Peter Diószeghy

2018
Mononeuropathies
Classification of lesions of peripheral nerves

Normal Neurotmesis Axonotmesis Neurapraxia

 Neurotmesis
Interruption of the entire nerve, including the supporting
connective tissues (both axons and nerve sheaths are involved).
Clinical features:
Motor signs - peripheral type of paresis

Sensory signs - sensation is affected on the region

supplied by the injured nerve.

Autonomic signs - vasomotor, sudomotor, trophic changes

Incomplete
Neurinoma
regeneration
 Axonotmesis
The axons lose their continuity but the connective tissue
remain more or less intact and the Schwann cell
basement membrane survives.
The Wallerian degeneration of the distal part of the
nerve is complete, but regeneration is always present
and runs its normal course.

Clinical features:
same like in the neurotmesis
 Neurapraxia
Transient loss of conduction without structural
changes, without interruption of the continuity
of any of the nerve components.

Recovery: takes place within days or weeks and is

complete in 6-8 weeks after removal of the cause.

Clinical features:
Motor signs: mainly paresis, no muscular atrophy
Sensory signs: mostly subjective (numbness, tingling,
burning), the loss of sensation is rare, the deep sensory
qualities may be affected.
 The most frequent causes of the injuries of the nerves

1. Trauma
2. Ischemia
occlusion
compression of arteries
3. Pressure
nerve compression

local edema ischemia

deformation
of myelin sheath
 Diagnosis of nerve lesions
1. Anamnesis
2. Physical examination
2. Special diagnostic methods
•Sweet test (Minor test, Ninhydrin test)
•Electrophysiology
ENG: Motor and sensory nerve conduction studies are
important to ascertain the site and nature of the injury.
EMG: localization, severity of the lesion, signs of
denervation and regeneration (time course),
differentiation of nerve-plexus-root injuries
 SENSORY MOTOR
Orthodrom

Antidrom
 Signs of denervations

Fibrillation potentials

Positive sharp waves

 Treatment
1. Conservative therapies
Purpose to preserve the best possible condition
to prevent the swelling of tissues
atrophy of muscles
contractures
Passive movements, re-educative exercises
Electrical stimulation
2. Surgical treatments
ends of transsected nerve should be sutured
Urgent? Postponed (in 2-3 months) operations?
Infected wounds postponed operation
Prevention of scar formation atraumatic surg.techniques
Long gap auto-, allograft
Entrapment syndromes decompression
 Regeneration

• Sprouting of the proximal stump

• The scars may impede the successful regeneration
• The time of complete regeneration is determined
gap between nerve stumps,
integrity of the Schwann cell bundle,
age.
• Speed of regeneration
1 - 1.5 mm/day (motor fibers)
 Upper brachial plexus lesion (Erb-Duchenne)
Cause
- traction on the arm at birth
- falling on the shoulder
- luxation/ cruel reposition of the
shoulder joint
Position of arm
- internal rotation
- elbow is extended
- hand pronated
Paresis
- abduction, external rotation of arm
- flexion of the elbow
Hypaesthesia
- incomplete,
on the outer surface of the arm
Reflexes
- biceps is absent
 Lower brachial plexus lesion (Dejerine-Klumpke)
Cause
- birth injuries
- trauma
- tumor
Motor signs
- paralysis and atrophy of intrinsic hand muscles
- claw position
Sensory disturbances (hypesthesia)
- inner side of the arm/forearm
- ulnar side of the hand
Sympathetic lesion
(branch inf. cervical ganglion)
- Horner syndrome
 Neuralgic amyotrophy (brachial plexus neuritis)
Unilateral or bilateral disorder of the brachial plexus
Onset: acute severe shoulder pain,
it follows infections,
injection of serum vaccine,
strenuous exercise, trauma, pregnancy
Cause: unknown
autoimmun neuropathy is suggested
Clinical characteristics:
rapidly developing proximal weakness, atrophy,
sensory deficit on the region of axillary nerve,
prognosis is usually good.
Electrophysiology: signs of lesions of brachial plexus.
Treatment
symptomatic - painkiller,
intensive physiotherapy,
anti-inflammatory preparations (corticosteroids)
Long thoracic nerve

