title: Childhood chronic inflammatory demyelinating polyneuropathy with nonuniform pathologic features.
abstract: Nonuniform pathologic changes in chronic inflammatory demyelinating polyneuropathy were previously reported only in adult humans. We analyzed the pathologic features of 12 children, aged 2-17 years, with chronic inflammatory demyelinating polyneuropathy. Six patients manifested a preceding illness. Five patients presented a chronic, monophasic course, and seven presented a relapsing-remitting course. Three patients exhibited multiple cranial-nerve involvement. Five of 12 (41.7%) patients presented nonuniform features. Two subtypes of nonuniform lesions were revealed. One exhibited varying myelinated fiber content between nerve fascicles, and one exhibited onion bulbs involving a variable number of fascicles. Macrophages were evident in 11 patients, and the number of CD3-positive T cells in the nonuniform group was greater compared with the uniform group (P = 0.045). Our results demonstrate that childhood chronic inflammatory demyelinating polyneuropathy exhibits pathologically nonuniform features, thus providing more evidence to assist in differential diagnoses of pediatric patients. However, clinical and electrophysiologic features, as well as responses to treatment, were similar in the nonuniform and uniform groups.
authors.affiliation: Laboratory of Neuropathology, Department of Neurology, Peking University First Hospital, Beijing 100034, China.
authors.forename: Xinghua
authors.initials: X
authors.lastname: Luan
authors.forename: Riliang
authors.initials: R
authors.lastname: Zheng
authors.forename: Bin
authors.initials: B
authors.lastname: Chen
authors.forename: Yun
authors.initials: Y
authors.lastname: Yuan
mesh_terms: D000293:Adolescent; D015703:Antigens, CD / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D004558:Electric Stimulation / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008264:Macrophages / Q000378:metabolism / Q000473:pathology; D008297:Male; D009431:Neural Conduction / Q000502:physiology; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000473:pathology* / Q000503:physiopathology*; D012189:Retrospective Studies; D013497:Sural Nerve / Q000473:pathology / Q000503:physiopathology
chemical_list: D015703:Antigens, CD