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

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

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)61-64
Number of pages4
JournalArchives of Neurology
Volume49
Issue number1
DOIs
Publication statusPublished - 1992

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology
  • Neuroscience(all)

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