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CYBER SECURITY FOR BEGINNERS A COMPREHENSIVE AND ESSENTIAL

GUIDE FOR EVERY NOVICE TO UNDERSTAND AND MASTER
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 Computer Networking and
Cybersecurity
A Guide to Understanding
Communications Systems, Internet
Connections, and Network Security Along
with Protection from Hacking and Cyber
Security Threats
© Copyright 2020
All Rights Reserved. No part of this book may be reproduced in any form without permission in writing from
the author. Reviewers may quote brief passages in reviews.
Disclaimer: No part of this publication may be reproduced or transmitted in any form or by any means,
mechanical or electronic, including photocopying or recording, or by any information storage and retrieval
system, or transmitted by email without permission in writing from the publisher.
While all attempts have been made to verify the information provided in this publication, neither the author
nor the publisher assumes any responsibility for errors, omissions or contrary interpretations of the subject
matter herein.
This book is for entertainment purposes only. The views expressed are those of the author alone, and
should not be taken as expert instruction or commands. The reader is responsible for his or her own actions.
Adherence to all applicable laws and regulations, including international, federal, state and local laws
governing professional licensing, business practices, advertising and all other aspects of doing business in the
US, Canada, UK or any other jurisdiction is the sole responsibility of the purchaser or reader.
Neither the author nor the publisher assumes any responsibility or liability whatsoever on the behalf of the
purchaser or reader of these materials. Any perceived slight of any individual or organization is purely
unintentional.
 Contents
PART 1: COMPUTER NETWORKING
INTRODUCTION
CHAPTER 1: COMPUTER NETWORKING BASICS
What is Modern Computer Networking?
CHAPTER 2: NETWORKING SERVICES EXPLAINED
Domain Name System (DNS)
Dynamic Host Configuration Protocol (DHCP)
Hypertext Transfer Protocol (HTTP)
File Transfer Protocol (FTP)
Network News Transfer Protocol (NNTP)
Simple Mail Transfer Protocol (SMTP)
Keeping the Networks Safe with Firewalls
CHAPTER 3: GETTING INTO THE NETWORKING HARDWARE
FUNDAMENTALS OF COMPUTER RELATIONSHIPS
Core Hardware Elements of a Network
Servers
Hubs, Switches, and Routers
Cabling and Cable Plants
Workstation Hardware
Utilizing the Network Traffic and Knowing the Network Hardware
Repeaters
Hubs (Concentrators)
Switches
Bridges
Routers
Gateways
The Core Fundamentals of Cable Topologies
Star Topology
Ring Topology
CHAPTER 4: WIRELESS COMMUNICATION SYSTEMS AND CONNECTING TO THE
INTERNET
Connecting to the Internet
Wide Area Networks
Internet and Intranet
Understanding the Features of Networking
File Sharing
 Printer Sharing
Application Services
E-mail
Remote Access
CHAPTER 5: COMMON NETWORK PROTOCOLS AND PORTS
TCP/IP and UDP
Domain Name System (DNS), allowing users to access websites with easy-to-
remember domain names instead of long numerical IP addresses.
Dynamic Host Configuration Protocol (DHCP), for assigning TCP/IP
addresses to the nodes in a network.
Hypertext Transfer Protocol (HTTP), which controls the transfer of data
between the client and the webserver.
File Transfer Protocol (FTP), which defines the method by which file data is
sent and received between the FTP client and the FTP server.
Network News Transfer Protocol (NNTP), specifically used for Usenet
discussion groups on the Internet.
Simple Mail Transfer Protocol (SMTP), which handles the sending and
receiving of e-mails from one e-mail server to another.
CHAPTER 6: LEARNING ABOUT THE OSI NETWORKING MODEL
Layer 1: The Physical Layer
Layer 2: Data-Link Layer
Layer 3: Network Layer
Layer 4: Transport Layer
Layer 5: Session Layer
Layer 6: Presentation Layer
Layer 7: Application Layer
CHAPTER 7: NETWORK SECURITY, CYBERSECURITY, AND HACKING METHODS
Account Security
Password Security
File and Directory Permissions
Practices and User Education
Understanding External Threats
Front-Door Threats
Back-Door Threats
DDoS Threats
Potential Attacks and Threats
Basics to Hacking and Network Design
The Process of Designing a Network
Assessing Network Needs
 Applications
Users
Network Services
Meeting the Network Needs
The Basics of Hacking: The Kill Chain Process
Phase 1: Reconnaissance
Phase 2: Intrusion
Phase 3: Exploitation
Phase 4: Privilege Escalation
Phase 5: Lateral Movement
Phase 6: Obfuscation
Phase 7: Denial of Service
Phase 8: Exfiltration
CHAPTER 8: USEFUL CYBERSECURITY TIPS FOR 2020
Vital Function of a Disaster Recovery Plan
Assessing Disaster Recovery Needs
Considering Disaster Scenarios
Assessing Backup Needs
Acquiring Backup Media and Technologies
Choosing Backup Strategies
CONCLUSION
PART 2: CYBERSECURITY
INTRODUCTION
CHAPTER ONE: CYBERSECURITY AND WHY IT’S IMPORTANT
What is Cybersecurity?
Why is Cybersecurity Important?
Cybersecurity Scenarios
Cybersecurity Terminology
History of Cyberattacks
CHAPTER TWO: CYBERATTACKS AND 10 TYPES OF CYBER HACKERS
Classification of Cybercrimes
Reasons for Cybercrimes
Types of Cyber Hackers
Common Tools Used by Hackers
Types of Cybercrime
CHAPTER THREE: RECOGNIZING AND COUNTERING A PHISHING ATTACK
What is Phishing?
Methods used for Phishing
 Common Ways to Tackle Phishing Attacks
Types of Phishing and How to Protect Yourself against Them
Identifying a Phishing Email
Identifying a Spoofed Email
CHAPTER FOUR: HOW TO IDENTIFY AND REMOVE MALWARE
Types of Malware
Protecting Yourself from Malware
CHAPTER FIVE: RECOVERING FROM RANSOMWARE
Defining Ransomware
Types of Ransomware
CHAPTER SIX: HOW TO SPOT AND STOP A SOCIAL ENGINEERING ATTACK
Social Engineering Methodologies
Spotting a Social Engineering Attack
How to Avoid Falling for Social Engineering
Tips
Look out for Banking Scams
CHAPTER SEVEN: NETWORK SECURITY AND PROTECTION TECHNIQUES
Network Security
How Does the Network Security Function?
Top Five Attacks Through a Network that can Affect Your Business
Protecting your Business from a Cyber Attack
Cybersecurity through Configurations and Settings
Some Tips to Maintain Cybersecurity
CHAPTER EIGHT: WEB APPLICATION AND SMARTPHONE SECURITY
Web Application Security
How Can You Secure Web Applications?
CHAPTER NINE: 9 SECURITY TESTING METHODS
Types of Security Testing
Types of Penetration Testing
CHAPTER TEN: SKILLS REQUIRED FOR A CAREER IN CYBERSECURITY
Entry-Level Roles
Senior Level Roles
Four Popular Careers in Cybersecurity and How to Get There
Required Skillset for a Career in Cybersecurity
Technical Skills
CONCLUSION
REFERENCES
 Part 1: Computer Networking
An All-in-One Beginner's Guide to
Understanding Communications Systems,
Network Security, Internet Connections,
Cybersecurity and Hacking
 Introduction
This book is a concoction of several computer topics that are
relevant and popular in the modern age. Throughout this book, the
reader will explore each topic with easy-to-understand terminology
and analogies. The book starts with a descriptive and comprehensive
insight into computer networking and then branches off to cover
network security, network designing, and hacking. The delivery of
topics is specifically designed to:

Entertain the needs of the readers.

