Hemimegalencephaly in a Patient With a Neurocutaneous Syndrome

Department

neurology

Document Type

Article

Abstract

A 5-year-old girl with a history of hypomelanosis of Ito and intractable epilepsy was evaluated for possible resective surgery. Magnetic resonance imaging showed an enlarged right hemisphere, whereas electrographic seizures were arising from the right hemisphere or had a generalized onset. The patient was believed to be a good candidate for hemispherectomy, but the family was hesitant and started a modified Atkins diet. Her seizure control has improved, but she continues to have weekly seizures. Hypomelanosis of Ito is a well-known cause of hemimegalencephaly and is often associated with intractable epilepsy and hemiparesis. Hemispherectomy can often be an effective treatment in intractable cases.

Medical Subject Headings

neurology

Publication Date

2008

Publication Title

Seminars in Pediatric Neurology

ISSN

1071-9091

Volume

15

Issue

4

First Page

190

Last Page

193

Digital Object Identifier (DOI)

10.1016/j.spen.2008.10.010

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