Forensic Medicine Div., National Institute of Scientific Investigation, Korea. tjkwon@nisi.go.kr

Cortical dysplasia is used to designate any cerebral developmental malformation, and encompass a spectrum of pathologic changes from mild cortical disruption to more severe pattern of cortical dyslamination with large bizarre neurons, balloon cells, and astrocytosis. The gross appearance of the brian reveals focal or diffuse cortical thickening with no clear cortical-white matter junction due to excessive spillover of abnormal neurons. Cerebellar cortical dysplasias are uncommon but has been reported in cases with more widespread cerebral malformations. We present two autopsy cases of cortical dysplasia, one of which involves both cerebrum and cerebellum. Case 1 was a 4-year-old boy and presented with severe intractable epilepsy of neonatal onset , cerebral palsy, microcephaly, and severe neurologic deficits. Both cerebrum and cerebellum were markedly reduced in size. Microscopic examination of cerebrum showed disorganized cortical architecture with abnormal neurons in the cortex and white matter. The cerebellum disclosed massive spillover of Purkinje cells into white matter. Case 2 was a 51-year-old woman and found in dead in her house. No previous clinical history was informed. At autopsy, no gross abnormality of the brain was observed, but microscopic examination showed giant, dysmorphic neurons throughout the cerebral cortex.

Key word : Cortical dysplasia, Cerebrum, Cerebellum, Neuronal cytomegaly