Provide valuable insights into the world of computer

networking in the modern era.

Deliver elaborative yet to-the-point knowledge.

Once the computer networking portion of this book is finished,

the reader will be greeted with the other topics in this all-in-one
guide. In the ending section, there is an informative description of
the kill-chain process in hacking. This topic is not only interesting but
also informative and refers to the methods hackers employ when
penetrating target systems and networks. So, buckle your seatbelts
and enjoy the ride.
 Chapter 1: Computer
Networking Basics
In this chapter, we will explore the fundamentals and the very basic
concepts of computer networking. By doing so, we will essentially
lay a conceptual foundation for the reader. This will help increase the
digestibility of the later discussed concepts and some technical
details of computer networking.
Hence, this chapter will be primarily focused on emphasizing the
basic details of computer networking and then steadily building upon
the knowledge as you learn.
To start things off, we will go through a brief introduction of what
computer networking actually is. Then we will delve deeper into the
details solidifying the understanding of each concept that the reader
will have successfully learned.
What is Modern Computer Networking?
The word “networking” describes a connection or set of connections
between multiple objects. Similarly, “computer networking” refers to
the interconnection of two or more computers together to allow for
sharing resources. A network of computers can be established in a
variety of environments, such as homes, offices, or even in large
business organizations. Computer networking stretches even to the
international scope where computers are connected through the
Internet. A more subtle and easily observed example of computer
networking is a printer or a scanner connected (wired or wireless) to
the user's personal computer.
Since the fundamental purpose of computer networking is to
interconnect different PCs, an interesting question arises; why do we
need computer networking in the first place?
The answer to this is very simple. If a person wants to watch a
movie, a video, or even a talk show, he can do so in two ways.
 By having a physical storage device that contains the
content, viewable on his computer, or simply by hopping
onto the Internet and accessing the website where he
can find the content and view it directly from there.

The latter approach involves computer networking. It

enables the viewer to connect to a remote computer
server that is the host of the website allowing the visitor
to use its resources to view his favorite content. This is
just a simple example of how computer networking
benefits an average user.

The phenomenon of the Internet is a by-product of computer

networking, and we all know what it’s like to spend a few days
without the Internet. While we have discussed how big a deal
computer networking is to a common person, it becomes even more
important and crucial for big organizations and institutes such as
hospitals, business companies, law enforcement departments, etc.
These organizations rely on computer networking to:

Efficiently establish an information channel through

which the entire organization communicates the data
being gathered and modified. Computer networking has
a huge influence on not only the professional sector but
also the business sector as well because, without a
proper networking infrastructure, the respective industry
would essentially just collapse.

Hence, to understand the importance and the very basic function

of computer networking, note that computer networking is the very
foundation of the Internet itself.
Transferring data from one place to another is the purpose of
networking. Another important thing we have to know about any
connection is how much data it can carry from one place to another.
This capacity to carry data is called bandwidth . If a connection has
a higher capacity bandwidth, it can carry more data within a specific
time slot.
The rate at which a network can transfer data is measured in bits
per second, which are abbreviated as ‘Bps.’ Bps means how many
bits a bandwidth can carry per second. In the modern age, computer
technology and networking have come a long way; this bandwidth
capacity is now measured in multiples of thousands. The different
measures of data transfer speed are:

Kilobytes - thousands of bits per second (Kbps)

Megabytes - millions of bits per second, or thousands

of Kilobytes per second (Mbps)

Gigabytes - billions of bits per second, or thousands of

Megabits per second (Gbps)

A closely related measurement you will also see in computer

networking is ‘hertz’; it is the number of cycles being carried per
second, in the same way ‘hertz’ expresses the number of cycles per
second in an alternating-current signal. Hertz is abbreviated as ‘Hz’
and is used to measure the computational speed of a processor. This
measurement is taken in multiples of thousands, and:

A thousand Hertz is known as a kilohertz (kHz)

A million Hertz is known as a Megahertz (MHz)

A billion Hertz is known as a Gigahertz (GHz)

For example, a processor running at 100 MHz means 100 million

cycles per second. Hz and bps are essentially the same
measurement but used for different elements. However, sometimes
they can be mixed up. For instance, saying that an Ethernet cable
supports a 10 MHz bandwidth instead of 10 Mbps is acceptable in
common usage.
Random documents with unrelated
content Scribd suggests to you:
 4 Collected Works, London, 1825, p. 523.

5 The Nervous System of the Human Body, London, 1830.

6 On the Brain and Spinal Cord, 1828, p. 419.

7 London Medical Gazette, vol. vii., 1830-31, p. 201.

8 Outlines of Human Pathology, London, 1836.

9 Archives générales de Méd., t. xxiv., Sept. and Oct., 1850.

Cruveilhier's studies were commenced as early as 1832, but his

results were not published until March, 1853,10 when he read his
memoir before the Academy of Medicine of Paris. He seems to have
made the first autopsy, and was much surprised at the absence of
any apparent lesion of the spinal cord. So enthusiastic and so
exhaustive was his study of the disease that his name, too, has
become almost inseparably associated with it, and the term
Cruveilhier's atrophy is one of those by which it is known. He
concluded from his earlier autopsies that the lesions were solely in
the muscular system, which is progressively destroyed, while the
brain and spinal cord may remain perfectly normal. In a later case
(his third), terminating January, 1853, he found atrophy of the
anterior roots of the spinal nerves, and then concluded that the
disease resided "not in the muscles themselves, but in the anterior
roots of the spinal nerves." But after the termination of his fourth
case, in which an autopsy was also secured, he placed the primary
lesion in the gray matter of the cord, whence he considered the
anterior roots take their origin.
10 Ibid., May, 1853, p. 561.

Thouvenet,11 an interne of Cruveilhier's, published in 1851 a thesis

based on some cases collected in the Charité, and was the first to
claim that the disease resides primarily in the peripheral nerves, and
that it must be classed among rheumatic affections.
 11 Gaz. des Hôp., Nos. 143 and 145, 1851.

In December, 1851, E. Meryon12 read a paper before the Medico-

Chirurgical Society of London entitled "Granular and Fatty
Degeneration of the Voluntary Muscles." His observations appear to
have been made quite independently of any preceding researches.
He argues that the primary morbid change is a default of nutrition in
the muscular fibres.
12 Med.-Chir. Trans., vol. xxxv. p. 73.

Subsequently, cases were published in 1853 by Bouvier, Landry,

Burg, and Niepce in France; in 1854 by Chambers in England, Guérin
and Robin in France, Cohn, Virchow, and Betz in Germany, and by
Schneevogt in Holland; in 1855 laborious essays were published by
Oppenheimer, Wachsmuth, and Eisenmann, and cases by Hasse,
Valentiner, Virchow, Meyer, and Diemer in Germany, and Gros in
France. Duchenne's work on Local Application of Electricity, also
published in 1855, contains much information on the subject.

Since 1855 the reports of cases and papers on the subject have
been so numerous as to make it unprofitable to enumerate them.
Among the most notable are those of Eisenmann, published in
Canstatt's Jahresbericht for 1856; Roberts's classic work on Wasting
Palsy, in 1858; the papers of Lockhart Clarke in 1866 and 1867,13
and of Swarzenski in 1867;14 Kussmaul's clinical lecture15 and
Friedreich's treatise16 in 1873; and Eulenburg's article on
"Progressive Muscular Atrophy" in Ziemssen's Cyclopædia of Practical
Medicine, published in German in 1875 and in English in 1877. An
important case, in consequence of the careful post-mortem study of
the nervous tissues, is one recently reported by Wood and Dercam.17
13 Med.-Chir. Transactions, xlix., 1866, p. 171, and l., 1867, p. 489.

