms. The disease as discovered and described by Jenner
was soon rediscovered and redescribed on all sides—by Sacco in
Italy, Hering in Germany, etc.
The pustular eruption usually appears on the udder in the case of
cows, and on the muzzle, nose, and lips in that of calves. In
exceptional cases the eruption may become generalised.
The pustules are round or slightly elliptical, and are preceded by
the appearance of red congested patches, followed by infiltration and
thickening of the skin.
The pustule is moderately prominent, and after some days there is
exudation at its centre, transforming it into a vesico-pustule. The
exuded liquid collects under the thickened layer of epidermis, which
it raises, and on examination it appears as a white or transparent
little central patch, with a thin grey periphery surrounded by a
reddish inflammatory zone. This liquid becomes thicker and the
pustule is flattened at its centre, then, towards the eighth or ninth
day, the pustule is ruptured, owing to tearing of the epidermic patch.
The vaccine thus escapes.
In what is termed spontaneous vaccinia the udder is covered with
a varying number of pustules, usually in different stages of
development. Some are very small, whilst others have attained the
size of sixpence and are already in course of cicatrisation.
When cow-pox is accidental or the result of inoculation, the
eruption occurs exactly at the point of inoculation, whether the latter
has been through an abrasion, a puncture, an incision, or any other
skin injury, and the eruption may assume the most varied
appearances, according to the nature of the primary lesion, although
the mode in which the pustules themselves form never varies.
Pustules experimentally produced by puncturing the parts may be
taken as a type of inoculation. On the day following the operation
nothing abnormal appears. On the third day there is a slight swelling
around the point punctured, and this increases until the fifth day,
when there is exudation, which converts the primary lesion into a
vesico-pustule. On the sixth day the vesico-pustule becomes
umbilicated at its centre, the exudation is abundant, and already
vaccine might be collected.
This may be termed the period of crisis; the appearances are most
characteristic. During the following days the vesicle is ruptured; the
discharge continues from the ninth to the twelfth day, when the
pustule diminishes in size and dries up. After the fifteenth day crusts
of a brownish colour form; these separate between the twentieth and
twenty-fifth days, leaving hard whitish-looking cicatrices, which
permanently remain.
Moderate itching accompanies the development of the eruption,
the principal functions are not disturbed, and fever only appears in
the event of the eruption becoming confluent or extending over a
large area. When the eruption is generalised the pustules or vesico-
pustules are found mostly in the region of the elbow, the lower
border of the neck, the flank and the inner surface of the limbs. They
present exactly the same appearance as the pustules on the surface of
the udder, but, being covered with hair, are less open to inspection.
In certain rare cases the eruption extends to the perineum and lips
of the vulva. Signs of inflammation then develop on one or both
sides, the tissues display œdematous infiltration and disseminated or
confluent pustules. The lymphatic glands and vessels in the
neighbourhood of the pustules are always swollen.
Causation. Cow-pox, or vaccinia, is a virulent disease
transmissible by accidental or intentional inoculation. The discharge
from the vesico-pustules and the crusts which afterwards cover them
are virulent, and inoculation can be performed by simply scratching
the skin. A first attack confers prolonged and sometimes perfect
immunity, the operation being successful if only one pustule
develops.
The disease is transmitted to healthy animals by milkers, by calves
in sucking, or by the conveyance in whatsoever form of virulent
material to sores or cuts.
The nature of the parasitic or microbic agent which produces the
disease is still unknown. Some investigators have described
intracellular parasites, others extra-cellular parasites, others, again,
blood parasites, etc., but the exact cause has always eluded research.
It is, however, known that filtration of vaccine, pure or diluted,
through porcelain removes the active material, which remains in the
residue arrested by the filter.
Prolonged exposure to a temperature above 104° Fahr. (40° C.)
greatly diminishes the activity of the vaccine. Simple desiccation has
no action. Warming to 140° Fahr. (60° C.) for fifteen minutes also
destroys its action completely.
These facts explain why it has always been so difficult to preserve
and cultivate vaccine in tropical regions.
Mixed with equal parts of neutral glycerine, the virulent material
preserves its activity unimpaired for from six to eight months.
The disease develops equally in man, the horse, the ox, the buffalo,
the goat, and the camel. Its development is less typical in the pig,
sheep, dog, and rabbit. Young animals are best adapted for its
cultivation.
The blood and serum of animals suffering from cow-pox possess
immunising properties, but only when administered in very large
doses, say from 6 to 12 lbs. of blood, or ½ to 1 lb. of serum.
The curative action of this serum against small-pox is
comparatively trifling.
 The diagnosis of cow-pox is not very difficult.
At first the disease might be mistaken for false cow-pox, the
eruptions of foot-and-mouth disease, or gangrenous coryza.
In false cow-pox, the nature of which is also little understood,
although it is known to be contagious and is regarded by some as
true vaccinia, the pustules are smaller and thinner, while the vesicle
is more developed, and the disease runs a more rapid course.
In foot-and-mouth disease the eruptions are of the nature of
vesicles or bullæ, not pustules. The eruption occurs in twenty-four
hours or less, and can only be mistaken for vaccinia during the
period of desiccation and the formation of crusts.
Finally, as regards gangrenous coryza, the hardened pustules do
not produce vesicles.
The prognosis is generally favourable. The disease runs its
course within relatively fixed periods, according to the development
of the pustules, and recovery occurs without complications.
Treatment. No curative treatment can be laid down, the
development of the disease being perfectly regular and tending to
recovery. Simple hygienic precautions and cleanliness are sufficient
to avoid complications due to suppuration.