In men doing heavy

physical work
• Clinical features
weakness:
– elevation of arm above
horizontal plane
– winging of the scapula
 Axillary nerve
Motor innervations:
- teres minor,
- deltoid
Sensory innervations:
- above deltoid muscle

Cause of the lesion

- trauma of the shoulder
(contusion, traction)

Clinical features
- weakness: abduction of the arm
- hypoesthesia: lateral surface of shoulder
 Ulnar nerve
Clinical features
weakness
- flexion, adduction of wrist
- flexion of ring, little fingers
- abduction of little finger
- adduction of thumb
atrophy
- hypothenar and interossei
clawing of the hand
sensory loss
Places of compression
of ulnar nerve
 Radial nerve

• Weekness
extension ('wrist drop'):
wrist, fingers
"park bench paralysis”,
"Saturday night palsy"
 Median nerve
"preacher's hand"
 Carpal tunnel syndrome
Localisation
compression of median nerve by transverse carpal ligament at wrist

Predisposing factors
constitutional features, fractures, arthritis, pregnancy, diabetes mellitus,
hypothyreosis, sudden increase in weight, gout, strenuous exercise

Clinical features
nocturnal brachialgia paresthetica, pain irradiating into 1-3 fingers, pain upon
pressure on the median nerve, typical sensory loss, hand is swollen, joints are
rigid, atrophy and weakness of the thenar muscles

Treatment
inmobiliztion of wrist, local infiltration with cortisol, decompression
Common peroneal nerve

Most frequent localization

Capitulum fibulae

Causes
Fracture of fibula
Compression
- long lasting kneeling
- crossing of the leg
- plaster cast,
- fibrous band
 N. peroneus communis
Clinical features
Weakness dorsiflexion of leg and toes
steppage gait, footdrop
Sensory loss back of the leg
anterior-lateral aspect of sole
Polyneuropathy refers to a disease
characterized by widespread simultaneous
involvement of the peripheral nerves.

The polyneuropathies of many different

etiologies have similar symptoms and signs.
 NEGATIVE POSITIVE
Motor Weakness, Atrophy, Fatigue, Fasciculations, Cramps,
Reduced muscle tone Myokymia
Reflex Reduced or absent (small fibre -------
polyneuropathy - normal)
Sensory
Small fibre Decrease of pain and temperature Spontaneous chronic and
sensation, Loss of visceral pain, paroxysmal pain, Cutaneous
Foot ulceration hyperesthesia,
Paresthesia (jabbing, shooting).
Large fibre Decrease of vibration and Paresthesia (“tingling”, “pins-and-
proprioception, Reduction of needles”),
touch-pressure sensibility,
Sensory ataxia, Postural tremor
Autonomic
Cardiovascular Orthostatic hypotension, Hypertension
Arrhythmia
Gastrointestinal Gastroparesis, Constipation Neuropathic diarrhoea
Urogenital Impotence, Retrograde
ejaculation, Urinary retention
Peripheral Decreased sweating, Hair changes Osteoarthropathy
Skeletal Clawhand, Equinovarus position of foot,
deformity Hammer toe, Pes cavus, Spinal deformity
• Hereditary • Toxic
• Autoimmun Drug induced, Heavy metals,
GBS, CIDP, MMN Industrial and Agricultural
• Infectious chemicals
AIDS, Lyme disease, Leprosy, • Metabolic
Diphtheria, Diabetes, Uremia, Alcoholism,
• Nutritive Liver disease, Hypothyroidism,
B12, Folic acid, Thiamine Thyrotoxicosis, Porphyria,
deficiency, Critically ill conditions
• Connective tissue diseases • Dysproteinemic
SLE, Sjögren’s syndrome, Monoclonal gammopathy,
Vasculitis, Scleroderma, Macroglobulinemia,
Rheumatic arthritis, Amyloidosis, Multiple myeloma,
Hypereosinophilic syndrome • Malignancies
 Motor Sensory Autonomic
MMN with conduction hereditary sensory acute pandysautonomia
block neuropathy
drug (diaphenylsulfone) B12 deficiency paraneoplastic
autonomic neuropathy
HMSN-s B6 intoxication
Guillain-Barré syndr. paraneoplastic amyloidosis
(axonal type -AMAN) Sjögren syndrome diabetes mellitus
CIDP drugs idiopathic small-fiber
(cysplatine, isonicide, neuropathy
critical illness pp. thalidomide, adriamycin,
hydralazine,
porphyria chloromycetine)
lead intoxication diabetes
drugs leprosy
(vincristine, cytarabine,
amiodarone, perhexiline)
Pure, Predominantly
 Pathology
Normal