14 Die Progressive Muskelatrophie, Berlin.

 15 "Ueber die fortschreidende Bulbärparalyse und ihr Verhältniss zur progressiven
Muskelatrophie." Sammlung klinische Vorträge, liv.

16 Ueber progressive Muskelatrophie, über wahre und falsche Muskelhypertrophie,

Berlin, 1873.

17 Therapeutic Gazette, March 16, 1885.

ETIOLOGY.—The cause of this affection in a large number of cases is

quite unknown. That hereditation plays an important part seems well
determined by numerous observations, among which may be
mentioned those of Roberts, Friedreich, Hemptenmacher, Trousseau,
Meryon, Eulenburg, Sr. and Jr., Naunyn,18 Hammond, and Osler.19 In
the Farr family, reported by Osler, 13 individuals in two generations
have been affected, 6 females and 7 males—a larger proportion of
the former than is common in this disease. Of these 9 had died at
date of publication of paper. With the exception of two, all occurred
or proved fatal after the age of forty. Of the 10 instances in the
second generation, 5 are the offspring of males and 5 the offspring
of females. The disease has not yet appeared in the third
generation, which promises between forty and fifty individuals,
several of whom are over thirty years of age.
18 Berliner med. Wochenschrift, Nos. 42 and 43, 1873.

19 Archives of Medicine, vol. iv., No. 3, Dec., 1880.

The over-use of the muscles involved seems to be a well-determined

cause in certain cases of true muscular atrophy. The following
interesting illustrations are given by Eulenburg:20 Betz observed
atrophy of the side three times in the cases of smiths and saddlers,
who had to do heavy work with the right hand; Gull, in a tailor after
excessive exertion; Hammond reports a case apparently due to
excessive use of one thumb and finger in playing faro; Friedreich,
one of a dragoon who may have exhausted his left hand in holding
the bridle while riding; another in a morocco-leather worker, who
used to press hard with his left hand; and a musician who played
several hours a day on the bass viol. Schneevogt names two cases
of primary atrophy of the shoulder-muscles, especially of the deltoid
of the right side—one of a sailor who had to pump for days together
on a leaking ship, and the other of the left side in a woman who
always carried her child on the left arm while suckling it. Continued
threshing and the handling of a musket have both been followed by
it in the muscles called into play by these exercises. Roberts was
able to trace the effects of over-muscular exertion in producing the
disease in 35 out of 69 cases. As a determining cause, at least,
therefore, we must admit the over-use of muscles.
20 Op. cit.

There is reason to believe, too, that this form of atrophy is one of

the consequences of senility—that the tendency to connective-tissue
overgrowth which characterizes old age operates to produce, in a
way to be presently explained, an atrophy of groups of muscles. In a
woman aged seventy, now under my care, the fingers of both hands
are clawed—became so inappreciably almost, and the condition is
still increasing.

In addition to the above-named causes, long-continued exposure to

cold, and especially to the action of very cold water, has been
named. Traumatic influences, such as injuries to nerve and muscle,
have been called upon to account for localized and progressive
atrophy, but these are excluded by our definition from the category
of true progressive muscular atrophy.

Cases have also occurred in the course of convalescence. Typhoid

fever, rheumatism, measles, scarlet fever, cold during salivation,
vaccination, childbed, excessive venery, syphilis,—have all been held
responsible for a certain number of cases.

AGE AND SEX.—In examining the literature of acute muscular atrophy

it is found that cases are reported at all ages. Thus, Wachsmuth,
quoted by Eulenburg, found among 49 cases 13 under the age of
fifteen, 8 from fifteen to twenty, 22 from twenty to fifty, and only 6
over fifty years. On the other hand, Roberts—who, following Aran,
divides the disease into the general form and partial form—says the
latter very rarely falls on individuals under adult age or over fifty,
while the average age of the instances of the partial form studied by
him was thirty-two years and four months. In 10 instances of the
general form the patients were under twelve, and 2 more are
reported as children; 1 was said to be sixty-nine and another fifty-
four, the average being twenty-eight years and three months. Of
Eulenburg's own cases, 7 acquired the disease before the age of ten,
6 before the twentieth year, 2 before the thirtieth, 8 before the
fortieth, 5 before the fiftieth, and none later. The latter observer also
finds that whenever the disease is hereditary it occurs earlier, usually
before the close of the twentieth year. This was certainly not the
case in the Farr family, reported by Osler.

I am inclined to believe, especially in the light of Charcot's21 and of

Erb's22 recent studies, that the true spinal form of progressive
muscular atrophy is a disease of adult life, and that the majority of
cases reported as occurring in early life are instances either of what
Erb calls the juvenile form of progressive muscular atrophy or of
pseudo-hypertrophic paralysis.
21 "Revision nosographique des Atrophies musculaires progressive," Le Progrès méd.,
No. 10, 1885, i. 314-335.

22 "Ueber die Juvenile Form der Progressive Muskelatrophie und ihre Beziehungen zur
sogenannten Pseudohypertrophie," Deutsches Archiv für klin. Med., xxxiv. 1884, S.
467.

As to sex, males predominate. Thus, according to Friedreich's

statistics, out of 176 cases but 33 were females, or about 19 per
cent. Of Roberts's collection of 99, 84 were males and 15 females.
Of 28 cases noted by Eulenburg, 17 were in men and 11 women.
This is doubtless owing to the fact that men are subjected to the
causes of the disease more than women. For Roberts early noted
that women who engage in needlework, washing, and household
service are apparently not less liable than men similarly employed,
and he found that of those whose labor did not press excessively on
any particular sets of muscles females formed even a majority of
cases.

Some singular freaks of selection have presented themselves in the

matter of sex, particularly in the cases which have been ascribed to
hereditation. Thus it will sometimes attack only the male members
of a family. A remarkable instance of this was observed by Meryon,
in which four sons were attacked and six daughters remained
unaffected; and, again, two boys were attacked and two sisters
escaped. This may occur also independent of hereditation.
Occasionally the reverse takes place, the sisters only being attacked,
while the brothers escape.

PATHOLOGICAL ANATOMY AND HISTOLOGY.—Two principal seats of change

have been found to exist in connection with progressive muscular
atrophy. The first and easiest recognized is, of course, the alteration
in muscles; the second, that in the nervous system.

The muscular change is simple, and affords a typical instance of

what is known as numerical atrophy. The muscular fasciculi one after
another undergo fatty metamorphosis, succeeded by absorption of
the resulting fat and substitution of connective tissue. The rate of
atrophy varies, but sooner or later the muscle is more or less
substituted by fibrous bands and cords, over which may be traced
reddish lines which represent muscular tissue in a normal state.

The rationale of these changes has not been always the same. The
older observers regarded them as the result of a primary fatty
metamorphosis of muscular fasciculi, followed by absorption of the
resulting fat. Later it was asserted that the atrophy is secondary to a
myositis or inflammation of muscle, beginning as a hyperplasia of
the interstitial connective tissue in its finest ramifications between
the single primitive fibrils. Along with this are seen the results of
irritation in the primitive bundles themselves, shown by swelling and
multiplication of the muscular corpuscles, proliferation of their nuclei,
and sometimes cloudy swelling. Even hypertrophied muscular
fasciculi and dichotomous and trichotomous subdivision have been
noted by Friedreich.

