Double cortex. A neuronal migration anomaly as a possible cause of Lennox-Gastaut syndrome.
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.
Ricci
S
S
Section of Neurophysiology, Bambino Gesù Children's Hospital, Rome, Italy.
Cusmai
R
R
Fariello
G
G
Fusco
L
L
Vigevano
F
F
D000293:Adolescent; D002540:Cerebral Cortex / Q000002:abnormalities* / Q000166:cytology / Q000503:physiopathology; D004569:Electroencephalography; D004827:Epilepsy / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D006429:Hemiplegia / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D009474:Neurons / Q000473:pathology; D012640:Seizures / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology; D013577:Syndrome