Document Detail


Childhood chronic inflammatory demyelinating polyneuropathy with nonuniform pathologic features.
MedLine Citation:
PMID:  20610120     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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:
Xinghua Luan; Riliang Zheng; Bin Chen; Yun Yuan
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Pediatric neurology     Volume:  43     ISSN:  1873-5150     ISO Abbreviation:  Pediatr. Neurol.     Publication Date:  2010 Aug 
Date Detail:
Created Date:  2010-07-08     Completed Date:  2011-01-14     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8508183     Medline TA:  Pediatr Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  103-9     Citation Subset:  IM    
Copyright Information:
Copyright 2010 Elsevier Inc. All rights reserved.
Affiliation:
Laboratory of Neuropathology, Department of Neurology, Peking University First Hospital, Beijing 100034, China.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Antigens, CD / metabolism
Child
Child, Preschool
Electric Stimulation / methods
Female
Follow-Up Studies
Humans
Macrophages / metabolism,  pathology
Male
Neural Conduction / physiology
Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / pathology*,  physiopathology*
Retrospective Studies
Sural Nerve / pathology,  physiopathology
Chemical
Reg. No./Substance:
0/Antigens, CD

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