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Neuralgic Amyotrophy

Title               

Ñ    Neuralgic Amyotrophy

Definition

Neuralgic amyotrophy is a syndrome of:

Ñ    Unknown aetiology

Affects the lower motor neurones of the:

Ñ    Brachial plexus

And or:

Ñ    Individual nerves

Ñ    Nerve branches

Pathogenesis

Causes

Ñ    Not known

Predisposing factors

Infections

Ñ    Viral infections:

Upper respiratory tract infections usually

Ñ    Bacterial infections:

Pneumonia

Typhoid

Ñ    Parasitic infections

Drugs

Ñ    Immunisations

RAG

Ñ    Childbirth

Surgery and Trauma

Medical investigations

Ñ    Lumbar puncture

Ñ    Contrast radiology

Systemic factors

LMS

Ñ    Polyarteritis nodosa, systemic lupus erythematosus, temporal arteritis

HS

Ñ    Lymphoma

IS

Ñ    Ehlers-Danlos syndrome

Genetic

Ñ    Rare hereditary form:

·          Autosomal dominant inheritance

·          Affects younger age group

·          Males and females equally affected

·          Recurrent bilateral attacks

·          Dysmorphic facial features:

Hypertelorism, long nasal bridge, facial asymmetry

Pathological Process

Ñ    Immune mediated inflammatory reaction against nerve fibres resulting in axonopathy with Wallerian degeneration

Ñ    Less commonly proximal conduction block

History

Sex

Ñ     Males > Females

Age

Ñ    Prevalence highest in young to middle aged adults

RS

Ñ    Shortness of breath:

Phrenic nerve involvement

CNS

Ñ    Pain

Ñ    Weakness

Ñ    Numbness:

May occur    

Ñ    Rarely cranial nerve involvement:

9^th, 10^th, 11^th, 12^th

Examination

CNS

Ñ    Wasting

Ñ    Weakness

Ñ    Absent reflexes:

If relevant nerves are involved

Ñ    Sensory loss:

May be detectable

LMS

Ñ    Pain on palpation

Ñ    Passive and active movements of the shoulder and scapula are painful if this area is involved

Investigations

Electrophysiology

Electromyography

Ñ    Denervation in affected muscles

Management

Symptom relief

Drugs

Ñ    Analgesics

Support

Surgery

Ñ    Nerve or tendon grafts or transfers may be required if the patient does not improve within 2 years

Staffing

Ñ    Physiotherapy

Ñ    Occupational therapy

Prognosis

Ñ    80 % recover by 2 years

Ñ    90 % recover within 3 years

Ñ    Recurrence in 5 %            

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