Natural history and treatment effects in Guillain-Barré syndrome: a multicentre study

Abstract

A retrospective multicentre study was performed to investigate the natural history and treatment effects in childhood Guillain-Barré syndrome in a large number of patients. Structured questionnaires were sent to 155 paediatric hospitals for details of patients who conformed to internationally accepted diagnostic criteria and who were treated from spring 1989 to summer 1994. Sixty nine hospitals reported data of 175 patients aged 11 months to 17.7 years. At the height of the disease 26% of the patients remained able to walk, but 16% had to be artificially ventilated. The median time from onset of symptoms to first recovery was 17 days, to walk unaided 37 days, and to be free of symptoms 66 days. There was a large group with a benign and a smaller one with a more protracted course. At long term follow up, 98/106 patients were free of symptoms and the remainder were able to walk unaided. Maximum disability grade was the most powerful prognostic factor. In children unable to walk but not yet tetraplegic, immunoglobulins were able to accelerate recovery. Corticosteroids were less potent. Plasmapheresis could not be evaluated because it was administered only in the most severe cases. The natural history of Guillain-Barré syndrome in children is extremely variable and more benign than in adults. Treatment with immunoglobulins should be considered in patients unable to walk. Corticosteroids are not as effective and should be withheld except when, in protracted courses, suspicion of chronic inflammatory demyelinating polyneuropathy arises.