The long-term clinical course of 12 children with idiopathic chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is reported from the University of Michigan, Ann Arbor, and Pennsylvania State University/Hershey Medical Center, Hershey, PA. Data on 62 adults with CIDP previously reported from these centers were used for comparison. In children aged 3 to 17 years, boys and girls were equally affected. Illness duration was 1 to 25 years. In contrast to adults with CIDP, children were more likely to relapse but none was progressive; 83% childhood cases relapsed cf to 35% adults. All were treated with prednisone, plasma exchange, or IV immunoglobulin, and recovery from each relapse was excellent. Only 2 with precipitous onset required ventilatory support. Adults showed a more variable outcome. [1]

COMMENT. According to the above study, the long-term prognosis for children with CIDP is excellent, but relapses are to be expected. Those with a slowly progressive onset have a similar outcome to the acute onset cases, and none develops a severe disability. A gradual weaning from therapy is often successful. These findings are in contrast to the experience at Washington University, St Louis, MO, and the Royal Children’s Hospital, Melbourne, Australia, as reviewed in Ped Neur Briefs Aug 1996 (Progress in Pediatric Neurology III, 1997;pp360-362). Nevo, Pestronk et al reported that childhood onset CIDP has in general a poor prognosis, the majority relapsing and having residual weakness, and attempts to withdraw steroids frequently unsuccessful. Furthermore, these authors distinguished two subtypes of CIDP: 1) with acute or subacute onset, a monophasic course, and complete recovery; and 2) a gradual onset, evolution of symptoms from 3 months to years, residual weakness, and no complete recoveries. Cases with an antecedent URI or tonsillitis recovered completely, whereas cases following MMR immunization had severe residual weakness.