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January 1992

Double Cortex: A Neuronal Migration Anomaly as a Possible Cause of Lennox-Gastaut Syndrome

Author Affiliations

From the Section of Neurophysiology (Drs Ricci, Cusmai, Fusco, and Vigevano) and the Department of Neuroradiology (Dr Fariello), Bambino Gesù Children's Hospital, National Medical Research Institute, Rome, Italy.

Arch Neurol. 1992;49(1):61-64. doi:10.1001/archneur.1992.00530250065017

• Band heterotopia, or "double cortex," is a neuronal migration disorder that consists of a symmetrical subcortical neuronal band. The overlying cortex may be normal or macrogyric. We describe two severely mentally retarded girls, aged 14 and 18 years, who had band heterotopia and Lennox-Gastaut syndrome. Band heterotopia was evident in both hemispheres as a subcortical symmetrical layer isointense with gray matter on magnetic resonance T1- and T2-weighted images. Both patients had atonic seizures, atypical absences, and tonic seizures. The electroencephalograms in both cases showed frequent generalized paroxysms and slow background activity. The association of a Lennox-Gastaut syndrome with double cortex in these two patients and in a previously reported autopsy-confirmed case suggests that this malformation may be responsible for other similar cases.

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