ANSWER 86
The most marked feature is the loss of power. The reduced tone and absent reflexes indi-
cate that this is a lower motor neurone lesion. The sensory disturbance is less severe and
he has a sensory level around L2/3. This man has Guillain–Barré syndrome(acute idio-
pathic inflammatory polyneuropathy). This disorder is a polyneuropathy which develops
usually over 2–3 weeks, but sometimes more rapidly. It commonly follows a viral infec-
tion or Campylobactergastroenteritis, and a fever is common. It predominantly causes a
motor neuropathy which can either have a proximal, distal or generalized distribution.
Distal paraesthesiae and sensory loss are common. Reflexes are lost early. Cranial and bulbar
nerve paralysis may occur and can cause respiratory failure. The CSF protein is usually
raised, but the cell count is usually normal, although there may be a mild lymphocytosis.
The disorder is probably due to a cell-mediated delayed hypersensitivity reaction causing
myelin to be stripped off the axons by mononuclear cells.
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- Guillain–Barré syndrome
- Lead poisoning
- Diphtheria
- Charcot–Marie–Tooth disease (hereditary motor and sensory neuropathy)
- Poliomyelitis
Differential diagnoses of motor neuropathyAn acute-onset neuropathy suggests:
- Guillain–Barré syndrome
- porphyria
- malignancy
- some toxic neuropathies
- diphtheria
- botulism.
This patient should be referred to a neurologist for further investigation and management. In
this patient who presents with weakness and sensory signs, it is important to make sure there is
no evidence of spinal cord compression or multiple sclerosis. However, these would tend to
cause hypertonia, hyper-reflexia and a more distinct sensory level. A magnetic resonance imag-
ing (MRI) scan of the brain and spinal cord should therefore be considered. Nerve-conduction
studies will confirm a neuropathy. He should be treated either with plasma exchange or intra-
venous immunoglobulin. His respiratory function should be monitored with daily spirometry,
and mechanical ventilation may be necessary. Most patients recover over a period of several
weeks.
- Guillain–Barré syndrome presents with predominantly a motor neuropathy although
sensory symptoms are usually present. - There is often a history of an infective illness in the previous 3 weeks, often
Campylobacter jejuni.