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.
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.
Purpose:
Polymicrogyria (PMG) is often associated with symptomatic focal epilepsy and neurologic dysfunction. We investigated the clinical and laboratory features of a group of children with congenital hemiparesis, unilateral polymicrogyria on magnetic resonance imaging (MRI), and a peculiar epileptic syndrome.
Methods:
Twelve patients (seven girls and five boys) with a mean age of 7.8 years (range, 5-13 years) were studied. All patients underwent clinical evaluation, computed tomography (CT) and MRI scanning, and neuropsychological assessment at initial examination. Patients were followed up from 1 to 7 years (mean, 4.5 years).
Results:
Partial motor seizures with secondary generalization with onset between age 1 and 6 years (mean age, 2 years) were recorded in all patients. The course of epilepsy was similar in all patients with development of atypical absences, negative myoclonus, and gait difficulties. EEG recording demonstrated continuous spike-wave or bilateral abnormality throughout. Frequent relapses of the atonic and myoclonic seizures were seen in seven patients. However, during follow-up, seven patients were seizure free, and the others have not developed this particular seizure pattern. A single case underwent cortical resection 23 months ago and has had no seizures since then. Mental retardation was mild in nine and moderate in three patients.
Conclusions:
Children with unilateral polymicrogyria may develop a syndrome of negative myoclonus seizures that appears to be age specific and responsive to antiepileptic drug (AED) treatment. Despite limited follow-up time, a good outcome was observed in most cases.
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