Document Detail


Unusual consequences of status epilepticus in Dravet syndrome.
MedLine Citation:
PMID:  20172746     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Although status epilepticus (SE) affects the course of Dravet syndrome (DS), it rarely alters dramatically psychomotor outcome. We report an unusual pattern in 3 patients who following refractory SE lasting respectively 2, 7 and 12h experienced persistent and severe cognitive and motor deterioration. We compared these patients to published data and to personal experience in Necker hospital, to find links between severe outcome and clinical features such as treatment or duration of refractory SE. The key point was that anoxoischemic-like lesions appeared on MRI although cardiovascular function had remained stable. Therefore, neither hemodynamic failure, nor abnormalities of cardiac rhythm could explain the lesions and neurological worsening. For theoretical reasons the responsibility of therapy common for the 3 patients, e.g., barbiturates was suspected.
Authors:
M Chipaux; N Villeneuve; P Sabouraud; I Desguerre; N Boddaert; C Depienne; C Chiron; O Dulac; R Nabbout
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Publication Detail:
Type:  Journal Article     Date:  2010-02-20
Journal Detail:
Title:  Seizure : the journal of the British Epilepsy Association     Volume:  19     ISSN:  1532-2688     ISO Abbreviation:  Seizure     Publication Date:  2010 Apr 
Date Detail:
Created Date:  2010-03-08     Completed Date:  2010-06-14     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9306979     Medline TA:  Seizure     Country:  England    
Other Details:
Languages:  eng     Pagination:  190-4     Citation Subset:  IM    
Copyright Information:
(c) 2010 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.
Affiliation:
Service de neurop?diatrie, Centre de r?f?rence ?pilepsies rares, AP-HP, H?pital Necker Enfants Malades, Paris, France. mathilde.chipaux@nck.aphp.fr
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MeSH Terms
Descriptor/Qualifier:
Anticonvulsants / adverse effects
Barbiturates / adverse effects
Brain / blood supply,  pathology*
Child, Preschool
Developmental Disabilities / etiology
Epilepsy / drug therapy,  genetics,  pathology*
Humans
Infant
Magnetic Resonance Imaging
Mutation
Nerve Tissue Proteins / genetics
Sodium Channels / genetics
Status Epilepticus / drug therapy,  genetics,  pathology*
Tomography, X-Ray Computed
Chemical
Reg. No./Substance:
0/Anticonvulsants; 0/Barbiturates; 0/Nerve Tissue Proteins; 0/Sodium Channels; 0/sodium channel, voltage-gated, type I, alpha protein

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