COW-POX AND HUMAN VARIOLA—PREPARATION OF

VACCINE.

Time and experience having proved that inoculation with cow-pox

or vaccinia protected human beings against small-pox, the question
arose as to the connection between the two diseases, whether or not
they were identical and whether vaccinia in the bovine animal might
not merely represent an alternative form of small-pox. The
importance of the question will at once be understood by bearing in
mind the danger to which human beings would be exposed by
vaccination with small-pox virus unmodified by passage through the
calf.
Nevertheless, at the present time the opinion of the early writers
appears to prevail, and the theory of identity is accepted by the great
majority of scientific men. The reason why experimenters in the
second group came to believe in duality is that the method of
inoculation chosen (by puncture) was not entirely reliable. The
inoculations proved too slight, and it is only after inoculation by
scarification or incision that typical eruptions can be reproduced in
series.
Preparation of vaccine. Whether cow-pox and small-pox are or
are not identical, the benefits resulting from vaccination are none the
less real, and it is to be hoped that vaccination and revaccination will
soon be made obligatory in all countries. We should then no longer
have to deplore those epidemics of small-pox which periodically
cause consternation in large cities and colonies.
The preparation of vaccine has been the subject of such minute
care in every country that neglect of vaccination is astonishing. The
material is obtained from calves or cows. In France the vaccine is
prepared from animals of five to eight months old, free from disease.
The old system of inoculation by puncture has been completely
abandoned, the yield being insufficient, scarifications or incisions
being now employed.
The animal is secured or, better still, laid down on a suitable table,
and is shaven over a sufficient surface. The inoculations are made on
the sides of the chest, over the thorax or elsewhere, but preferably
over the flank and thorax, as being most readily accessible. The
region of operation is rendered aseptic as far as possible, and
scarified in lines about 1 to 2 inches in length, the lines of one
horizontal row alternating with those in the next. It is imperative
that the slight bleeding which may result should entirely cease before
inoculation is attempted.
The scratches are inoculated with the purest vaccine obtainable,
preferably with glycerinated pulp which has been kept for six weeks
or two months. From the third day the lines of inoculation become
prominent, and an indurated longitudinal swelling, with all the
characteristics of a pustule, soon projects above the neighbouring
portions of skin. On the fifth day exudation commences, and from
the sixth to the seventh day a large quantity of vaccine lymph may be
collected. The line of inoculation appears slightly umbilicated and
surrounded by a greyish-white zone and a hard peripheral swelling.
Vaccine may be collected from the fifth day in summer to the
eighth day in winter.
 The inoculated area having been cleansed with boiled water and
carefully dried, the little crusts covering the inoculation wounds are
loosened and the wounds themselves gently scraped with a special
curette of small size. The exuded liquid is very active.
The base of each swelling is then grasped in a little special clamp,
which acts like a pressure forceps and causes the discharge of a
further large quantity of active vaccine lymph. All the material thus
obtained is mixed; an equal quantity of neutral glycerine is added,
the whole is finely triturated, passed through a cloth, and stored in
little sterilised glass tubes, which are hermetically sealed.
The vaccine thus prepared retains its activity for from five to eight
months, if kept from the action of heat and light. Accidental germs
which may have developed in the wounds and thus gained entrance
to the vaccine gradually lose their activity. After from forty to sixty
days the vaccine may be regarded as absolutely pure and incapable of
producing accidental suppuration, as sometimes occurs when fresh
vaccine is employed.
The old electuaries, dried vaccines, vaccine pastes, etc., have been
almost entirely given up, the above method always yielding a pure
and active vaccine. Vaccination with calf lymph should always be
preferred to vaccination from arm to arm, in view of possible
transmission of grave disease, such as syphilis.

