Lissencephaly

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Abstract
Two babies with lissencephaly are presented in whom extensive neurological evaluation was performed before death. They had the same clinical pathological pictures. The pathological findings are similar to those described previously. There was an absence of sulci over the cerebral hemispheres poorly developed primary fissures a thick cerebral mantle hypoplastic white matter in the cerebral hemispheres colpocephaly heterotopia of grey matter abnormal lamination of the cerebral cortex microencephaly. Such cases can be readily differentiated from cases of pachygyria focal agyria other morphologically similar anomalies. Patients with lissencephaly can be distinguished from those with other brain anomalies by their appearance. They are microcephalic demonstrate severe motor retardation fail to thrive. They are decerebrate unresponsive to environmental stimuli. They have an early onset of convulsions recurrent infections die before 2 years of age. Minor congenital anomalies are often noted striking similarity in facial appearance has been found. Similar clinical pictures were found in instances of lissencephaly in the literature. Definite abnormalities were indicated by the electroencephalogram pneumoencephalogram. Chromosomal studies were normal. The clinical picture the findings on the special studies may make It possible to make this diagnosis during life.

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