AN UNUSUAL TYPE OF PRIMARY CEREBRAL HEMIHYPOTROPHY WITH SIGNS OF DYSFUNCTIONAL NEURONAL MIGRATION

We describe the neuropathological features of a complex brain malforma tion characterized by cerebral hemihypotrophy with ipsilateral lissenc ephaly, periventricular nodular heterotopia and macrogyria. The contra lateral hemisphere showed only slight alterations of the gyral pattern and a limited periventricular gray matter heterotopia. The clinical p icture of the patient, who died at the age of 15 years, consisted of s evere oligophrenia, intractable seizures and left hemiparesis. We disc uss the nosological status of this neuronal migration disorder of appa rently unknown origin.