TETANUS.

Tetanus is a disease characterised by tonic contraction of the

muscles of one or more limbs or of all the muscles of the body.
Causation. It is due to the growth of Nicolaïer’s bacillus in some
part of the body (in accidental wounds, in the uterine cavity after
parturition, etc.), and the contraction of muscles is due to toxins
(elaborated by the microbe), which have a selective affinity for the
nervous centres.
These toxins, secreted by bacilli localised in wounds, are absorbed
and carried away by the lymphatic and vascular channels and
distributed throughout the body. They seem chiefly to affect the cells
of the central nervous system. Infection is due to microbes capable of
living as saprophytes outside the animal body.
 Nicolaïer’s bacillus assumes the form of a straight rod, one end of
which is swollen by the presence of a spore. It is anerobic, grows in a
number of different media, most rapidly at a temperature of 100° to
102° Fahr. (38° to 39° C.), and stains well by Gram’s method.
Though quite common in the horse, tetanus is rare in other
domestic animals.
In the ox it may result either from mechanical injuries,
suppurating sores, or surgical operations. In the cow, goat, and sheep
it sometimes assumes the form of a true enzooty after parturition if
the byres, etc., are not disinfected. In male animals it principally
follows castration by one of the cutting methods, and in lambs is
seen after amputation of the tail. A large number of animals
belonging to one flock may be affected, and Moussu has known two-
thirds of a given number of castrated lambs to die of tetanus.
Despite the sensitiveness of domesticated animals to tetanic
infection they may all be protected, either by injections of cultures,
or by gradually increasing injections of specific toxin. The latter,
however, are more efficacious when modified by the addition of
terchloride of iodine or of iodine water. The blood of immunised
subjects rapidly acquires antitoxic powers, which may be greatly
increased for the purpose of obtaining anti-tetanic serum.
The symptoms of tetanus are the same in all species.
In the first stage the animal appears stiff, walks in a jerky way and
holds the head high, with the ears pricked. The eyes are slightly
withdrawn into the orbits, and the animal shows marked general
excitability.
In the second stage there are muscular contractions, together with
trismus, stiffness of the neck, limbs and vertebral column, spasm of
the spinal muscles and muscles of the limbs (tonic contraction), and
the animal has a peculiar, staring look.
In the third stage mastication becomes difficult or impossible,
respiration is impeded, and the animal suffers from spontaneous
attacks of muscular contraction or from attacks due to external
stimulation (noises, sudden movements, changes from darkness to
light, etc.).
In the fourth stage the animal is liable to fall, asphyxia threatens,
and death occurs from respiratory syncope.
 Recovery is quite exceptional in the sheep, goat, and ox; death
usually occurs between the second and sixth days.
Diagnosis. Tetanus being less common in the ox, sheep, and goat
than in the horse, the diagnosis is not so easy in these animals, but as
it develops under different conditions, and as it usually attacks
several animals in one byre or fold, the diagnosis is rarely very
difficult. At the worst some hesitation may be felt at first, the
condition being mistaken for disease of the brain.
The prognosis is extremely grave.
The treatment is, above all, of a preventive nature, investigation
having proved that injections of anti-tetanic serum, before the first
appearance of tetanus, are invariably effectual.
If, therefore, one case of tetanus appears in a byre at calving time
or in a flock at the season when the lambs are castrated, no
hesitation should be felt in preventively inoculating all the castrated
animals and the cows which have calved. The quantities required are,
for a cow 10 cc. (about 3 fluid drachms) and for a sheep 5 cc. of anti-
tetanic serum.
This treatment, however, should be supplemented by general
hygienic precautions and internal treatment, such as irrigation and
disinfection of the parts affected.
Curative treatment has little chance of success. Experiments have
also proved that when the first symptoms of tetanus appear, anti-
tetanic serum is powerless to prevent the development of the disease.
Nevertheless, as its gravity is in direct ratio to the quantity of toxin
absorbed, and as the degree of this absorption depends on the length
of time that the place remains infected, the first thing to be done is to
disinfect and, in certain cases, curette the wounds which are believed
to be the source of mischief. Although antiseptics have little action
on Nicolaïer’s bacillus, they may be used. Solutions of iodine appear
most active, both as regards ordinary wounds and infection of the
uterus.
General tonics, diuretics, and lukewarm gruels can be given.
Unfortunately the patients are often unable to swallow them. In such
cases both liquids and medicines may be directly introduced into the
rumen by puncturing the parts with a trocar and canula, the latter
being left in position.
 Intravenous injections of large quantities of normal salt solution
are also of considerable value, 4 to 6 quarts per day for an ox and 20
to 40 fluid ounces per day for a sheep.

