Guide for GP's

Variants of Guillain-Barré Syndrome

Miller Fisher Syndrome is a variant of Guillain-Barré Syndrome, characterised by opthalmoplegia, ataxia and araflexia without significant weakness, or sensory symptoms or signs. Like Guillain-Barré Syndrome it usually follows a respiratory tract infection. The initial symptoms are ataxia of gait, double vision and headache. The ataxia is out of proportion to weakness or sensory disturbance. Treatment is the same as for Guillain-Barré Syndrome.

Acute sensory loss with absent reflexes is another rare manifestation of acute polyneuritis in which the sensory features are more prominent than the associated weakness.

Acute autonomic neuropathy (acute pandysautonomia is an acute auto-immune disorder affecting the autonomic nervous system and manifested by postural hypotension, impairment of sweating, lacrimation and bladder and bowel function.

Chronic inflammatory demyelinating polyneuropathy (CIDP) has a similar pathogenesis to Guillain-Barré Syndrome, but has a slower onset, usually developing over weeks or months. It may run a relapsing and remitting or progressive course over many years. Nerve conduction studies are abnormal. The condition usually responds to treatment with corticosteroids, immunosuppressive agents and plasmapheresis or intravenous immunoglobulin.

Summary

Guillain-Barré Syndrome affects 10 to 20 million people each year. [Australian figures 1 in 80,000-100,000]. The GP must be responsible for recognising the disease in its early stages. Respiratory failure may develop rapidly and early hospitalisation is essential to minimise the risk of death and other complications. The diagnosis is essentially a clinical one depending on the history. A high index of suspicion of the disease is necessary to prevent tragedy, and the onus is on the GP to be alert to the possibility of Guillain-Barré Syndrome.