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

Stefano Ricci, MD; Raffaella Cusmai, MD; Giuseppe Fariello, MD; Lucia Fusco, MD; Federico Vigevano, MD

Arch Neurol. 1992;49(1):61-64.

Abstract
• 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.

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.

Footnotes
Accepted for publication July 11, 1991.

Reprint requests to Section of Neurophysiology, Bambino Gesù Children's Hospital, National Medical Research Institute, Piazza S. Onofrio 4, 00165 Rome, Italy (Dr Vigevano).

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