It sometimes happens that the hyperplastic process in the

intermuscular connective tissue is succeeded by a fatty infiltration of
the cells of the connective tissue, and there results a lipomatosis
which is invariably outside of the muscular fasciculi and between
them. This gives rise to an appearance of hypertrophy which is only
apparent, for the muscular fasciculi are themselves wasted, and
proportionally paralytic. This is seen to occur particularly in the
muscles of the calves of the legs, in which is produced an
appearance identical with that in the disease known as pseudo-
hypertrophic muscular paralysis, with which, indeed, the condition
under consideration is considered by some identical. But although
we must admit in certain cases a complication of a certain degree of
lipomatosis with progressive muscular atrophy, the two diseases are
essentially different; and it is quite likely that in some instances
pseudo-hypertrophic muscular paralysis has been mistaken for
progressive muscular atrophy.

The changes in the nervous system are not nearly so simple. They
have been noted in the peripheral nerves, both in their trunks and in
their intermuscular branches; in the anterior roots of the spinal
nerves; and in different parts of the spinal cord, including the central
gray matter, the antero-lateral and posterior columns; also in the
sympathetic system. These nerve-changes are not simultaneous, nor
have they been discovered in every case. It is a noteworthy fact,
however, that as methods of examination have improved and the
manipulative skill of observers has increased the number of negative
cases has diminished.

First, as to alterations in peripheral nerves in their ultimate

distribution: The character of these is of a kind usually described as
irritative; that is, there is a hyperplastic process in the connective-
tissue sheaths (neurilemmæ) and their internal prolongations,
consisting in nuclear proliferation and thickening of the tubular
membrane or sheath of Schwann. Varicose distortion of the
medullary sheaths and their subsequent disappearance, together
with destruction of the axis-cylinders, also occurs.

The changes in the peripheral nerve-trunks, as studied in the

median, ulnar, radial, and musculo-spinal, are essentially the same,
resulting in thinning of the diameters of the nerves. These changes,
however, are by no means constant.

The anterior roots of the spinal nerves exhibit alterations in a large

number of instances. Cruveilhier called attention to them in the
celebrated case of the rope-dancer Lecomte. At the autopsy, the
brain, the cord, and posterior roots were found normal, but the
anterior roots, from the point of exit to where they unite with the
posterior, were greatly atrophied. In another case the anterior roots
were to the posterior in thickness, in the cervical region, in the ratio
of 1:10, while the normal ratio is 1:3; in the dorsal region as 1:5,
while the normal is as 1:1½ or 2. The posterior roots, brain, and
cord were again unchanged. Up to 1876, Eulenburg had collected 26
cases in which this alteration existed, and 19 in which it was absent.
In the case of Wood and Dercum, referred to, this atrophy of the
anterior nerve-roots existed, making 27 positive cases and 19
negative.

We come, finally, to the spinal cord as the seat of changes, and we

are met by Eulenburg's statistics, according to which, up to the date
of his article, there were 34 cases of positive disease and 15
negative. To the former we have again to add the case of Wood and
Dercum, making 35 against 15. These alterations are by no means
constant as to seat and character. Thus, Valentiner, who seems to
have been the first after Cruveilhier, in 1853, to record any, found in
1855, in the centre of the cord, in the neighborhood of the three
lowest cervical and upper dorsal nerves, that the elements in the
region of transition from gray to white substance were obliterated,
and the softened place contained numerous compound granule-cells.
Schneevogt also found a softening of the cord from the fifth cervical
to the second dorsal nerve, Frommann described a red softening
from the medulla oblongata downward, involving chiefly the anterior
and lateral columns, and especially the commissures and the
innermost parts of the anterior columns lying next the commissure.

Luys found the gray matter in the neighborhood of the cervical

enlargement full of hyperæmic vessels, which were surrounded with
granular masses (compound granule-cells?). The same granular
masses, together with numerous corpora amylacea, were scattered
throughout the gray substance. The ganglion-cells of the anterior
cornua had almost disappeared in the part affected, and appeared to
be replaced by the granular masses. Here and there a few ganglion-
cells could be recognized in a state of retrograde metamorphosis,
pigmented and bereft of their polar prolongations. In this case the
degeneration affected principally the left anterior cornu, and it was
the left side of the body which was affected by the atrophy. The
anterior roots of the spinal nerves on the left side were also
atrophied. Lockhart Clarke found essentially the same changes in no
less than six cases, and Duménil, Schueppel, Hayem, Charcot (six or
seven autopsies), Joffroy, and lately Wood and Dercum,23 have
added others. The last two observers found changes in the lower
portion of the cervical enlargement of the cord, and state in the
report of their case that "in the anterior cornua of the gray matter
there is a marked diminution in the number of nerve-cells. Of the
three groups of these cells, the anterior has almost entirely
disappeared, the lateral group is represented by but a few individual
cells, while the internal group seems to have undergone a less
marked change. All of these cells, with the exception of a few in the
internal group, appear shrunken, and are evidently much diminished
in size. They have lost in great part their polygonal shape, many of
them being fusiform, and present but few processes. Only in the
internal group are these cells in any way approaching the normal
type, and these are few and seen in only a few of the sections. They
present the characteristic size and numerous processes of the typical
motor-cell, while they disclose a well-defined nucleus and nucleolus.
In the atrophied cells the nuclei can only be distinguished with
difficulty.

"The neuroglia of the anterior cornua is increased in amount; the

vessels appear shrunken, with thickened walls and large perivascular
lymph-spaces.

"In the lumbar cord the cells in the anterior cornua appear normal:
in this respect the lumbar cord is in marked contrast with the
cervical."
23 Loc. cit.

Another class of cases recorded by Gull,24 Schueppel and Grimm,

Hallopeau and Westphal, consist in dilatation of the central spinal
canal with more or less complete destruction of the gray substance,
and in Grimm's case hyperplasia of the connective tissue in the white
substance along with increase of the axis-cylinders. The nerve-roots
were in a state of fatty degeneration, especially the finer fibres of
the anterior roots.
24 Guy's Hospital Reports, 1862.

Still another set of observations discovers a degenerative atrophy of

the white columns only of the cord, sometimes the antero-lateral
columns and sometimes the posterior. Virchow, Friedreich, and
Swarzenski each found typical gray degeneration of the posterior
columns, in one instance recognizable by the naked eye. Atrophy of
the antero-lateral columns was noted by Frommann and
Baudrimont; atrophy of the antero-lateral columns, conjoined with
inflammatory changes in the gray substance and atrophy of
ganglion-cells, by Duménil; changes in the antero-lateral gray
substance and posterior columns by Clarke. Changes have even
been found in the posterior cornua and posterior nerve-roots in a
few cases, although not confined to them.
Finally, the lesions of this singular disease have been sought also in
the sympathetic, and not without some success. Eulenburg's analysis
discovered 5 positive observations and 14 negative ones. To the
positive must be added the case of Wood and Dercum, who reported
a marked increase in the amount of connective tissue and a granular
state of the ganglion-cells without diminution in number. Among the
changes in the sympathetic were thinning of its trunk and of the two
upper ganglia observed by Swarzenski, and advanced fibrous fatty
change of the cervical and thoracic portion, with abundant
hyperplasia of connective tissue, disappearance of nerve-fibres and
regressive metamorphosis of ganglion-cells by Duménil.

PATHOGENY.—We come now to consider the relation of these changes

to the muscular atrophy which constitutes the conspicuous symptom
of the disease. There are three possible views of the pathology of
this affection. According to one, it is a muscular or myopathic
disease in the strict sense of the term. Such muscular disease may
be primarily inflammatory, a myositis—as Friedreich sought to prove
in his great work—followed by fatty metamorphosis of the sarcous
substance and subsequent absorption of the fat; or it may be a
simple fatty metamorphosis. According to a second view, it is
primarily an affection of peripheral nerves or of the anterior roots of
the spinal nerves, with secondary muscular atrophy. According to a
third, it is a disease of the spinal cord, and more particularly of the
anterior cornua of the gray matter—a poliomyelitis anterior.

