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Lissencephaly (smooth brain) refers to the configuration of the cortical mantle as seen in human neuronal migration disorders.   In cases of lissencephaly, the cerebral cortex is severely malformed such that the surface of the cortex lacks the gyri and sulci seen in normal brain as illustrated by the above MRI.  Patients with this brain malformation are severely mentally retarded, visually inattentive due to cortical blindness, and 80% have infantile spasms. Over 90% of patients with this disorder have seizures at some point in their life.

 

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Gordon and Mary Cain Pediatric Neurology Research Foundation Laboratories
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Last Modified: April 26, 2006