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Abstract

Diffuse neuronal migration disorders associated with epilepsy can now be recognized by modern neuroimaging techniques, particularly high-resolution MRI. We report 10 patients with a recently described MRI picture of continuous or generalized band heterotopia underlying the cortical mantle, giving the appearance of a “double cortex.” They have epilepsy, and almost all have mental retardation. The epileptic disorder varies in nature and degree of severity. Patients may present with infantile spasms, a Lennox-Gastaut syndrome, or other forms of secondary generalized or multifocal epilepsy. Response to medical treatment is variable. Callosotomy may lead to considerable reduction of drop attacks, present in 60%. Mental retardation is usually mild or moderate, and only rarely severe. It correlates with the type of epileptic syndrome, and is greater in patients with more disorganized cortex overlying the heterotopia. Recognition of this entity by MRI is important for appropriate diagnosis of the epileptic disorder, planning of therapeutic strategy, and prognosis.

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Published In

Neurology®
Volume 41Number 10October 1991
Pages: 1656
PubMed: 1922811

Publication History

Published online: October 1, 1991
Published in print: October 1991

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Authors

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A. Palmini, MD
Department of Neurology and Neurosurgery (Drs. Palmini, F. Andermann, Tampieri, E. Andermann, and Robitaille), McGill University and Montreal Neurological Institute and Hospital, Montreal, Quebec, Canada; and Service de Neurologic Infantile (Drs. Dulac, Chaves, Ponsot, and Pinard), Hôpital Saint-Vincent-de-Paul, and Department of Pediatrics (Drs. Aicardi, Goutíères, and Livingston), Hôpital des Enfants Malades, Paris, France.
F. Andermann, MD, FRCP(C)
Department of Neurology and Neurosurgery (Drs. Palmini, F. Andermann, Tampieri, E. Andermann, and Robitaille), McGill University and Montreal Neurological Institute and Hospital, Montreal, Quebec, Canada; and Service de Neurologic Infantile (Drs. Dulac, Chaves, Ponsot, and Pinard), Hôpital Saint-Vincent-de-Paul, and Department of Pediatrics (Drs. Aicardi, Goutíères, and Livingston), Hôpital des Enfants Malades, Paris, France.
J. Aicardi, MD
Department of Neurology and Neurosurgery (Drs. Palmini, F. Andermann, Tampieri, E. Andermann, and Robitaille), McGill University and Montreal Neurological Institute and Hospital, Montreal, Quebec, Canada; and Service de Neurologic Infantile (Drs. Dulac, Chaves, Ponsot, and Pinard), Hôpital Saint-Vincent-de-Paul, and Department of Pediatrics (Drs. Aicardi, Goutíères, and Livingston), Hôpital des Enfants Malades, Paris, France.
O. Dulac, MD
Department of Neurology and Neurosurgery (Drs. Palmini, F. Andermann, Tampieri, E. Andermann, and Robitaille), McGill University and Montreal Neurological Institute and Hospital, Montreal, Quebec, Canada; and Service de Neurologic Infantile (Drs. Dulac, Chaves, Ponsot, and Pinard), Hôpital Saint-Vincent-de-Paul, and Department of Pediatrics (Drs. Aicardi, Goutíères, and Livingston), Hôpital des Enfants Malades, Paris, France.
F. Chaves, MD
Department of Neurology and Neurosurgery (Drs. Palmini, F. Andermann, Tampieri, E. Andermann, and Robitaille), McGill University and Montreal Neurological Institute and Hospital, Montreal, Quebec, Canada; and Service de Neurologic Infantile (Drs. Dulac, Chaves, Ponsot, and Pinard), Hôpital Saint-Vincent-de-Paul, and Department of Pediatrics (Drs. Aicardi, Goutíères, and Livingston), Hôpital des Enfants Malades, Paris, France.
G. Ponsot, MD
Department of Neurology and Neurosurgery (Drs. Palmini, F. Andermann, Tampieri, E. Andermann, and Robitaille), McGill University and Montreal Neurological Institute and Hospital, Montreal, Quebec, Canada; and Service de Neurologic Infantile (Drs. Dulac, Chaves, Ponsot, and Pinard), Hôpital Saint-Vincent-de-Paul, and Department of Pediatrics (Drs. Aicardi, Goutíères, and Livingston), Hôpital des Enfants Malades, Paris, France.
J. M. Pinard, MD
Department of Neurology and Neurosurgery (Drs. Palmini, F. Andermann, Tampieri, E. Andermann, and Robitaille), McGill University and Montreal Neurological Institute and Hospital, Montreal, Quebec, Canada; and Service de Neurologic Infantile (Drs. Dulac, Chaves, Ponsot, and Pinard), Hôpital Saint-Vincent-de-Paul, and Department of Pediatrics (Drs. Aicardi, Goutíères, and Livingston), Hôpital des Enfants Malades, Paris, France.
F. Goutières MD
Department of Neurology and Neurosurgery (Drs. Palmini, F. Andermann, Tampieri, E. Andermann, and Robitaille), McGill University and Montreal Neurological Institute and Hospital, Montreal, Quebec, Canada; and Service de Neurologic Infantile (Drs. Dulac, Chaves, Ponsot, and Pinard), Hôpital Saint-Vincent-de-Paul, and Department of Pediatrics (Drs. Aicardi, Goutíères, and Livingston), Hôpital des Enfants Malades, Paris, France.
J. Livingston, MD
Department of Neurology and Neurosurgery (Drs. Palmini, F. Andermann, Tampieri, E. Andermann, and Robitaille), McGill University and Montreal Neurological Institute and Hospital, Montreal, Quebec, Canada; and Service de Neurologic Infantile (Drs. Dulac, Chaves, Ponsot, and Pinard), Hôpital Saint-Vincent-de-Paul, and Department of Pediatrics (Drs. Aicardi, Goutíères, and Livingston), Hôpital des Enfants Malades, Paris, France.
D. Tampieri, MD
Department of Neurology and Neurosurgery (Drs. Palmini, F. Andermann, Tampieri, E. Andermann, and Robitaille), McGill University and Montreal Neurological Institute and Hospital, Montreal, Quebec, Canada; and Service de Neurologic Infantile (Drs. Dulac, Chaves, Ponsot, and Pinard), Hôpital Saint-Vincent-de-Paul, and Department of Pediatrics (Drs. Aicardi, Goutíères, and Livingston), Hôpital des Enfants Malades, Paris, France.
E. Andermann MD, PhD
Department of Neurology and Neurosurgery (Drs. Palmini, F. Andermann, Tampieri, E. Andermann, and Robitaille), McGill University and Montreal Neurological Institute and Hospital, Montreal, Quebec, Canada; and Service de Neurologic Infantile (Drs. Dulac, Chaves, Ponsot, and Pinard), Hôpital Saint-Vincent-de-Paul, and Department of Pediatrics (Drs. Aicardi, Goutíères, and Livingston), Hôpital des Enfants Malades, Paris, France.
Y. Robitaille, MD
Department of Neurology and Neurosurgery (Drs. Palmini, F. Andermann, Tampieri, E. Andermann, and Robitaille), McGill University and Montreal Neurological Institute and Hospital, Montreal, Quebec, Canada; and Service de Neurologic Infantile (Drs. Dulac, Chaves, Ponsot, and Pinard), Hôpital Saint-Vincent-de-Paul, and Department of Pediatrics (Drs. Aicardi, Goutíères, and Livingston), Hôpital des Enfants Malades, Paris, France.

