MRI spectrum of cortical malformations in tuberous sclerosis complex

The diagnostic and prognostic value of magnetic resonance imaging in the tu berous sclerosis complex has increasingly been recognized. In this paper, w e review the presumed pathogenesis of the cerebral dysgenesis seen in this condition in the light of magnetic resonance imaging features of selected p atients. In addition to typical findings related to tubers, we show and dis cuss varied cortical malformations (from simple localized cortical dysplasi a to transmantle dysplasia and schizencephaly) similar to those seen in spo radic cerebral dysgenesis. These cases support the hypothesis that the tube rous sclerosis complex focally affects the radial glial-neuronal complex as a basic unit for brain development. Abnormal stem cells would create dyspl astic glia and neurons that fail to differentiate, proliferate, migrate and form a normally organized cortex. (C) 2000 Elsevier Science B.V. All right s reserved.