ACTINOMYCOSIS.

Actinomycosis is a disease produced by a fungus belonging to the

group of oomycetes (Actinomyces bovis) which develops in the
depths of living tissues in man and the ox, producing grave and
sometimes incurable lesions, most commonly in and about the jaws.
Actinomycosis is very common in America, and is also met with in
all parts of Europe.
Symptoms. The disease assumes many different clinical forms,
but it more frequently attacks some parts of the body than others,
and by far the greater number of cases occur in the ox.

ACTINOMYCOSIS OF THE MAXILLA.

Actinomycosis of the maxilla attacks young animals, and its usual

seat is in the molar region, although occasionally it affects the
incisors.
The earliest symptoms consist in swelling of the bone, which may
be overlooked if within the mouth, but the outline of the jaw soon
becomes deformed, generally in the middle region of the row of
molars. Somewhat tender and firm to the touch at first, the tumour
gradually increases in size, invades the deeper regions of the skin,
and displays fluctuation at one or two points, followed by abscess
formation. The pus discharged may be white, creamy, and
inoffensive, but the cavity of the abscess shows no tendency to
cicatrise, and the opening through which the pus has escaped is
transformed into a fistula. From this moment the pus discharge is of
a greyish, sanious nature, and contains a greater or less number of
little yellowish grains. It soon acquires an offensive odour, and the
fistulous opening is surrounded by exuberant granulations, forming
a fungoid mass.
 Fig. 268.—Actinomycosis of the jaw.

