CHILDHOOD CHRONIC INFLAMMATORY DEMYELINATING NEUROPATHIES - CLINICAL COURSE AND LONG-TERM FOLLOW-UP

Chronic inflammatory demyelinating neuropathy (CIDP)is a rare disease in childhood. We reviewed the clinical characteristics, response to th erapy, and long-term prognosis in 13 children (1.5 to 16 years of age) diagnosed with CIDP at Washington University Medical Center, St. Loui s, and the Royal Children's Hospital, Melbourne, Australia, between 19 79 and 1994. The most common presenting symptom (in 11/13 [85%]) was l ower extremity weakness associated with difficulty in walking. Precedi ng events within 1 month of onset, mostly intercurrent infections or v accinations, occurred in seven children (54%). The disease was monopha sic in three children (23%). One relapse occurred in four (30%) and mu ltiple relapses in six (46%). All patients had at least short-term res ponse to steroids. Three children (23%) recovered completely during th e first year. Ten children (77%) had residual weakness after an averag e follow-up of 6 years. There seems to be two populations of children with CIDP. One subgroup, with a favorable prognosis, progressed to pea k disability over less than 3 months; these children often have a mono phasic course with complete resolution of symptoms and signs and withd rawal from all medications by 1 year after onset. A second subgroup pr ogressed for 3 months or longer; these children all required substanti al doses of prednisone for prolonged periods and had considerable long -term morbidity with persistent weakness.