An infant with isolated Lissencephaly

Abstract

Lissencephaly or agyria is a prototype of disorders of neuronal migration, a rare type of hereditary malformation of the brain, which manifests with smooth cerebral surface, poorly defined sylvian fissures with thickened cerebral cortical mantle. A case of an infant with isolated variety is presented highlighting some of the major associated clinical features, which include profound intellectual impairment, seizures and hypotonia that later evolves to spasticity in the 2nd year of life. Morbidity is usually high and mortality is dependent on associated deficits.