Childhood chronic inflammatory demyelinating polyneuropathy: clinical course and long-term outcome

We reviewed the clinical history, electrophysiologic and pathologic finding s, and response to therapy of 16 children with chronic inflammatory demyeli nating polyneuropathy. The majority presented with lower limb weakness. Sen sory loss was uncommon. The illness was monophasic in seven children, relap sing in six, and three had a slowly progressive course. All patients were t reated with immunosuppressive agents. In 11, the initial treatment was pred nisolone. All had at least a short-term response but five went on to develo p a relapsing course. Intravenous immunoglobulin was the initial treatment in four patients. Three responded rapidly, with treatment being stopped aft er a maximum of 5 months. In resistant chronic inflammatory demyelinating n europathy, in addition to prednisolone and immunoglobulin, plasma exchange, azathioprine, cyclosporine, methotrexate, cyclophosphamide and pulse methy lprednisolone were tried at different times in different patients. On seria l neurophysiologic testing slowing of nerve conduction persisted for long p eriods after clinical recovery. Follow-up was for an average of 10 years. W hen last seen 14 patients were asymptomatic, two having mild residual defic its. Childhood chronic inflammatory demyelinating neuropathy responds to co nventional treatment and generally has a favourable long-term outcome. (C) 2000 Elsevier Science B.V. All rights reserved.