[Benign myoclonic epilepsy in infancy: natural history and behavioral and cognitive outcome].
BACKGROUND
Benign myoclonic epilepsy in infancy (BMEI) is a well-defined electro-clinical syndrome, classically associated with a good prognosis. However, in the last years several studies have been published with variable results of neuropsychological outcome in BMEI. AIM. To analyze the natural history and the cognitive and behavioral outcome in BMEI patients.


METHODS
We report a long-term follow-up of 10 patients with BMEI. During the follow-up, all the patients underwent neurocognitive and behavioral evaluations.


RESULTS
Sixty percent of patients became seizure free on valproic acid. The intelligence quotient of the whole cohort was between 74 and 93, with three patients in the range of borderline intelligence and six in the range of medium-to-low intelligence. Nine of the 10 patients met criteria for attention deficit hyperactivity disorder, and two patients associated another learning disorder. All patients showed poor motor and visuospatial coordination signs and three patients had a behavior disorder.


CONCLUSIONS
The term 'benign' in BMEI has to be used with caution in refer to its behavioral and cognitive outcome. Early onset of seizures and a worse epilepsy control may be risk factors of a poor neuropsychological outcome.
Hospital Infantil Universitario Nino Jesus, 28009 Madrid, Espana.
Jana
J
Domínguez-Carral
Juan José
JJ
García-Peñas
M Ángeles
MÁ
Pérez-Jiménez
M Concepción
MC
Fournier-Del Castillo
Inmaculada
I
Carreras-Sáez
Saioa
S
Jiménez-Echevarría
D000927:Anticonvulsants / Q000627:therapeutic use; D001289:Attention Deficit Disorder with Hyperactivity / Q000453:epidemiology / Q000209:etiology; D002653:Child Behavior Disorders / Q000453:epidemiology / Q000209:etiology*; D003072:Cognition Disorders / Q000453:epidemiology / Q000209:etiology*; D018450:Disease Progression; D004831:Epilepsies, Myoclonic / Q000150:complications / Q000188:drug therapy / Q000453:epidemiology* / Q000523:psychology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007360:Intelligence; D007859:Learning Disabilities / Q000453:epidemiology / Q000209:etiology; D008297:Male; D009069:Movement Disorders / Q000453:epidemiology / Q000209:etiology; D011379:Prognosis; D012189:Retrospective Studies; D014635:Valproic Acid / Q000627:therapeutic use
D000927:Anticonvulsants; D014635:Valproic Acid