Chronic inflammatory demyelinating polyradiculoneuropathy in children: I. Presentation, electrodiagnostic studies, and initial clinical course, with comparison to adults.

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is rare in children. We reviewed features of 15 children with idiopathic CIDP, and compared these to 69 adults with idiopathic CIDP. Children demonstrated many similarities to adults: (1) Antecedent events were uncommon. (2) There was a high frequency of weakness and reflex loss, a relatively high frequency of sensory loss, and a low frequency of pain and cranial neuropathies. (3) Cerebrospinal fluid protein levels were usually elevated. (4) On electrodiagnostic testing, not all nerve segments were abnormal, and not all children satisfied electrodiagnostic criteria for CIDP. Children differed from adults with CIDP in several ways: (1) The onset of symptoms was usually more precipitous. (2) Gait abnormalities were a more frequent presenting symptom. (3) Children always 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.