A careful study of the morbid conditions as described in the various

cases reported leads me to adopt the last view. In the first place, the
number of instances of positive disease of the spinal cord exceed
those of any other seats of alteration, and although the changes do
not always involve the anterior cornua, yet it will be noted, from an
examination of the foregoing paragraphs, that a decided majority
involve either the anterior cornua alone or these in connection with
the antero-lateral columns, the number of cases of disease of the
antero-lateral columns alone or of the posterior columns and
posterior nerve-roots being very limited. Again, the number of
instances in which lesions of the anterior cornua are found increases
as our means of accurate investigation improve.

If we add to these considerations the fact that the symptoms are

best explained by such a view, little more seems required to
establish it. Recalling the well-known observation of Waller,
confirmed by Bernard and others, that after section of the anterior
root of a spinal nerve the distal end wastes, while the central end
remains intact, because it is still connected with its own trophic
centre, we have in this the explanation why atrophy of the anterior
roots is also so common a symptom in progressive muscular atrophy.
The fibres of the anterior roots arise from the cells of the anterior
cornua, and disease of the latter must unfavorably influence the
nutrition of the former; hence their atrophy. This atrophy of motor
nerve-filaments is continued into the mixed nerves distributed to
muscles, but is less easily demonstrable by reason of the gradually
diminishing size of the nerve-trunks and by the fact that they are
united in the mixed nerve with the sensory fibres from the posterior
roots, which do not suffer atrophy. In consequence of the
degeneration of these nerves follows degeneration of the muscles to
which they are distributed, so that the alterations in the latter are
altogether secondary.

From this point of view the disease in question is to be regarded as

a chronic form of poliomyelitis anterior, while the essential infantile
paralysis of Rilliet and Barthez would correspond to the acute form
of the disease.

The association of changes in the anterior roots with others in the

spinal cord may be explained either on the ground of extension by
continuity to adjacent parts, or on that of coincidence. In illustration
of the latter I may refer to a case recently reported from Mendel's
clinic25 in Berlin, in which the symptoms of progressive muscular
atrophy were associated with those of tabes dorsalis or progressive
locomotor ataxia. Here it is not unlikely that the coincidence is
merely accidental; and this was Mendel's opinion in this case. In
other instances the involvement of other portions of the spinal cord
may be a result of an extension of the disease from its true seat,
while many cases described as progressive muscular atrophy are not
such at all, but are in part the result of other affections of the spinal
cord. It is evident, also, that this order may be reversed, as in a case
reported by Eulenburg26 to the Berlin Medical Society.
25 Philada. Medical News, Sept. 12, 1885, p. 188.

26 Berliner klin. Wochenschr., No. 15, April 13, 1885.

SYMPTOMS.—The first distinctive symptom of the disease under

consideration is the muscular atrophy or wasting. However general it
may subsequently become, it is at first localized. The upper
extremity is by far the most frequently involved—7 out of 9 times in
Aran's cases. Sandahl out of 62 cases found the right upper
extremity attacked 37 times, the left in 14 instances, and both in 11.
In Friedreich's statistics it occurred first in the upper 111 times out
of 146, while the lower was invaded 27 times, and the lumbar
muscles 8. Most frequently it begins in some muscle or group of
muscles in the right hand, either the interossei or those of the ball of
the thumb. Of the interossei, the external interosseus is usually the
first affected. Thence it extends to the other interossei, and soon
very striking depressions make their appearance between the
metacarpal bones, and the extensor tendons on the dorsum, and the
flexors in the palm become as distinct as if dissected out.
Succeeding this follows contraction of the flexor tendons until the
picture seen in Fig. 32 is produced, in which 1 exhibits the anterior
surface of the hand, and 2 the posterior.

FIG. 32.
 (1) HAND, PALMAR SURFACE. (2) DORSAL SURFACE (after Duchenne).

a, Ends of the metacarpal bones; b, Tendons of the flexor sublimis; c, Muscles

of the ball of the thumb.

Opinion is not unanimous as to whether the atrophy when beginning

in the hand involves first the thenar muscles or the interossei.
Roberts, Wachsmuth, and Friedreich say that it begins, as a rule, in
the thumb; Eulenburg, that it invariably begins in the interossei.
From the interossei it may creep up the forearm, and thence to the
arm, or it may skip the forearm and pass into the arm, although the
triceps extensor muscle is usually spared. It may come to a standstill
in either of those places, but may involve the muscle of the shoulder,
especially the deltoid. When the latter and the arm are involved, a
picture like that of Fig. 33 is produced.

FIG. 33.

Showing Atrophy of the Right Deltoid and Arm, and the Left Arm.
 FIG. 34.

Showing Atrophy of the Deltoid, posterior aspect, and the Scapular Muscles.

Beginning most frequently in the right, both upper extremities

become sooner or later involved.

In other instances in which the upper extremities are previously

involved the atrophy begins in the shoulder, in the deltoid—here
again the right first. Succeeding the deltoid, the scapular and
trapezius muscles may be involved in any order, while a
grotesqueness of effect is often produced by reason of certain
adjacent muscles retaining their natural size or even being
hypertrophied. This is particularly the case with the anterior part of
the trapezius, which is almost never involved. With the shoulders
first affected, the arm and forearm may retain their usefulness and
strength; but the power of lifting the arm from the side, and
especially of raising it above the head, is lost. And if the patient
wishes to lay hold of anything, he must swing his arm forward with a
jerk until it is brought in reach of his fingers, and then it must often
be caught up by the pathologically hooked terminations of these.

The muscles of the trunk do, however, become at times involved—

the pectorales, the latissimi, serrati, and intercostales, and even the
diaphragm and abdominal and lumbar muscles. Life is seriously
jeopardized when the intercostals and diaphragm are affected, in
consequence of interference with respiration. If the intercostals
cease to contract, the upper part of the thorax ceases to move, and
if the diaphragm is involved, the epigastric and hypogastric regions
are drawn in during inspiration, and talking and singing are
interfered with. Even a mild bronchitis is apt to be fatal in
consequence of the difficulty in expelling the secretions.

The muscular atrophy thus produced is generally accompanied by a

corresponding wasting and retraction of the skin, so that this
continues applied to the muscles in the usual manner. In some
instances, however, this is not the case, and in these a baggy
condition of the skin is added, which gives its subject an appearance
which has more than once rendered him valuable to the showman as
the elastic-skin man, etc. It sometimes happens, on the other hand,
that the atrophy is obscured by an accumulation between the muscle
and skin of adipose tissue, and an appearance of hypertrophy rather
than atrophy may be produced in consequence, analogous to the
same state of affairs in pseudo-hypertrophic paralysis, the relations
of which disease to progressive muscular atrophy will be considered
under the head of Diagnosis.
At almost any stage the disease may come to a standstill, and may
continue thus for many years. The time required to attain its various
degrees also varies greatly, but the spread is usually slow, requiring,
as a rule, years for its completion. A general involvement of the
voluntary muscles of the entire body is exceedingly rare.

