CHRONIC INFLAMMATORY DEMYELINATING POLYRADICULONEUROPATHY IN CHILDREN.1. PRESENTATION, ELECTRODIAGNOSTIC STUDIES, AND INITIAL CLINICAL COURSE, WITH COMPARISON TO ADULTS

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is ra re in children. We reviewed features of 15 children with idiopathic CI DP, and compared these to 69 adults with idiopathic CIDP. Children dem onstrated many similarities to adults: (1) Antecedent events were unco mmon. (2) There was a high frequency of weakness and reflex loss, a re latively high frequency of sensory loss, and a low frequency of pain a nd cranial neuropathies. (3) Cerebrospinal fluid protein levels were u sually elevated. (4) On electrodiagnostic testing, not all nerve segme nts were abnormal, and not all children satisfied electrodiagnostic cr iteria for CIDP, Children differed from adults with CIDP in several wa ys: (1) The onset of symptoms was usually more precipitous. (2) Gait a bnormalities were a more frequent presenting symptom. (3) Children alw ays presented with significant neurological dysfunction, and not with the minor symptoms initially seen in some adults. The initial response of children with CIDP to immunomodulating therapy was excellent. (C) 1997 John Wiley & Sons, Inc.