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March 1994

Familial Diffuse Cortical Dysplasia

Author Affiliations

From the Department of Neurology, University of Alabama Epilepsy Center, University of Alabama at Birmingham School of Medicine.

Arch Neurol. 1994;51(3):307-310. doi:10.1001/archneur.1994.00540150109027

Two brothers are described with a syndrome characterized by mental retardation, atypical absence, atonic and generalized tonic-clonic seizures, and bilateral symmetrical slow spike and wave discharges on electroencephalograms. Magnetic resonance imaging revealed dysplastic cortex probably representing pachygyria primarily over the parietal regions bilaterally in both patients. The presence of an identical clinical, electroencephalographic, and cortical developmental abnormality on imaging studies in these brothers suggests that a genetic mechanism may play a role in some developmental disorders of the cerebral cortex.

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