Hemimegalencephaly is a unique form of neuronal migration disorders(NMD's) that is presented with dysmorphic enlargement of a single cerebral or focal hemisphere due to hamartomatous overgrowth. The thickened cortical gyri contain bizarre cellular elements, enlarged ventricle, and frequently combined with other neuronal migration disorders such as enlarged ventricle, and frequently combined with other neuronal migration disorders such as macrogyria, polymicrogyria, nodular heterotopia, Most of the patients with Hemimegalencephaly show a mental deterioration, intractable focal seizure, and developmental delay. We experienced and reported a case of Hemimegalencephaly with no cutaneous abnormality in a 13 years old male patient who showed focal motor seizure with march on the left side, dysarthria, fine motor dysfunction and restin myoclonus on the left arm. After treatment with carbamazepine for 10months, the frequency of seizure were decreased about 70percent.
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