The neighbouring tissues become hardened and lose their

sensitiveness, the jaw becomes completely deformed, and a condition
is set up which the old writers considered as true cancer of the jaw or
maxillary osteosarcoma (Fig. 268).
A probe passed into the fistula penetrates deeply, usually into the
thickness of the jaw itself, and however carefully manipulated injures
the diseased tissues and causes free bleeding.
If neglected, these lesions become steadily worse, mastication is
more difficult, being possible only on the healthy side, and the
animals lose condition and eventually die of exhaustion. The external
lesion, represented by the fungoid mass, increases in size, assumes a
blackish colour, and discharges an offensive liquid. Portions of it
undergo mortification and give off a characteristic and extremely
fœtid odour. The molars become loose and in some cases fall out, but
development is usually slow, and some weeks or months elapse
before this stage is reached.
When the disease attacks the region of the incisors the symptoms
are much sooner apparent, and treatment is much easier. The
parasitic invasion results from an injury to the jaw caused by
shedding of the milk teeth. Swelling of the body of the jaw thrusts the
lower lip downwards, interferes with the prehension of food, and
calls for prompt treatment. The disease is rarely allowed to attain the
degree of development shown in Figs. 269 and 270.
As in the preceding instance, the animals die of exhaustion unless
relieved.
For reasons
difficult to explain,
but probably
because
inoculation is less
easy,
actinomycosis is
much rarer in the
upper than in the
lower jaw. The
disease develops
exactly as above
described, but
shows much less
tendency to
external
ulceration. It
invades the
maxillary sinus
and the region of
the palate, and
fistulæ are found
Fig. 269.—Actinomycosis in the region of the opening into the
incisors. buccal cavity,
while at the same
time the region of
the forehead is often deformed.
 ACTINOMYCOSIS OF
THE TONGUE.

Actinomycosis
attacks the tongue
apart from any
lesion of the jaws,
and produces what is
commonly called
“wooden tongue.”
The disease
develops in the
tongue itself,
generally in the
submucous zone,
and causes chronic
interstitial
inflammation,
infiltration of the
connective tissue,
and, in time, changes
in the muscular
Fig. 270.—Actinomycosis in the region of the
structures
incisors.
themselves.
The tongue shows
progressive hypertrophy, and becomes hard, sensitive, rigid, and
incapable of free movement. As a result the patients first have
difficulty in grasping food, then in swallowing their saliva, which
dribbles from the mouth, and finally are quite unable to feed
themselves.
The tongue is enlarged and indurated, and fills the entire cavity of
the mouth. Sometimes it projects beyond the incisors, excoriated and
bleeding. On passing the hand into the mouth it is found that the
surface is covered with little yellowish or red ulcerated nodules,
varying in size from that of a large pin’s head to that of a lentil.
In eating, the animals seize food between the lips and lift the head
high, so as to allow the food to fall between the rows of molars. The
motion is very similar to that of a fowl drinking.
 ACTINOMYCOSIS OF THE
PHARYNX, PAROTID GLANDS
AND NECK.

Actinomycosis may
sometimes leave the mouth
and tongue unaffected and
attack the pharynx, from
which it extends in the
direction of the parotid
glands and external surface
of the neck. In these cases,
however, the inoculations
are more localised than
when the surface of the
tongue is attacked, and the
lesions consist of
vegetations, polypi, or
actinomycomata.
The growths develop on
the posterior pillars of the
Fig. 271.—Actinomycosis of the tongue. fauces, on the sides of the
pharynx, or near the
entrance to the œsophagus.
They interfere with swallowing, and produce symptoms which are
easy to detect and interpret.
The lesions may also affect deeper seated tissues and produce
growths in the parotid or subparotid region, or lead to the
development of fistulæ in the region of the neck. Most fistulæ,
however, in this region are due to specific inoculation of external
injuries. Fistulæ originating in the parotid region and in the upper
part of the neck usually resemble in appearance the maxillary
fistulæ. The external fungoid growth, however, is less exuberant,
suppuration is less abundant, and the surrounding induration less
extensive.
Various localisations. Although the disease generally attacks
the mouth, tongue or pharynx, it may invade the œsophagus, rumen,
reticulum, liver and intestine, larynx, trachea, lung, peritoneum,
epiploon, and even the udder.

Fig. 272.—Actinomycosis of mammary gland (cow).

