Lissencephaly includes a range of severe brain malformations, including agyria (absent gyri), pachygyria (broad gyri), and subcortical band heterotopia. In lissencephaly (which literally means smooth brain), the surface of the brain appears smooth. It may occur as an isolated lissencephaly or in association with certain syndromes (Miller-Dieker syndrome).
Lissencephaly is caused by a defect in neuronal migration during embryonic development between 12 and 24 weeks of gestation that results in the absence of normal development of brain gyri and sulci. Children with lissencephaly present with significant developmental delays and mental disability, but these vary from child to child depending on the degree of brain malformation and intractable epilepsy.
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