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INSTRUCTION

Examine this patient's lower limbs, in which weakness began distally.

SALIENT FEATURES

History

· Weakness: difficulty in rising up from sitting position or climbing stairs; legs usually affected before upper limbs.· Dyspnoea late in the course, suggesting diaphragmatic and intercostal muscle weakness.· Cranial nerve involvement: diplopia, drooling of saliva, regurgitation of food.· Paraesthesias.· Urinary symptoms.· Systemic symptoms: fatigue.· Ascertain whether the (inset was preceded by a trivial viral illness.

Examination

· Weakness of distal limb muscles.· Distal numbness.· Arefiexia.

Proceed as follows:

Tell the examiner that you would like to:· Assess respiratory function (forced vital capacity).· Check blood pressure (for labile blood pressure).

DIAGNOSIS

This patient has Guillain-Barrd syndrome (lesion) and is currently experiencing weakness of the distal limb muscles (functionalstatus).

QUESTIONS

What is the pathology?

It is a demyelinating neuropathy.

ADVANCED-LEVEL QUESTIONS

How is the diagnosis confirmed?

· Nerve conduction studies demonstrate slowing of conduction or conduction block.· CSF shows albumino-cytological dissociation, i.e. cell count is normal but the protein concentration is frequently raised.

What is Miller-Fisher syndrome?

A rare proximal variant of Guillain-Barre syndrome which initially affects the ocular muscles and in which ataxia is prominent.

What is the differential diagnosis?

Poliomyelitis, botulis ,primary muscle disease or other neuropathy (porphyric, diphtheric, heavy metal or organophosphoruspoisoning).

How would you treat such patients?

· High-dose intravenous y-globulin during the acute phase to reduce the severity and duration of symptoms (N Eng! J Med 1992;326:1123-9). This is equivalent to plasma exchange in reducing disability, but the combination of intravenous immunoglobulinand plasma exchange offers no significant additional advantage (Dmcet 1997; 349: 225-30).· Ventilatory support if respiratory muscles are affected.· Physiotherapy and occupational therapy for muscle weakness..