Localisations in the udder and peritoneum are commonest in pigs,

and it is believed that inoculation occurs either through the
galactophorous sinuses or through the abdominal wounds made for
purposes of castration.
Causation. The cause of actinomycosis is to be sought in the
development of Actinomyces bovis within living tissues. It seems
problematical whether the germs to be found in the pus or saliva of
affected animals ever directly infect new hosts, and it is difficult to
carry out infection in this manner even in very sensitive
experimental animals. Nevertheless, the persistence of the disease in
certain byres would seem to support the view of direct infection.
 On the other hand, it is proved that the actinomyces is a parasite
affecting vegetables, principally the graminaceæ, and that domestic
animals are most commonly infected through injuries caused by
vegetable substances. This is suggested by the discovery of the débris
of grain at the point where the lesions have originated.

Fig. 273.—Actinomycosis of mammary gland (cow), showing

growth invading lobules. (Figs. 272 and 273 are from blocks
kindly supplied by Mr. Gilruth, F.R.C.V.S.).

Inoculation is commonest in the mouth and on the surface of the

tongue, parts which are, so to speak, permanently excoriated. The
shedding of the temporary molars favours such accidents, and this is
why actinomycosis of the jaw is, relatively, so common. The incisor
region may also be inoculated during the shedding of the milk teeth,
but as the infected food comes more closely and for much longer
periods in contact with the molars, it is easy to understand why
actinomycosis is rarer in the incisor region.
The conditions are less favourable for inoculation of the pharynx,
because food does not remain in position there for more than a
second or two, but when the epithelium has been shed as a
consequence of laryngitis or pharyngitis, infection may occur.
As regards cutaneous inoculation, the parasite only seems
dangerous when the skin is excoriated or injured either accidentally
or as the result of surgical interference.
Actinomycosis of the
lung is probably caused by
the germs being inhaled
along with the inspired air.
Lesions. The lesions
are very peculiar in
character, and end in
completely destroying the
tissues invaded.
Once lodged within an
organ, the disease shows a
tendency to extend in all
directions, and, despite the
defensive reaction of the
tissues, it soon forms
numerous parasitic
centres.
In bones, for example,
actinomycosis invades the
spongy tissue with the
greatest ease. It causes
Fig. 274.—Old-standing bone lesions subacute ostitis, which
in a case of actinomycosis of the jaw. leads to diffuse
suppuration and local
hypertrophy of the bone,
destruction of the compact layers, and the development of an abscess
with fungoid, exuberant, granulating walls which show no reparative
tendency whatever.
 The pus of the abscess and the liquid from the fistula contain
varying quantities of yellowish grains, representing clusters of
actinomyces. The surrounding tissues, muscles, tendons, skin, etc.,
are all involved before long in the inflammatory process, and the
granulating masses themselves are invaded by the yellowish parasitic
tufts. All the fistulæ are surrounded by enormous zones of
infiltration, which on incision exhibit a lardaceous appearance. On
section it may appear that the lesion is confined entirely to the bone,
though this is exceptional (Fig. 274). Ordinarily the neighbouring
tissues are also destroyed, and not infrequently there is
communication with the external air. Sections then display a fungoid
tissue, interspersed with perforated lamellæ of bone and lardaceous
tissue containing cavities crammed with actinomyces.
The lesions in
the parotid
regions, the neck
or other parts
attacked always
present the same
appearance, viz.,
wide, tortuous,
bifurcated
fistulæ, with
exuberant
granulations
both in the
direction of the
cavities and of
the exterior,
together with
lardaceous
induration of the
tissues and
abundant fœtid
liquid pus.
Fig. 275.—Highly-magnified clump of
When it
actinomyces.
affects the
tongue the
parasite is to be found in the submucous region, where it causes little
swellings, which, when superficial, rapidly undergo ulceration. The
subjacent regions, the interstitial connective tissue, and the muscular
tissue become infiltrated, hardened and progressively sclerosed. The
tongue is gradually hypertrophied, and soon it becomes as hard as
wood, whence the term “wooden tongue.”
Actinomycosis of the lung may easily be mistaken for tuberculosis,
for the centres, although usually confined to one lobe, may also be
