Presentation
Intractable epilepsy.
Patient Data
MRI of the brain demonstrates the right cerebral hemisphere to be markedly enlarged. The grey matter is disorganized and thickened, consistent with migration arrest. The right lateral ventricle demonstrates an abnormal morphology and is also markedly enlarged. There is diffuse T2 hyperintensity throughout the white matter of the right hemisphere. The right cranial vault is also expanded.
Additionally the right cerebellar hemisphere is also enlarged with abnormal cortical formation.
The left hemisphere is essentially normal in appearance.
Case Discussion
This case demonstrates features of total right hemimegalencephaly (the rarest type of hemimegalencephaly) with severe disorganization and migration arrest as described above.
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