TY - JOUR
T1 - Double Cortex
T2 - A Neuronal Migration Anomaly as a Possible Cause of Lennox-Gastaut Syndrome
AU - Ricci, Stefano
AU - Cusmai, Raffaella
AU - Fariello, Giuseppe
AU - Fusco, Lucia
AU - Vigevano, Federico
PY - 1992
Y1 - 1992
N2 - 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.
AB - 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.
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U2 - 10.1001/archneur.1992.00530250065017
DO - 10.1001/archneur.1992.00530250065017
M3 - Article
C2 - 1728265
AN - SCOPUS:0026542082
VL - 49
SP - 61
EP - 64
JO - Archives of Neurology
JF - Archives of Neurology
SN - 0003-9942
IS - 1
ER -