Document Detail

Benign myoclonic epilepsy in infants: electroclinical features and long-term follow-up of 34 patients.
MedLine Citation:
PMID:  16499765     Owner:  NLM     Status:  MEDLINE    
PURPOSE: Benign myoclonic epilepsy in infants (BMEI) is a rare epileptic syndrome characterized only by generalized myoclonic seizures (MSs) in normal children during the first 2 years. Our aim was to assess the electroclinical features and the follow-up of this syndrome. METHODS: BMEI was confirmed by electroencephalogram (EEG) in four neuropediatric units in France between 1981 and 2002. Clinical and electroencephalographic findings at diagnosis and during the follow-up were collected. The Vineland scale or Wechsler scale or both were used to perform neuropsychological evaluations. RESULTS: We report 34 patients with BMEI characterized by MSs occurring many times a day. The ictal EEG showed a generalized discharge of polyspikes, polyspikes-and-waves, or spikes-and-waves. The interictal EEG was usually normal. A family history of febrile seizures (FSs) or epilepsy was noted in six patients. A history of FSs was noted in 11 patients. Eleven patients had reflex MSs. Monotherapy with valproic acid was effective in 23 of 30 treated patients. The onset of epilepsy was known in all patients. Four patients had seizures after the initial symptoms. Juvenile myoclonic epilepsy developed in two patients, and cryptogenic partial epilepsy in another. Neuropsychological outcome was evaluated in 20 patients (10 with Wechsler scales and 17 with the Vineland scale). Cognitive functions were normal in 17 patients, whereas developmental delay was observed in three others. CONCLUSIONS: BMEI is clinically characterized by myoclonic seizures involving the upper part of the body, occurring many times a day. The ictal EEG showed a generalized discharge of polyspikes, polyspikes-and-waves, or spikes-and-waves. The interictal EEG was usually normal. Reflex MSs were frequently observed, suggesting that two distinctive syndromes are not necessary. BMEI may be followed by juvenile myoclonic epilepsy. Despite a generally favorable neuropsychological outcome, mental retardation can be observed more frequently than in the general population.
Stéphane Auvin; Florence Pandit; Jullita De Bellecize; Nicole Badinand; Hervé Isnard; Jacques Motte; Nathalie Villeneuve; Marie-Dominique Lamblin; Louis Vallée
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Publication Detail:
Type:  Comparative Study; Journal Article    
Journal Detail:
Title:  Epilepsia     Volume:  47     ISSN:  0013-9580     ISO Abbreviation:  Epilepsia     Publication Date:  2006 Feb 
Date Detail:
Created Date:  2006-02-27     Completed Date:  2006-05-02     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  2983306R     Medline TA:  Epilepsia     Country:  United States    
Other Details:
Languages:  eng     Pagination:  387-93     Citation Subset:  IM    
Department of Pediatric Neurology, Lille University Hospital Roger Salengro, 59037 Lille Cedex, France.
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MeSH Terms
Age of Onset
Anticonvulsants / therapeutic use
Cerebral Cortex / physiopathology
Child, Preschool
Developmental Disabilities / diagnosis,  epidemiology
Electroencephalography / statistics & numerical data*
Epilepsies, Myoclonic / diagnosis*,  epidemiology,  physiopathology
Follow-Up Studies
France / epidemiology
Longitudinal Studies
Myoclonic Epilepsy, Juvenile / diagnosis,  epidemiology
Neuropsychological Tests / statistics & numerical data*
Seizures, Febrile / diagnosis
Treatment Outcome
Valproic Acid / therapeutic use
Wechsler Scales
Reg. No./Substance:
0/Anticonvulsants; 99-66-1/Valproic Acid

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

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