A Case of Band Heterotopia

Kim, Sung Koo; Kim, Young Jun; Lee, Young Ah; Choi, Ha Joo; Chung, Woo Kap

Abstract

The "band heterotopia" or "double cortex" is a brain anomaly that is presumed to result from a premature arrest of neuronal migration. Generalized disorders of neuronal migration to the cerebral cortex have been recognized since the end of the 19th century. Recently, development of neuroimaging technique, such as MRI, have permitted easy diagnosis of generalized neuronal migration disorder. This syndrome is prevalent in females. Most patients present with generalized or multifocal epilepsy, some mental retardation, pyramidal signs and in some dysarthria. Full scales I.Q.s ranging from severely low to normal have been reported. EEG investigations usually demonstrate generalized spike-and-wave discharges or multifocal EEG abnormalities. Classic MRI findings demonstrate a band of subcortical gray matter heterotopia underlying the cortical mantle and separated from it by a thin rim of white mater. We report a 3 year old girl who present a single episode of generalized tonic clonic seizure with fever. Her MRI showed characteristic findings of band heterotopia. She has not been showed further seizure attack. She is being follow up at OPD without medication.