Pronounced
Early demyelinisation
demyelinisation „onion bulb”

„onion bulb”
Axonal lesion
Normal
 Clinical signs and pathology
The clinical course/prognosis are determined by the underlying
pathology.
Demyelinisation Axonal lesion
• Acute and relapsing course, • Acute and chronic progressive
course
• Rapid and full recovery, • Slow and generally incomplete
recovery,
• Severe paresis, • Severe paresis,
• Lack of significant atrophy, • Prominent atrophy,
• Global areflexia, • Achilles areflexia (patella, upper
extremity reflexes are preserved),
• Motor signs are more pronounced, • Significant sensory simptoms and
signs.
 Protocol of clinical evaluation
1. Neurological examination including detailed history
2. Electrophysiological studies
3. Laboratory examinations
4. Genetic studies
5. Histology

The first two steps are the neurological examination and the
electrophysiological studies.
They help to make a rational investigational program, the
special studies can appropriately be applied only after these
examinations.
 Locus Gene product Type of genetic
lesion
CMT (Charcot-Marie-Tooth) type 1 (demyelinisation)
CMT 1A 17p11-12 PMP22 dupl/pm
CMT 1B 1q22-23 PMPO pm
CMTX Xq13 connexin 32 pm
CMT type 2 (axonal)
CMT 2A 1p36 ismertlen ismertlen
CMT 2B 3q ismertlen ismertlen
HNPP 17p11-12 PMP22 deletio
CMT 3 17p11-12 PMP22 pm
(Dejerine-Sottas)
PMP22 = peripheriás myelin protein 22 kD
PMP0 = peripheriás myelin protein 0 (28 kD)
Cx32 = Connexin 32
HNPP = Hereditary neuropathy with liability to pressure palsies
 Guilllain Barré Syndrome (GBS)

Jean-Alexandre André Strohl

Georges Guillain
Barré

Definition
Acute, autoimmune, polyradiculoneuropathy affecting the
peripheral nervous system, usually triggered by an infectious
process. It exhibits as an ascending paralysis in the legs that
spreads to the upper limbs, face and respiratory muscles.
 Variants of GBS syndrome

GUILLAIN-BARRE
SYNDROME

Regional Functional
Demyelinating Axonal
variants variants

Acute Acute motor

Syndrome
inflammatory axonal
Miller Fisher Ataxic
demyelinating neuropathy
MFS
neuropathy (AIDP) (AMAN)

Sensory Acute Acute

AIDP motor sensory Facial diplegia pandysautonomia
neuropathy
AMSAN
Pharyngo
Cervico
Brachial
 Epidemiology
• Relatively rare disorder:
incidence:1 - 2 / 100.000

