Double cortex. A neuronal migration anomaly as a possible cause of Lennox-Gastaut syndrome

Arch Neurol. 1992 Jan;49(1):61-4. 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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Cerebral Cortex / abnormalities*
  • Cerebral Cortex / cytology
  • Cerebral Cortex / physiopathology
  • Electroencephalography
  • Epilepsy / etiology
  • Epilepsy / pathology*
  • Epilepsy / physiopathology
  • Female
  • Hemiplegia / etiology
  • Hemiplegia / pathology*
  • Hemiplegia / physiopathology
  • Humans
  • Magnetic Resonance Imaging
  • Neurons / pathology
  • Seizures / etiology
  • Seizures / pathology*
  • Seizures / physiopathology
  • Syndrome