disseminated. The lesions, however, are surrounded by an abundant
fibro-sclerous inflammatory tissue.
In the abdominal cavity, particularly in sows, actinomycotic
lesions occur as little masses varying in size between that of a pea
and that of a haricot bean, attached to the epiploon and peritoneum
and filled with pus containing mycosic grains.
Diagnosis. Actinomycosis is usually easy to recognise, both on
account of the special character of the lesions and the presence of the
little grains formed by the parasite. The practitioner will rarely fail to
recognise at once the signs of actinomycosis of the jaw, but
actinomycosis of the tongue is more apt to be mistaken for deep-
seated sclerosing glossitis, although a careful examination will
always enable the different symptoms to be distinguished.
It is otherwise with regard to growths in the pharynx and
œsophagus, for, until after removal, simple polypi cannot be
distinguished from actinomycotic growths. In such cases the
administration of iodide of potassium affords valuable indications.
The prognosis is grave, whatever the clinical form of the disease.
Important advances, it is true, have lately been made, and the iodide
of potassium treatment is of great value, but too much must not be
expected of it, and its benefits have certainly been exaggerated.
Clinical experience suffices to prove that only actinomycosis of soft
tissues can be cured by drugs, bony lesions being amenable only to
medical and surgical treatment combined. Even combined treatment
is often unsuccessful.
Treatment. Thomassen in 1885 first explained the favourable
action of iodide of potassium on actinomycotic growths, and Nocard
in 1892 again directed public attention to the advantages attending
the use of this drug both in man and the lower animals. As too
frequently happens, however, the benefits of this treatment have
been exaggerated, and iodide of potassium has been held out as a
specific even against lesions in bone. With very few exceptions this is
incorrect, and, as Moussu has shown, when the disease affects bone
tissue it only yields to mixed treatment.
The treatment of actinomycosis may therefore be considered
under two heads.
Firstly, the treatment of actinomycosis of soft tissues; and,
secondly, that of bone.
Actinomycosis of soft tissues, muscle, skin, lymphatics, serous
membranes, etc., comprises the most common forms of
actinomycosis, viz., those of the tongue, pharynx, parotid glands,
neck, etc.
The second form comprises actinomycosis of the lower jaw (molar
region), the upper jaw, region of the incisors, etc.
Iodide of potassium in daily doses of from 2 to 3 drachms is almost
a specific in dealing with the first form of disease.
In lingual actinomycosis, for example, the effects may be seen a
few days after treatment is begun. The tongue becomes softer and
more mobile, can be protruded beyond the mouth and retracted into
it, and day by day tends progressively to resume its normal
appearance.
The patients, which were previously slowly dying of inanition
because they were unable to feed themselves, again take to their food
and begin to put on flesh. To ensure the treatment being efficacious
it should, as a rule, be continued for three or four weeks.
During the course of this treatment the system becomes saturated
with the drug, but no bad effects follow. The patients suffer from
lachrymation, coryza, bronchorrhæa, and especially iodic eczema,
but all these symptoms diminish and disappear soon after the
administration of the drug is discontinued.
Recovery, however, is not always permanent, and even when the
tongue has resumed its normal appearance a relapse may occur. We
have seen several such cases after treatment extending over more
than six weeks, and it is therefore often advisable to fatten the
animals as rapidly as possible and prepare them for slaughter.
 If no relapse occurs, and recovery is regarded as permanent,
another complication may make its appearance, viz., sclerous
atrophy of the tongue. This is almost as dangerous as the primary
lesion, because it prevents the animals from feeding, and constitutes
an additional reason for following the course above suggested.
Other lesions of soft tissues, such as disease of the parotid or
cervical glands, etc., yield to the same treatment, but it is advisable
first of all to clean out the fistulæ, scrape off exuberant granulations,
cleanse the irregular culs-de-sac, and thoroughly curette all
accessible parts.
Treatment is much longer than in the case of actinomycosis of the
tongue, but it is not always necessary to push the remedy to extreme
limits. As soon as symptoms of iodism appear only a drachm or two
of the drug need be given daily.
