PERIPHERAL NEUROPATHY

Prof.Dr/ Ahmed Gamal Azab

Definition

 Itis inflammation or degeneration of the

peripheral or cranial nerves resulting in
impairment of conduction along these nerves
leading to motor , sensory and/or autonomic
manifestations.

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Classification

1- Mononeuropathy : affection of a single

nerve trunk in one limb.
2- Mononeuropathy multiplex : affection of
more than one nerve trunk in the same limb.
3- Polyneuropathy : affection of more than
one nerve trunk in more than one limb.

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Causes of mononeuropathy &
mononeuropathy multiplex

1- Traumatic : - Compression by a bony fragment or

callous formation.
- Dislocation.
- Wrong injection into a nerve .
- Crossed leg palsy & Saturday night paralysis.
2- Infective : - Leprosy – Herpes zoster.
3- Vascular : Polyarteritis nodosa .
4- Metabolic : Diabetes mellitus.
Causes of Polyneuropathy

A- Heridofamilial :
- Peroneal muscle atrophy .
- Hypertrophic interstitial polyneuropathy.
- Refsum disease.
B- Acquired :
1- Infective : - Viral : mumps , measles.
- Bacterial : Typhus , Typhoid , Tetanus.
- Mycobacterial : Leprosy.
Acquired Polyneuropathy ( cont.. )

2- Toxic : - Inorganic : lead, copper, arsenic, antimony, gold…. ( all

heavy metals ).
Organic : alcohol, insecticides.
3- Metabolic & Endocrinal :
Diabetes mellitus, uremia, amyloidosis, acromegaly,
myxoedema.
4- Nutritional : Pellagra, Beri-beri, subacute combined
degeneration.
5- Iatrogenic : INH, sulphonamides, phenytoin, vincristine.
6- Autoimmune : Guillain Barre syndrome , collagen vascular
disorders.
7- Paraneoplastic : bronchogenic carcinoma , lymphoma.
Pathology

1- Axonal neuropathy : in which the nerve cell

body & axon are primarily affected.
- It shows mild reduction in the nerve
conduction velocity but regeneration is slow.
- It occurs in traumatic, ischemic, nutritional
and toxic causes.
Pathology ( cont.. )

2- Demyelinating neuropathy :
- In which Schwann cell is affected w/out
involvement of the axon.
- It shows marked reduction of the conduction
velocity, but with rapid regeneration.
- occurs in infective & metabolic causes.
Clinical Picture of Polyneuropathy

 Regardless of the cause, It is one of various

combinations of motor, sensory and/or
autonomic manifestations .
 The manifestations are usually bilateral,
symmetrical, distal more than proximal,
involving the lower limbs earlier than the
upper limbs.
Clinical Picture ( cont…)

A- Sensory manifestations :
1- Subjective : pain, parasthesia ( numbness,
tingling ) at the limbs periphery.
2- Objective : glove & stocking hyposthesia +
distal deep sensory loss > sensory ataxia.
Clinical Picture ( cont…)

B- Motor manifestations :LMNL ( wasting,

weakness, hypotonia, hyporeflexia ).
C- autonomic manifestations :
- Vasomotor : coldness , cyanosis .
- Trophic : loss of hair, brittle nails , trophic
ulcers (in severe cases).
Investigations

1- EMG & NCS : reduced nerve conduction

velocity.
2- Nerve biopsy : usually the sural nerve.
3- To detect the cause: Bl.sugar, tests for
collagen vascular disorders, s.creatinine.
Diabetic Neuropathy
1- Diabetic sensorimotor polyneuropathy :
-Common in insulin-dependent diabetics.
-Sensory > motor .
-Burning pain, stock, glove hyposthesia, impaired deep sensation.
-Lost ankle reflex.
-Autonomic neuropathy may occur.
-In long standing cases > distal wasting, weakness may occur.
Pathogenesis (still controversial) :
-in elderly patients it may be an ischemic neuropathy.
-in young patients > it may be due to a metabolic factor (hyperglycemia >
sorbitol accumulation > decreased myoinositol uptake by nerve fibres
> impaired impulse conduction) .

