The unilateral megalencephaly is a rare brain malformation characterized by cerebral asymmetry and cortical dysplasia caused by faulty migration of the subependymal neuroblasts.
We experienced a case of unilateral megalencephaly in a two day-old male with the chief complaint of asymmetric head appearance. Large left hemisphere with agyria, pachygyria, dilatation of lateral ventricle, and the thick cortex of the
ipsilateral
hemisphere were showed in brain MRL, Clinical findings in this case were intractable seizure, hemiparesis, and psychomotor retardation.
A review of literatures was also presented briefly.
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