Document Detail

Incontinentia pigmenti: association with anterior horn cell degeneration.
MedLine Citation:
PMID:  3822137     Owner:  NLM     Status:  MEDLINE    
Incontinentia pigmenti (IP) has been associated with CNS involvement, including seizures, retardation, motor abnormalities, and malformations in greater than 30% of patients. Motor symptoms include spasticity and hyperreflexia; however, weakness and flaccidity have also been described. Peripheral nervous system neuropathology in patients with IP has not previously been reported. The infant with IP in this report showed generalized weakness due to anterior horn cell degeneration. The neuropathologic findings in both the central and peripheral nervous systems will be reviewed and contrasted to previous reports. Patients with IP and weakness should undergo neurodiagnostic evaluation of peripheral nervous system function.
R Larsen; S Ashwal; N Peckham
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Neurology     Volume:  37     ISSN:  0028-3878     ISO Abbreviation:  Neurology     Publication Date:  1987 Mar 
Date Detail:
Created Date:  1987-04-08     Completed Date:  1987-04-08     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0401060     Medline TA:  Neurology     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  446-50     Citation Subset:  AIM; IM    
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MeSH Terms
Anterior Horn Cells / pathology*
Incontinentia Pigmenti / pathology*
Motor Neurons / pathology*
Muscles / pathology
Pigmentation Disorders / pathology*
Skin / pathology

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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