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Benign myoclonic epilepsy in infancy: natural history and behavioral and cognitive outcome

J. Domínguez-Carral, J.J. García-Peñas, M.A. Pérez-Jiménez, M.C. Fournier-Del Castillo, I. Carreras-Sáez, S. Jiménez-Echevarría [REV NEUROL 2014;58:97-102] PMID: 24469935 DOI: https://doi.org/10.33588/rn.5803.2013477

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Volumen 58 | Number 03 | Nº of views of the article 22.887 | Nº of PDF downloads 991 | Article publication date 01/02/2014

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ABSTRACT

INTRODUCTION 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.

PATIENTS AND 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. KeywordsBehavioral disorder Benign myoclonic epilepsy in infancy Learning disorder Natural history Neuropsychological outcome Risk factors CategoriesEpilepsias y síndromes epilépticos Neurología del Lenguaje y la Comunicación Neuropsicología

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Original

Benign myoclonic epilepsy in infancy: natural history and behavioral and cognitive outcome