As stated, the disease may begin in the lower extremity, but much
more rarely. It is very seldom that the same order of invasion
pursued in the upper extremity is followed in the lower—that is,
beginning with the interossei. It may begin in the thigh and involve it
alone, or extend to both thighs, or both legs as well. Under these
circumstances weakness of the legs is a striking symptom, the
patient being unable to stand, often falling down or requiring a cane
or crutches to assist him. In illustration of this mode of invasion may
be related one of Roberts's cases, that of an adult woman thirty-
eight years old, a domestic servant, in whom at thirty-six was
perceived a weakness in the right thigh. She first noticed that it
grew tired sooner than the left. This gradually increased, until she
was compelled to sit much of the day, then to use a stick, and finally
crutches. This was accompanied by a gradual wasting of the thigh-
muscles. Even in this case the loss of power was greater than would
have been expected from the degree of atrophy, the loss of bulk
incident to which Roberts believed to have been in part replaced by
fat. In other instances, however, the extremest degree of atrophy
has been noted where the disease has commenced in the lower
extremities.

The deformity produced by the wasting muscle is sometimes further

increased—more frequently in the earlier stages—by a painful
swelling of the joints, first mentioned by Remak, called by him
neuro-paralytic inflammation, and referred to the sympathetic. This
may affect the small (phalangeal) as well as the larger joints
(shoulder and elbow).

Cases apparently beginning in the face are reported, when the

distorted expression resulting is very characteristic.
Aran first, and Roberts afterward, divided cases of the disease into
two groups, the partial and general. In the former are included those
involving the extremities only; in the latter become involved, sooner
or later, the muscles of the trunk, neck, face, mouth, pharynx
(muscles of deglutition), thorax (muscles of respiration), and even of
the abdomen. Even the tongue is reported as undergoing atrophy.

General wasting palsy, as was early observed by Roberts, is

unquestionably a rare disease, and in no case have all the muscles
of the body been found implicated in one individual, and a few seem
altogether exempted. Such are the muscles of mastication and of the
eyeball, including the levator palpebræ.

A second muscular symptom, more or less distinctive, is fibrillar

contraction. This consists in a wave-like contraction running along
small bundles of muscular fasciculi. The contractions occur
spontaneously or are excited by any slight stimulus, as a breath of
air or a dash of water, or by tapping the patient, or passing a
galvanic current through the parts, and at any stage of the disease,
except that they do not occur in muscles wholly destroyed.
Sometimes they can be felt by the patient. At other times he is
wholly ignorant of them. They are not invariably present, and often
they have been observed in muscles atrophied from other causes.
They possess, however, a certain amount of diagnostic value,
especially when spontaneous.

More rare, and less destructive, are cramps, twitches, and clonic
contractions of groups of affected muscles. These, when present,
are sometimes exceedingly painful.

Coincident with the wasting of muscles is their loss of function. The

power of abducting and adducting the fingers gradually disappears,
so also that of flexion and extension, and everywhere the loss of
function goes pari passu with the atrophy. As Roberts graphically
puts it, "The tailor discovers that he cannot hold his needle; the
shoemaker wonders he cannot thrust his awl; the mason finds his
hammer, formerly a plaything in his hand, now too heavy for his
utmost strength; the gentleman feels an awkwardness in handling
his pen, in pulling out his pocket handkerchief, or in putting on his
hat. One man discovered his ailment in thrusting on a horse's collar;
another, a sportsman, in bringing the fowling-piece to his shoulder."

Along with the atrophy of muscle and loss of power comes a

gradually diminishing response to electrical stimulus. Direct muscular
faradization fails first to excite contraction, and sometimes fails
completely even before voluntary mobility is lost. Indirect muscular
faradization continues longer to excite contraction, but it also finally
fails. Response to the constant current continues still longer, but it
also finally fails to elicit contractions, stronger and stronger currents
being required, until finally all fail. The galvanic excitability of nerve-
trunks is maintained for quite a long time, but finally also
disappears. Some irregularities present themselves in this respect.

A singular electrical reaction, first described by Remak, and said by

him to be of frequent occurrence in muscular atrophy, was named by
him deplegic contraction. He describes it as follows: When the
cathode or negative pole is put below the fifth cervical vertebra,
contractions can be produced in the atrophied muscles of the arm
when the anode or positive pole is placed in an irritable zone, which
extends from the first to the fifth cervical vertebra, or, still better, in
the carotid fossa or the triangle between the lower jaw and the
external ear. The contractions always take place on the side opposite
to that at which the anode is placed, while when the electrodes are
placed on the median line they occur on both sides, although when
the current is very weak they are limited to the muscles most
seriously involved. Meyer, Drissen, and Erb confirmed Remak's
statement, while Fieber, Benedikt, and Eulenburg failed to do so.
Remak interprets these contractions as reflected from the superior
cervical ganglion of the sympathetic. He bases this view upon the
fact that the patient perceived a sensation behind the ball of the eye
when the current was closed. Eulenburg, on the other hand, regards
them as genuine reflex contractions, independent of the
sympathetic, and caused either by excessive irritability of the central
reflex apparatus or by an abnormal excitability of the muscles
themselves.

Sensibility is, in many cases, unchanged, the tactile sense being as

delicate as ever, and pain, except accompanying the cramps above
described, is absent. At times, however, the atrophy is preceded by
paroxysms, which may or may not accompany the clonic
contractions referred to. It is sometimes in the course of nerve-
trunks, but as often diffuse, as though the muscles themselves were
its seat. At other times it is variously described as a soreness, an
aching, or a rheumatic pain. Accompanying advanced degrees of the
atrophy, however, there is very rarely—in 3 out of 105 cases,
according to Roberts—a slight diminution of sensibility, especially in
the ends of the fingers, while the faradic sensibility may be similarly
diminished.

Modified sensations, as those of cold, numbness, and formication,

may be experienced, and reflex excitability may be increased, while
the knee-jerk is said to be absent. Unusual sensitiveness to cold is
sometimes noted, and a loss of muscular power under its influence,
which is again restored by artificial warmth.
Among more inconstant symptoms, denominated vaso-motor, are, in
the early stages, fever and slight elevation in local temperature from
2° to 3° C. Fever is less frequently observed toward the termination
of the disease, and at this stage a fall of local temperature, as much
as 4° C., has been noted. In the same category of vaso-motor
symptoms are classed the skin contractions already referred to,
hyperidrosis or excessive sweating, and certain very rare oculo-
pupillary symptoms, consisting mainly of contraction of the pupil and
slow reaction, but including also, in a case reported by Voisin,
flattening of the cornea on both sides and defective sight.

COMPLICATIONS.—Progressive muscular atrophy is not infrequently

associated with amyotrophic antero-lateral sclerosis and with labio-
glossal or progressive bulbar paralysis. Both affections may result as
an extension of the disease from the anterior cornua of gray matter,
the former into the antero-lateral columns, the latter into the
medulla oblongata, or the affection may be primary in either of
these two situations, and extend thence into the anterior cornua of
gray matter.

When there is also lateral sclerosis, there is rigidity of the lower

limbs in addition to the atrophy of the upper—at first temporary, but
afterward permanent. This may extend to the upper also, and the
arms become fixed in semipronation and semiflexion.

When there is bulbar paralysis there is difficulty in moving the

tongue, in speaking, and in swallowing. The mouth remains open,
the lower lip drops, the patient cannot whistle or kiss or blow out a
candle; he speaks through his nose. On the other hand, the upper
part of the face is natural, the orbicularis palpebrarum muscle and
occipito-frontalis acting well. As a consequence, the carrying of the
food back into the oesophagus is rendered difficult or impossible;
swallowing is imperfectly successful; the food sometimes enters the
larynx, and the patient dies of suffocation. The saliva dribbles from
the mouth. Later, respiration is embarrassed, and performed
principally by the diaphragm; there is difficulty in raising mucus, and
if bronchitis supervenes the patient dies of suffocation, because he
cannot raise the phlegm. Such was the death of Prosper Lecompte,
the historic patient of Cruveilhier.

