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Hemimegalencephaly (HME) is a severe and heterogeneous condition1 with a wide spectrum of clinical and anatomic severity,2 characterized by an enlarged and dysplastic hemisphere. MRI shows a simplified gyral pattern, with thickened gray matter, blurring of gray-white matter, and enlarged lateral ventricles.3 Histologic abnormalities include giant neurons and balloon cells3 resembling focal cortical dysplasia. Although focal cortical dysplasia and dysembryoplastic neuroepithelial tumors (DNT) share common histologic characteristics,4 an association of DNT with an enlarged hemisphere has not been reported.
Treatment of medically resistant epilepsy associated with HME is based on functional hemispherectomy or disconnection of the malformed hemisphere (hemispherotomy).5
We present a patient who had a homogeneously enlarged hemisphere with a discrete structural abnormality in the temporal lobe. Intractable complex partial seizures were successfully treated with temporal resection and the resected tissue showed changes consistent with DNT.
Case report.
This 25-year-old right-handed man had normal neurologic and cognitive development, along with bursts of aggressive behavior. From age 3 years, he had multiple per day episodes of unresponsiveness, staring, and pallor lasting several seconds, often followed by generalization. Antiepileptic drugs were ineffective. From age …
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