Actinomycosis of Bone.—As a general rule, actinomycosis of
bone resists the administration of iodide of potassium, a fact
probably explained by the much less abundant blood supply in bone
as compared with very vascular tissues, such as the tongue.
To have any chance of success the iodide treatment must be
supplemented by surgical interference. As regards the surgical aspect
of the case, the affected bone should be removed as far as possible,
together with all broken down tissue. Should this be neglected, the
disease returns in a little while.
In actinomycosis of the region of the incisors the method is radical
when adopted in time. The body of the maxilla can be partially
removed with a fine saw, two cuts being made disposed thus: < (the
letter V sideways). The upper and lower layers of compact tissue
should be spared as much as possible, so that the body of the bone
may not afterwards break. Recovery is only a matter of time.
A very small local iodoform dressing is applied, and, when healthy
granulations appear, cicatrisation can be left to natural means.
Cases of actinomycosis of the jaw are much more troublesome. If,
as usually happens, the lesion is ulcerated before the practitioner is
called in, the external fungoid growth should be removed by means
of an elliptical incision through the skin, the axis of the ellipse being
parallel with the branch of the maxilla. The bony fistula is then
exposed.
 In following up this fistula care must be taken not to injure the
facial artery, the facial vein, or Stenon’s duct. Once the bone is
exposed the disease can be attacked in the depths. The diseased
interior is cut away by means of a special curette, all affected
portions being removed, and an iodine or iodoform dressing is then
applied.
The operation is extremely troublesome, owing to the enormous
bleeding, and sometimes it is impossible to carry out successfully, as
in the case of old-standing and extensive lesions. To ensure recovery
under such circumstances, it is necessary to remove a portion of the
branch of the jaw, and this, though quite possible from the scientific
standpoint, would not be worth while in an animal, the value of
which is usually small.
Curettage of the bone is only of value in dealing with recent
lesions, and even then should not be practised except in the case of
animals which the owners particularly desire to keep.
In cases of actinomycosis of the upper jaw surgical treatment is
just as difficult as in the lower jaw, and calls for similar precautions.
The diseased portions of bone having been removed, the cavity is
plugged with iodoform or cotton wool, or a dressing saturated with
boric acid and iodoform.
In all surgical operations it is important not to injure the dental
arteries or nerves, or the alveolo-dental periosteum.
 TUBERCULOSIS.
Tuberculosis is a contagious disease produced by the action of
Koch’s bacillus. It is common to man and all domesticated animals,
but it specially affects animals of the bovine species. Its existence has
long been recognised, although in oxen it was formerly confounded
with the lesions of peripneumonia and echinococcosis.
It was not until the beginning of the nineteenth century that
Laënnec (1811) described the tuberculous lesion from the anatomical
and pathological standpoint. Gürlt pointed out for the first time in
1831 the similarity, the identity in fact, of tuberculous lesions in man
and the ox.
In 1865 Villemin showed that tuberculosis could be conveyed from
animal to animal, always producing similar lesions, and in 1868
Chauveau proved that, in the calf, infection might arise simply from
the eating of tuberculous material.
At a somewhat later date doubts were entertained regarding the
identity of human and bovine tuberculosis. Virchow denied the
identity of the two diseases on the basis of a comparative study of the
lesions. His opinion, however, has not prevailed, and the doctrine of
the identity of tuberculosis in mammals still appears probable, in
spite of the recent declarations of Koch (1901).
Causation. Tuberculosis is due solely to the activity of the
tubercle bacillus. In 1884 Koch isolated and cultivated this bacillus in
living animals, and always reproduced typical tuberculous lesions by
injecting cultures. In 1887 Nocard and Roux described a rapid
method of cultivating the bacillus, and in 1890 Koch announced the
discovery of tuberculin.
The tubercle bacillus assumes the form of a little rod, five or six
micromillimètres in length, and ·03 to ·05µ in thickness. It has a
special staining reaction when treated with Ehrlich’s or Ziehl’s
solution. It grows between 98° and 104° Fahr. (37° and 40° C.) in
various artificial media containing glycerine.