DIAGNOSIS.—As our knowledge of progressive muscular atrophy

increases we realize more and more that there have heretofore been
included under this name many cases which must now be relegated
to other categories. If we confine the disease, as I think we must, to
those cases in which there are degenerative changes in the anterior
cornua of the gray matter of the cord, we must endeavor to
associate with these lesions a set of symptoms which are sufficiently
constant, and exclude all other similar combinations. Such a set of
symptoms includes the following: insidious and progressive atrophy
of groups of muscles, beginning usually in the hand or shoulder,
from which, however, it may extend to others in a diffuse and rarer
form of the disease. The atrophy is accompanied by a corresponding
loss of power in the affected muscles and partial or complete
reaction of degeneration in the same, and by fibrillar twitchings.
Along with this, sensibility, the special senses, the reflexes, as a rule,
and sphincters always remain normal.

This complex of symptoms is to be distinguished from the so-called

juvenile progressive muscular atrophy of Erb, and from pseudo-
hypertrophic muscular paralysis. In the first there is also slow,
symmetrical, but intermittent and often stationary, wasting and
weakness of certain groups of muscles, preferably those encircling
the shoulder and upper arm, the pelvis and upper thigh and back
—"an atrophy," says Erb, "which is very frequently combined with
true or false muscular hypertrophy, with a peculiar toughness of the
atrophying muscles, but without fibrillar contraction or any trace of
the reaction of degeneration or other lesion in the body, be it of the
nervous system, organs of sense, vegetative organs, or external
integuments."27 The average age in the juvenile form is much less,
Erb's cases ranging from seven to forty-six, or an average of twenty-
six and a half, while in the spinal form, or true progressive muscular
atrophy, although the age is reduced by reason of the admixture of
other cases than those of true progressive muscular atrophy, the
average age is much greater. Of Roberts's cases, all of which seem
true cases, the youngest was twenty, while the age of the remaining
four was thirty-nine, forty-seven, sixty-seven, and thirty-eight.
27 "Juvenile Form der Progressive Muskelatrophie," Deutsches Archiv für klinische
Medizin, Bd. xxxiv., 1884, S. 471.

There are certain symptoms in common in progressive muscular

atrophy, as heretofore described, and pseudo-hypertrophic paralysis;
and I have already said that Friedreich and others are disposed to
consider them one and the same disease; but such is not the case.
First of all, while there is wasting of muscle, although obscured in
the lower extremities by the fatty infiltration, and while there is loss
of power, there are in pseudo-hypertrophic paralysis absolutely no
alterations in the spinal cord. Pseudo-hypertrophic paralysis always
begins in the lower extremities, while progressive muscular atrophy
begins for the most part in the upper. Pseudo-hypertrophic paralysis
is a disease of childhood, and strikingly hereditary; and while
progressive muscular atrophy in its broadest application is also a
disease of childhood and hereditary, it is much less so than pseudo-
hypertrophic paralysis; and if, with Erb, we separate the juvenile
form from muscular atrophy, progressive muscular atrophy is not a
disease of childhood, while heredity is almost entirely removed from
it.28
28 It cannot but help the reader to get a correct notion of this interesting but still
somewhat imperfectly understood disease to be familiar with Erb's formulated
conclusions (loc. cit., p. 510):

"There is a peculiar form of progressive muscular atrophy which is characterized by a

definite location, definite course, definite behavior of affected muscles, and definite
alteration in them, but without alterations in the spinal cord—the condition named by
me the juvenile form. It begins in youth or childhood.
 "This form agrees in its symptomatology—especially in its localization in the upper
half of the body, partly also in the lower—entirely with the so-called pseudo-
hypertrophy of muscles, only that in the former a decided lipomatosis leading to an
increase in volume is wanting; on the other hand, true muscular hypertrophy is not
infrequent in both forms of the disease.

"If this juvenile form occurs in the earliest childhood, it may in all its details be
identical with pseudo-hypertrophy, except that the lipomatosis is wanting.

"The anatomico-histological alterations of the muscles are exactly the same in the
juvenile form as in pseudo-hypertrophy.

"The juvenile form not infrequently occurs in entire groups in one family, producing
the so-called hereditary—better named family-muscular—atrophy.

"If this juvenile-hereditary form occurs after puberty, it affects most frequently,
although not exclusively, the upper half of the body. If it sets in, on the other hand, in
earliest childhood, it affects preferably the lower extremities and the pelvis.
Transitional forms, however, occur also in family groups.

"In the latter form, that occurring in earliest life, we have that which Leyden has
proposed to designate as hereditary muscular atrophy.

"Thus, hereditary muscular atrophy is in all essential points identical with pseudo-
hypertrophy, and is distinguished from it only in the slighter degree of lipomatosis of
the muscles.

"All of these forms have probably nothing to do with spinal progressive muscular
atrophy; they differ from it in localization and course, anatomical changes and clinical
phenomena in the muscles, and alterations in the spinal cord."

Still another myopathic condition, which in the light of modern

knowledge has to be separated from progressive muscular atrophy,
is Duchenne's hereditary infantile atrophy. This is characterized by
onset at an early age and by its beginning in the facial muscles. Its
clinical features are thus described by Charcot29 and his pupils Marie
and Guénon.30 Although it mostly begins in infancy, it may not come
on until adolescence, or even until middle or advanced age; it is
often hereditary; the face-muscles are first involved, particularly the
orbicularis oris, and there is a peculiar expression of the
countenance; whistling is impossible, and the articulation of labials
difficult; the eyes cannot be completely closed or the eyebrows
raised. Subsequently other muscles become involved, particularly
those of the shoulder girdle, except the deltoid, the muscles of the
arm, the long supinators of the forearm, and in the lower extremities
the muscles of the buttocks, thighs, and of the anterior external
aspect of the leg. The muscles of the hands and fingers are spared.
Fibrillar tremors are not present, and there is no reaction of
degeneration. The distribution of the atrophy is almost identical with
that of Erb's form, except that it begins in the face. It is likewise an
hereditary or family disease.
29 Le Progrès médical, No. 10, 1885.

30 Revue de Médecine, October, 1885.

PROGNOSIS.—The course of progressive muscular atrophy is never

rapid—essentially chronic. Recovery in a well-established case is not
to be expected, although it is rare for any one to die of the direct
effects of the disease. It is often arrested in its course, and remains
at a standstill for years. The wider its distribution and the more
numerous the foci of involvement, the more rapid is its course; and
when the muscles of deglutition and respiration are involved, and
the carrying back of food interfered with, death from asphyxia is
liable to be produced by the entrance of food into the larynx or from
the accumulation of mucus in what under ordinary circumstances
would be a slight catarrh of the respiratory passages.

TREATMENT.—Treatment directed specifically to the cure of the disease

is limited. Only where there is reason to believe that syphilis is
responsible for it do we find an opportunity to strike at the fons et
origo mali by mercurials and iodide of potassium. Yet in Cooke's
case, quoted by Roberts,31 the disease after progressing
continuously for five years, during which a variety of modes of
treatment was tried, had its further progress stopped by a course of
mercury, although no cause of the disease could be assigned.
31 Op. cit., p. 1; also Cooke On Palsy, Lond., 1822, p. 31; also quoted by Graves in his
Clinical Lectures, L. lxxxiii.

