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 journalArticle

51 Citations (Scopus)

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

Fingerprint

Seizures
Group II Malformations of Cortical Development
Absence Epilepsy
Mentally Disabled Persons
Electroencephalography
Autopsy
Magnetic Resonance Spectroscopy
Lennox Gastaut Syndrome
Double cortex
Anomaly
Syndrome
Causes
Cortex
Heterotopia
Gray Matter

ASJC Scopus subject areas

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

Cite this

Double Cortex : A Neuronal Migration Anomaly as a Possible Cause of Lennox-Gastaut Syndrome. / Ricci, Stefano; Cusmai, Raffaella; Fariello, Giuseppe; Fusco, Lucia; Vigevano, Federico.

In: Archives of Neurology, Vol. 49, No. 1, 1992, p. 61-64.

Research output: Contribution to journalArticle

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