Abstract
Malformations of the cerebral cortex are being recognized more frequently as a cause
of epilepsy, developmental delay, neurological deficits, and mental retardation. Nonetheless,
a standard nomenclature and classification system of these malformations, based upon
state-of-the art knowledge derived from genetics, embryology, imaging, and pathology,
has not been devised. In this manuscript, we propose such a classification system.
Moreover, we have constructed the system such that both the framework and the classifications
themselves are flexible and can be adapted as our knowledge of the embryology, genetics,
imaging, and pathology of these disorders advances. We believe that the use of this
classification system will help both clinicians and researchers to understand and
think about these disorders and their causes better. In turn, we hope that this improved
understanding will lead to further refinements in classification, to advances in our
knowledge and, ultimately, to improvements in therapy.
Key words
Agyria - Heterotopia - Lissencephaly - Neuronal migration - Polymicrogyria
