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A female case of West syndrome with remission of spasms following multiple cerebral hemorrhages.
MedLine Citation:
PMID:  21051162     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Multiregional wide-distribution hemorrhages of the left hemisphere occurred at 1month of age in a girl with congenital factor V deficiency. At the age of 4months, symmetrical spasms appeared in clusters and electroencephalography showed diffuse background attenuation in the left side and hypsarrhythmia only in the right. Brain CT scan showed that the left hemisphere including Rolandic area was completely infarcted. She was diagnosed with West syndrome and spasms were not controlled by anti-epileptic drugs. Following multiple intracerebral and subarachnoid hemorrhaging involving the right hemisphere at approximately 2years of age, spasms and hemi-hypsarrhythmia abruptly disappeared, and complete remission of spasms persisted for 2years. Taken together, the right-hemispheric cortex seemed to be primarily responsible for generation of symmetric spasms and hemi-hypsarrhythmia on electroencephalography. This case study failed to support the hypothesis that ictal discharges needs to be propagated from one to the other side though the corpus callosum in order to generate symmetric spasms. Rather, symmetric spasms can be explained by activation of subcortical structures such as the brain stem, ipsilateral spreading of electrographic discharges from the residual hemisphere, or intra-hemispheric propagation of ictal discharges.
Authors:
Naomi Hino-Fukuyo; Kazuhiro Haginoya; Mitsugu Uematsu; Shigeru Tsuchiya
Publication Detail:
Type:  Journal Article     Date:  2010-11-03
Journal Detail:
Title:  Brain & development     Volume:  33     ISSN:  1872-7131     ISO Abbreviation:  Brain Dev.     Publication Date:  2011 Sep 
Date Detail:
Created Date:  2011-08-03     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7909235     Medline TA:  Brain Dev     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  678-82     Citation Subset:  IM    
Copyright Information:
Copyright © 2010 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
Affiliation:
Department of Pediatrics, Tohoku University School of Medicine, Japan.
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