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Cited By
  1. Reply to: “Susceptibility‐Weighted Imaging Reveals Subcortical Iron Deposition in PLAN : The “Double Cortex Sign”” , Movement Disorders, 38, 10, (1973-1973), (2023).https://doi.org/10.1002/mds.29592
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  2. Reply to: “ Susceptibility‐Weighted Imaging Reveals Subcortical Iron Deposition in PLAN : The ‘Double Cortex Sign’” , Movement Disorders, 38, 10, (1973-1974), (2023).https://doi.org/10.1002/mds.29591
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  3. Malformations of cortical development and epilepsy, Dialogues in Clinical Neuroscience, 10, 1, (47-62), (2022).https://doi.org/10.31887/DCNS.2008.10.1/rjleventer
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  4. Incremental changes in interhemispheric functional connectivity after two-stage corpus callosotomy in a patient with subcortical band heterotopia, Epilepsy & Behavior Reports, 18, (100525), (2022).https://doi.org/10.1016/j.ebr.2022.100525
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  5. Non-pharmacological treatment options of drug-resistant epilepsy in subcortical band heterotopia: systematic review and illustrative case, Child's Nervous System, 39, 2, (451-462), (2022).https://doi.org/10.1007/s00381-022-05638-w
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  6. Subcortical Band Heterotopia Presented With Refractory Epilepsy and Reversible Aphasia, Cureus, (2021).https://doi.org/10.7759/cureus.16990
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  7. Case Report: PAFAH1B1 Mutation and Posterior Band Heterotopia With Focal Temporal Lobe Epilepsy Treated by Responsive Neurostimulation, Frontiers in Neurology, 12, (2021).https://doi.org/10.3389/fneur.2021.779113
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  8. Neuronal migration disorders, Cellular Migration and Formation of Axons and Dendrites, (577-588), (2020).https://doi.org/10.1016/B978-0-12-814407-7.00026-2
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  9. Spontaneous epileptiform activity in a rat model of bilateral subcortical band heterotopia, Epilepsia, 60, 2, (337-348), (2018).https://doi.org/10.1111/epi.14633
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  10. Genetic generalized epilepsies, Epilepsia, 59, 6, (1148-1153), (2018).https://doi.org/10.1111/epi.14042
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  11. See more
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