Antecedent events
• They usually occur 1 - 3 weeks before the onset of
clinical symptoms.
• The most often reported is a benign upper
respiratory or gastrointestinal infection
(campylobacter jejuni)
 Clinical Symptoms and Signs
Paresthesias
• Herald the onset of the disorder in at least 50% of patients
• Distal, symmetrical
• Spreads proximally but seldom extends beyond the ankles
and wrists
Weakness
• Weakness is first noted a few days after the onset of
paresthesias
• It begins in the lower extremities
• Ascends to the upper extremities
Diminished or absent deep tendon reflexes
• One of the earliest findings
 Diagnostics
CSF analysis:
• Protein concentration: INCREASED
• Cell count: NORMAL
Electrophysiology:
• Nerve conduction studies

Prognosis
• Mortality: ~ 5 %
• Recover without serious neurological sequels ~ 75 %
• Recovery ensues over 6 to 12 months
• Permanent, substantial neurological sequels: ~ 7 - 15 %
Bilateral foot drop
Intrinsic hand muscle weakness and wasting
Sensory ataxia
Burning dysesthesias
 Treatment
Specific therapy
• Plasmaexchange
• IvIg treatment
Supportive care
• Patients with suspected GBS should be hospitalized.
• ICU admissions occurs in ~ 30% of GBS patients.
• Major life-threatening complications:
Respiratory failure
Dysautonomia
Venous thromboembolism
Heterogenious group of polyneuropathies. Common features:
1. Immunmediated neuropathy.
2. Chronic progressive or relapsing course.
Progressive phase: > 4 weeks
Duration: > 2 months
3. Signs:
• Symmetrical
• Motor/Sensory
4. CSF: protein – cell dissociation.
5. Treatment:
• Metilprednisolon
• Immunglobulin
• Plasmaexchange
 Alcoholic polyneuropathy
• Pathology: primary axonal neuropathy
• Cause: impaired gastrointestinal absorption
Thiamine deficiency
Reduced aldehide dehydrogenase activity
• Lower extremities > Upper extremities
• Sensory signs:
distal burning paresthesias,
diminished sensation with "stocking-to-glove" distribution.
• Gait ataxia: difficulty walking, history of frequent falls.
• Motor signs: weakness of distal predominance
• Areflexia
• Therapy: abstinence
vitamin B1
 Diabetic polyneuropathy
Aetiology
chronic hyperglycaemia

Classification
A. Diffuse Neuropathy
1. Distal symmetric sensory-motor pp.
2. Proximal symmetric motor pp. on the lower extremities
3. Autonomic neuropathy
B. Focal neuropathies
1. Cranial neuropathies
2. Radiculopathy/plexopathy
3. Entrapment neuropathies
 Clinical features

Most frequent form: distal symmetrical

• Sensory disturbances are the prevailing symptoms
and signs.
• Glove and/or stocking like distribution of sensory
loss
• Painful symptoms 30%
• Significant motor deficit is not common
Oculomotor
neuropathy
 Drug induced neuropathy

Clinical Gyógyszerek
features Antimicrobic Cytostatic Cardiovasc.
Sensori Ethionamid Procarbazin
neuropathy Chloramphenicol Nitrofurazon
Diamino substances
Paresthesia Colistin Cytarabin Propranolol
Streptomycin
Nalidixic acid
Sensorimotor Isoniazid Vincristin Perhexilin
neuropathy Streptomycin Chlorabucil Hydrallazin
Nitrofurentoin Clofirat
Dominanarly Sulphonamidok
motor Amphotericin
 Drug induced neuropathy

Clinical Drugs
features
Hypnotic Antirheumatic Others
Sensori Phenelsine Sulfoxon
neuropathy Ergotamin
Thiouracil
Chlorpropamid
Paresthesia Thalidomid Gold Methysergid
Glutethimid Indomethacin Phenytoin
Amitriptylin Ghloroquin Disulfiram
Phenylbutazon Tolbutamid
Sensorimotor Imipramin Chlorpropamid
neuropathy Dapson