In the majority of instances treatment must consist merely in efforts

to maintain the general health and strength of the patient and to
counteract the obstinate tendency of the spinal disease to produce
wasting of the muscles by depressing their nutrition. The former is
accomplished by an abundance of nutritious food, fresh air, and out-
door life, by gymnastics, chalybeate and other tonics, including
arsenic, strychnine, and quinine. The second is attained by
electricity, frictions, and massage. Both forms of electricity are
useful, the induced current with rapid interruption with a view to
counter-irritate and to stimulate the circulation, or by slow
interruptions to stimulate individual muscles to contraction, and thus
maintain their nutrition. Duchenne recommended the application of
currents of moderate intensity, with not too frequent interruptions,
and for a few minutes only at a time, so as not to fatigue the fibres
undestroyed. He urged particularly the treatment of important
muscles like the diaphragm through the phrenic nerves, of the
intercostals, and of the deltoids before they were actually invaded by
the disease. He relates the case of a man named Bonnard who had
lost many of his trunk-muscles, and who was beginning to suffer
with dyspnoea, on whom faradization of the phrenic nerves,
repeated three or four times a week, was of great service, enabling
him to walk considerable distances and to go up stairs without
fatigue. Another patient, whose arms were much wasted, was so far
restored that at the end of six months he was again able to support
his family.

The direct current—galvanism—is useful in advanced stages of the

disease, where even the strongest faradic currents fail to produce
response. Even where galvanic currents fail to exert contractions the
treatment ought to be persevered in for a long time. It may be
necessary to use very strong currents at the outset, which may be
gradually weakened as contractility returns.

Remak, who especially advocated the use of the continuous current,

advised to place the positive pole in front of one mastoid process
and the negative pole on the opposite side of the neck near the
spinous processes of the vertebræ, not higher than the fifth cervical,
by which he produced the contractions already described as diplegic
in the fingers and other paralyzed parts.

Galvanization of the sympathetic has been apparently useful in the

hands of some—viz. Roberts, Benedikt, M. Meyer, Guthzeit, Erb,
Neseman, and others, while the latter reports a case of complete
cure by this treatment. Eulenburg tells us, however, that a relapse is
said to have occurred in this case; also that neither he nor Rosenthal
have had any results from it.

Massage is equally important, and should be used at the same time

with electricity, but at a different time of day. Eulenburg refers to a
case which was said to have brought the disease to a standstill.
There can be no doubt of the value of the measure as an adjuvant
to treatment.

In families in which an hereditary tendency exists prophylactic

treatment should be used. It should include hygienic measures of
the kind already referred to, and the avoidance of undue fatigue and
exposure; and in the selection of an occupation these matters should
be kept in view.

On the supposition that the disease is a purely local one,

gymnastics, involving the exercise of the groups of muscles prone to
attack, would be indicated, but assume less importance from our
standpoint that it is a spinal disease. At the same time, the patient
should have the benefit of any existing uncertainty in the pathogeny
of the affection; and as gymnastics are eminently calculated to
improve the general health, and thus indirectly to avert the disease,
their use is indicated on these grounds.

PSEUDO-HYPERTROPHIC PARALYSIS.

BY MARY PUTNAM JACOBI, M.D.

SYNONYMS.—Hypertrophic paraplegia of infancy (Duchenne); Myo-

sclerosic paralysis (Duchenne); Progressive muscular sclerosis
(Jaccoud); Atrophia musculorum lipomatosa (Seidel); Lipomatous
myo-atrophy (Gowers); Muscular hypertrophy (Kaulich, Griesinger);
Lipomatosis musculorum luxurians progressiva (Heller);
Myopachynsis lipomatosa (Uhde); Pseudo-hypertrophic paralysis
(Ross); Pseudo-hypertrophy of muscles (Friedreich).

DEFINITION.—Pseudo-hypertrophic paralysis is a rare and

predominantly infantile disease, characterized by a considerable
increase in the volume of some or all the muscles of the lower
extremities, associated with progressive diminution in their functional
energy, and accompanied or followed by paresis and atrophy of the
muscles of the trunk and upper limbs. Many of the hypertrophied
muscles subsequently atrophy; many of the muscles in which
atrophy is the most conspicuous lesion pass through a preliminary
period of hypertrophy. The proximate cause of these alterations is a
profound disturbance in the nutrition of the muscles, attended by
great increase of their connective tissue, by wasting of the
contractile substance, and by the ultimate replacement of this by fat.
HISTORY.—The honors of the discovery of this remarkable disease
may be divided between Duchenne, Meryon, and Griesinger. In
18521 the English physician published a series of six cases, four
belonging to one family, two to another; but these were described
by him under the name of progressive muscular atrophy; and it was
left to Duchenne, who in 18612 published as a new disease the first
case observed by himself, to demonstrate the identity of Meryon's
cases with his own.3 In 1868, Duchenne had collected twelve
additional cases, and published an extensive monograph on the
subject.4 But in 1865, Griesinger5 had excised a portion of muscle
from a patient suffering with the disease, and made the first
histological examination of its structure. On this account several
German writers habitually refer to Griesinger as the earliest authority
on the subject. Before Meryon, Partridge in 1847,6 and Sir Charles
Bell in 1830,7 had described cases of pseudo-hypertrophic paralysis,
but without recognizing their separate morbid entity. Bell's case is
the following: "A boy at eight years of age began to experience
difficulty in rising from a chair. The disease gradually progressed, till
at eighteen he had to twist and jerk his body about to get upright.
The muscles of the lower extremities, hips, and abdomen were
debilitated and wasted. The extensor quadriceps femoris on both
sides wasted, but the vasti externi had not suffered as much; a firm
body, remarkably prominent, just above the knee-joint, marked the
position of the vastus externus. No defect of sensibility or affection
of the sphincters. The upper part of the body, shoulders, and arms
were strong."8
1 Lond. Med. Gaz.

2 De l'Électrisation localisée.

3 Duchenne at first doubted this identity.

4 Archives générales, 1868.

5 Archiv der Heilkunde.

 6 Lond. Med. Gaz., 1847.

7 Nervous System, 2d ed., 1830, p. 163.

8 Loc. cit. This case is quoted in an appendix to Gowers's monograph.

Autopsies.—The first was made by Meryon: the first which included

microscopic examination of the spinal cord was by Cohnheim on a
patient of Eulenburg's.9 Since then autopsies have been made in 12
genuine cases, and in 2 others frequently, though erroneously,
ranked with them.10
9 Vhdlg. der Bul. Med. Ges., 1866, Heft 2, p. 191, quoted by Eulenburg in Ziemssen's
Handbuch, Bd. xii. 2.

10 Cases of Barth and Müller.

Of cases without autopsies a collection of 80 was made by Friedreich

in the monograph on pseudo-hypertrophy which accompanies his
longer monograph on progressive muscular atrophy.11 Mobius has
increased this list to 94;12 Gowers describes 24 cases,13 and refers in
an appendix to 20 more—18 observed by Adams, 2 by Clifford
Albutt.14 Hammond in the sixth edition of his treatise on nervous
diseases, quotes 17 American cases, of which 6 were observed by
himself.15 Gowers estimated that in 1879 about 220 cases had been
reported, divided up among a much smaller number of families.
11 Ueber Progressiv Muskel Atrophie.

12 "Ueber Hereditare Nerven Krankheiten," Volkmann's Samml., 171.

13 Clinical Lecture on Pseudo-hypertrophic Paralysis, Lond., 1879.

14 Among Mobius's cases is that related by Pick in the Deutsches Archiv f. klin. Med.,
Bd. vi., and really a case of progressive muscular atrophy in an adult complicated by
lipomatosis in the calf-muscles. Of the other cases, 6 are quoted from the Swedish, 6
are hitherto unpublished, and have been collected by the author from several clinics.
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