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Diabetic neuropathy( cont…)

2- Diabetic autonomic neuropathy:

-Sweating, diarrhea, postural hypotension,
impotence .
3- Diabetic proximal neuropathy :
-Common in non-insulin dependent .
-Start by pain front of the thigh followed by
proximal leg weakness, lost knee reflex.
Diabetic neuropathy( cont..)
4- Diabetic mononeuropathy:
- Affects peripheral or cranial nerves.
- Nerves vulnerable to compression are the commonest ,such as
: Median, ulnar , radial, common peroneal and lateral
cutaneous nerve of the thigh.
- Ocular nerves are commonly affected(improve in 3-6 months).
5- Diabetic truncal neuropathy :
-Recurrent attacks of truncal pain w/sensory deficit in the
distribution of a single thoracic root.
-Spontaneous recovery occurs in few months.

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Treatment of diabetic neuropathy

1- Proper control of diabetes.

2- Pancreatic transplantation : minor recovery
occurs 3.5 years post transplant.
3- Carbamazepine or Gabapentin for burning
pain & Amitriptylline for aching pain.
4- Vitamins B1,B6,B12 ( help regeneration ).
5- Vasodilators.
6- Physiotherapy for motor weakness.
Peroneal Muscle Atrophy ( HMSN )
= ( Charcot-Marie-Tooth
disease )

Type l : the commonest type, autosomal dominant,

occurs in the first decade .
- Starts by distal leg weakness & wasting w/ inverted
champagne-bottle appearance ( as it involves the
muscles transversely, does not extend above the
junction between lower and middle third of the thigh).
- Later on, there is hand weakness.
- Stocking, glove hyposthesia, pes cavus,
- There may be associated tremors.
HMSN ( cont…)

Type ll : less common, autosomal dominant , onset in

the second decade.
- Pes cavus, hand weakness and sensory loss are
less frequent.
Type lll: uncommon, autosomal recessive, starts in
infancy > delayed walking.skeletal deformities.
Treatment :
- Physiotherapy.
- Ankle splints for foot drop.
Diphtheritic Neuropathy.

Clinical picture :
1-Starts by true bulbar palsy (within few days) followed by >
2- Paralysis of eye accomodation ( ciliary muscle paralysis ) >
blurred near vision.
3-Generalized sensorimotor polyneuropathy.
4-Diaphragmatic paralysis.
Prognosis :
- Good if the child survives.
- Bulbar & accomodation weakness improve within 6 weeks.
- Polyneuropathy recovery takes months.
Diphtheritic neuropathy ( cont…)

Treatment :
- Penicillin G : 600,000 u/12 hours for 14 days.
- Diphtheritic antitoxin : should be given early
as 100,000 u IM.
- Assisted ventillation if need .
Leprotic Neuropathy
- Organism : Mycobacterium leprae .
- Long Incubation period ( about 3.5 years ).
- Insidious onset.
Types :
1- Lepromatous : nodules over the face > leonine facies.
2- Tuberculoid : maculo-anaesthetic skin patches, trophic ulcers.
- It may be a mono or polyneuropathic affection.
- Commonly affacts the facial, sensory 5th, great auricular, ulnar and common
peroneal nerves.
- Thickened affected nerves.
- Sensory > motor affection.
Treatment :
- Dapsone 100mg/day + Rifampicin 600 mg/day ( for 1-2 years ).
- Nerve grafting : for severe mononeuropathy with trophic ulcers.

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Guillain-Barre syndrome ( acute post-
infective polyradiculoneuropathy )

Aetiology (theories) :
1- Post infection : 50% of cases have
preceding respiratory or GIT viral infection.
2- Post vaccination : following vaccination
against Swine influenza virus.
3- Lymphoma (Hodgkin disease).
4- Autoimmune theory.
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Guillain-Barre ( cont…)

Clinical picture :
- Initial febrile illness.
- Usually starts in LLs, then ascends to ULs.
- Proximal > distal, bilateral symmetrical affection.
- Bilateral LMNL 7th paralysis.
- Bulbar weakness, followed by respiratory muscle
paralysis.
- Glove , stocking hyposthesia
- Papilloedema (due to increased CSF proteins >
diminished absorption.).
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Guillain-Barre ( cont…)

Prognosis :
- Recovery in 3-6 months.
- May be with mild residue in 40% of cases.
- Survival for 8 weeks > good prognosis.
Investigations :
- CSF proteins > 2 g/L with normal cell count.
Guillain-Barre (cont…)

Treatment:
1- Nasogastric tube feeding for bulbar palsy.
2- S.C heparin to guard against DVT.
3- Assisted ventillation.
4- Plasmapheresis ( must be done in the first 2
weeks).
5- IVIG : 0.4 g/Kg /day for 5 succesive days.
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