Document Detail

Hippocampal sclerosis in severe myoclonic epilepsy in infancy: a retrospective MRI study.
MedLine Citation:
PMID:  15857436     Owner:  NLM     Status:  MEDLINE    
PURPOSE: Severe myoclonic epilepsy in infancy (SMEI; Dravet's syndrome) is a malignant epilepsy syndrome characterized by early prolonged febrile convulsions (PFCs) with secondary psychomotor delay and a variety of therapy-resistant seizures. Although the initial symptoms are repeated PFCs, the MRI performed at the onset of disease shows no hippocampal structural abnormalities. We aimed to assess clinical and serial MRI data of patients with SMEI with a special attention to the temporomedial structures. To our knowledge, this is the first systematic MRI study in this disease. METHODS: Clinical and MRI data of all SMEI patients treated in our hospitals between 1996 and 2004 were reviewed. RESULTS: Twenty-eight MRIs from 14 children (one to four images/patient) were included. Age at disease onset was between 3 and 9 months; age at initial MRI was 5 months to 13 years. Ten of 14 patients showed hippocampal sclerosis (HS) during the course of the disease (nine unilateral, one bilateral). Six of these 10 had a normal initial MRI. Age at the first verified HS was between 14 months and 13 years. Neither complex partial seizures nor anterior temporal irritative zone was recorded in these children. CONCLUSIONS: After initially normal structures, in most patients with SMEI, HS develops several months or years after the first PFC. These data support the hypothesis that PFC might be responsible for HS, but other factors and individual sensitivity should play a role in this process.
Zsuzsanna Siegler; Peter Barsi; Magdolna Neuwirth; Judit Jerney; Maria Kassay; Jozsef Janszky; Eva Paraicz; Marta Hegyi; Andras Fogarasi
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Publication Detail:
Type:  Case Reports; Comparative Study; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Epilepsia     Volume:  46     ISSN:  0013-9580     ISO Abbreviation:  Epilepsia     Publication Date:  2005 May 
Date Detail:
Created Date:  2005-04-28     Completed Date:  2005-06-01     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  2983306R     Medline TA:  Epilepsia     Country:  United States    
Other Details:
Languages:  eng     Pagination:  704-8     Citation Subset:  IM    
Epilepsy Center, Bethesda Children's Hospital, Budapest, Hungary.
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MeSH Terms
Age of Onset
Child, Preschool
Epilepsies, Myoclonic / diagnosis,  pathology*
Epilepsy, Temporal Lobe / diagnosis
Follow-Up Studies
Functional Laterality
Hippocampus / pathology*
Magnetic Resonance Imaging / statistics & numerical data*
Seizures, Febrile / diagnosis

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