Malformations of cerebral cortical development are common birth defects tha
t can cause delayed development, epilepsy, focal neurologic deficits, and m
ental retardation. Rational classification of these disorders is essential
for proper prognosis, genetic testing and counseling, and investigation of
the underlying molecular causes. A rational approach to this classification
is a framework based on whether these disorders are the result of abnormal
cell proliferation or apoptosis, abnormal migration of immature neurons, o
r abnormal horizontal and radial orientation in the cortex. Superimposed on
this framework are subclassifications that are based on topology of the ma
lformation, associated central nervous system (CNS) or extra-CNS malformati
ons, and results of molecular genetic testing. Characteristics that correla
te with and enforce this system of classification can be identified by mode
rn neuroimaging studies. Curr Opin Neurol 14:145-149 (C) 2001 Lippincott Wi
lliams & Wilkins.