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 language | English |
|---|---|
| Pages (from-to) | 61-64 |
| Number of pages | 4 |
| Journal | Archives of Neurology |
| Volume | 49 |
| Issue number | 1 |
| DOIs | |
| Publication status | Published - 1992 |
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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 journal › Article
}
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.
UR - http://www.scopus.com/inward/record.url?scp=0026542082&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0026542082&partnerID=8YFLogxK
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 -