Early ontogenic disturbances in cell migration in mentally disabled adult

Disturbances in cell migration are heterogenic disorders of brain developme nt commonly associated with epilepsy and mental retardation. We report a 45 -year-old oligophrenic man with defect of lower limbs and family history of mental retardation who died because of brainstem hemorrhage. At post-morte m and histopathological examination, complex brain malformation characteriz ed by bilateral periventricular heterotopia, cortical dysgenesia, partial a genesia of corpus callosum and thin-walled blood vessels was found. Immunoh istochemical examination revealed the presence of fibronectin, collagen IV and laminin in wall of pathological vessels. Cyclooxygenase-2 (COX-2) expre ssion in neurons within heterotopias and dysgenic cortex was negative. It m ay indicate their maturity and indirectly, normal activity of postsynaptic NMDA receptors that explains absence of epileptic attacks in our patient. T he presence of COX-2-negative neurons and compounds of basal lamina in feta l-like vessels within heterotopias and dysgenic cortex, suggests that these probably initially immature structures became mature during 45 years of pa tient's life.