Article

Myoclonic-astatic epilepsy in a child with Sturge-Weber syndrome.

Department of Neurology, The Johns Hopkins Medical Institutions, Baltimore, Maryland 21287, USA.
Pediatric Neurology (impact factor: 1.52). 03/2007; 36(2):115-7. DOI:10.1016/j.pediatrneurol.2006.08.006
Source: PubMed

ABSTRACT A child with Sturge-Weber syndrome and a left occipital leptomeningeal angioma developed focal seizures at 6 years of age that responded initially to oxcarbazepine. After 7 months of seizure freedom, the patient developed typical myoclonic-astatic seizures associated with generalized electrographic discharges, which worsened as oxcarbazepine was increased. The seizures and electroencephalogram improved dramatically in 3 weeks as the oxcarbazepine was withdrawn and valproic acid was initiated. This case demonstrates the importance of recognizing that children with epilepsy due to focal lesions can develop secondary bilateral synchrony that can be aggravated by medications that are effective for partial seizures. In such cases, treatment with a broad-spectrum antiepileptic may be advantageous.

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Keywords

3 weeks
 
6 years
 
7 months
 
broad-spectrum antiepileptic
 
cases
 
children
 
focal lesions
 
focal seizures
 
generalized electrographic discharges
 
left occipital leptomeningeal angioma
 
medications
 
partial seizures
 
Sturge-Weber syndrome
 
typical myoclonic-astatic seizures