A particular type of epilepsy in children with congenital hemiparesis associated with unilateral polymicrogyria

R Caraballo1, R Cersósimo, N Fejerman

  • 1Servicio de Neurologia, Hospital de Niños J. P. Garrahan, Buenos Aires, Argentina.

Epilepsia
|July 14, 1999
PubMed

Insights

Children with unilateral polymicrogyria (a brain malformation) may develop a specific seizure syndrome. This epilepsy appears age-specific and often responds well to antiepileptic drugs, with many achieving seizure freedom.

Area of Science:

  • Neurology
  • Pediatrics
  • Neuroimaging

Background:

  • Polymicrogyria (PMG) is a congenital brain malformation often linked to epilepsy and neurological deficits.
  • Congenital hemiparesis and unilateral PMG on MRI can present with unique epileptic syndromes in children.

Purpose of the Study:

  • To investigate the clinical and laboratory features of children with congenital hemiparesis, unilateral PMG, and a specific epilepsy syndrome.
  • To characterize the presentation, course, and outcomes of this particular group of pediatric patients.

Main Methods:

  • Study included twelve pediatric patients (7 female, 5 male) with a mean age of 7.8 years.
  • Methods involved clinical evaluation, CT/MRI, neuropsychological assessment, and follow-up (1-7 years).

Main Results:

  • All patients experienced partial motor seizures with secondary generalization (onset 1-6 years).
  • Epilepsy progression included atypical absences, negative myoclonus, and gait issues; EEG showed continuous abnormalities.
  • Seven patients became seizure-free during follow-up, and nine had mild to moderate intellectual disability.

Conclusions:

  • Children with unilateral PMG can develop an age-specific syndrome of negative myoclonus seizures.
  • This epilepsy syndrome appears responsive to antiepileptic drug (AED) treatment, with most cases showing a good outcome.
Abstract

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