Document Detail

A case of atypical benign partial epilepsy with action myoclonus.
MedLine Citation:
PMID:  23280272     Owner:  NLM     Status:  MEDLINE    
We describe a boy, 3 years and 6 months old, who experienced a rolandic seizure accompanied by a cluster of atypical absence seizures, the EEGs for which corresponded to those of atypical benign partial epilepsy (ABPE). Of note, this patient suffered from developmental delay beginning in infancy and exhibited giant middle-latency somatosensory evoked potentials with action myoclonus. With the exceptions of ethosuximide, acetazolamide, and adrenocorticotropic hormone, which have been reported to be effective in ABPE, the atypical absence seizures were intractable despite extensive treatment with various anticonvulsants. The drugs that were effective led to a remarkable reduction in seizure frequency and EEG improvement, but the efficacy was temporary. The patient demonstrated moderate mental retardation without regression and could not walk with support or speak any meaningful words at the age of 3 years and 6 months. Based on thorough differential diagnosis, although further studies will be necessary, we propose that this boy may present a new phenotype of ABPE: ABPE with action myoclonus.
Satoru Kobayashi; Takehiko Inui; Keisuke Wakusawa; Soichiro Tanaka; Tojo Nakayama; Mitsugu Uematsu; Masaru Takayanagi; Toshiyuki Yamamoto; Kazuhiro Haginoya
Publication Detail:
Type:  Case Reports; Journal Article     Date:  2012-12-29
Journal Detail:
Title:  Seizure     Volume:  22     ISSN:  1532-2688     ISO Abbreviation:  Seizure     Publication Date:  2013 Apr 
Date Detail:
Created Date:  2013-03-11     Completed Date:  2013-08-26     Revised Date:  2014-10-21    
Medline Journal Info:
Nlm Unique ID:  9306979     Medline TA:  Seizure     Country:  England    
Other Details:
Languages:  eng     Pagination:  242-5     Citation Subset:  IM    
Copyright Information:
Copyright © 2012 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.
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MeSH Terms
Anticonvulsants / therapeutic use
Child, Preschool
Epilepsies, Partial / complications,  diagnosis*,  drug therapy
Evoked Potentials, Somatosensory
Intellectual Disability / complications,  diagnosis*
Myoclonus / complications,  diagnosis*,  drug therapy
Treatment Outcome
Reg. No./Substance:

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