Paediatrics: Guillain–Barre syndrome

Guillain–Barre syndrome

G-BS is an acute, potentially fatal, demyelinating polyneuropathy. It often follows an intercurrent infection, classically campylobacter enteritis. Initially, there are motor signs that progress up the body. That is, first there is gait disturbance, which then progresses to involvement of the arms, and then respiratory and bulbar involvement in severe cases. Children may com-plain of muscle pain, which can mask the weakness. Sensory involvement also occurs, but this feature may be overlooked.

Aetiology

·  EBV, cytomegalovirus.

·  Measles, mumps.

·  Enteroviruses.

·  Mycoplasma pneumoniae.

·  Borrelia burgdorferi.

·  Campylobacter.

Diagnosis

The differential diagnosis includes myasthenia gravis, polio, spinal cord compression/myelitis, and botulism. The main diagnostic features of G-BS are as follows.

·  Clinical picture: muscle weakness, with loss of reflexes in an ascending fashion.

·  Nerve conduction studies: demonstrate characteristic features.

·  Cerebrospinal fluid: elevated protein, but this does not occur at onset.

·  Variants: Miller–Fisher variant includes bulbar cranial nerve involvement, ophthalmoplegia, ataxia, and areflexia.

·  The bladder should be spared.

Course

·  Onset: starts 1–2wks after an antecedent illness.

·  Ascending weakness: the initial deterioration, normally lasts <2wks.

·  Plateau phase: symptoms are static, normally lasts for 1–2wks.

·  Recovery: should begin within 2–4wks, in a descending manner, though full recovery sometimes takes a number of months. The reflexes are the last to recover.

Management

·  Immediate admission for monitoring: of respiratory status (use forced vital capacity (FVC); and autonomic involvement. Dysautonomia leads to tachycardia, fluctuating BP, and GI disturbance. Pain control. Feeding/swallowing assessment.

·  Early introduction of physiotherapy and occupational therapy to avoid joint contracture.

·  Control of pain.

Immunoglobulin: IV treatment (400mg/kg/day for 5 days) is normally used initially, with plasmapheresis reserved for refractory cases. Note risk of transmissible infection and allergic reactions to IVIG.