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January 1, 1999

Evidence for nodular epileptogenicity and gender differences in periventricular nodular heterotopia

January 1, 1999 issue
52 (2) 336

Abstract

Objective: To determine whether clinical differences between the sexes seen in periventricular nodular heterotopia (PNH) have structural correlates on imaging.
Background: PNH is the most common dysgenesis associated with hippocampal sclerosis (HS). Women with PNH have normal intellect; men may have mental retardation and other changes. Familial PNH, seen in women, is linked to Xq28—a region also abnormal in a sporadic male infant with PNH and retardation—suggesting sexual differences in gene expression. Epilepsy associated with PNH may be refractory to drugs, and surgery for associated HS does not stop seizures, suggesting intrinsic epileptogenicity of PNH.
Methods: Quantitative MRI analysis was performed using established techniques for detecting subtle structural changes in 13 female patients (11 sporadic and two familial) and four male patients (sporadic).
Results: There is structural heterogeneity in PNH, even in patients with bilateral PNH. On MRI, men have more cerebral abnormalities beyond PNH than control subjects or female patients (p < 0.005).
Conclusions: The findings support the concept of intrinsic epileptogenicity of PNH. There may be additional structural abnormalities relevant to seizure generation, especially in men. Structural heterogeneity, and widespread abnormalities, may need consideration when patients are referred for surgical treatment or when additional studies of patients with PNH are conducted.

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Information

Published In

Neurology®
Volume 52Number 2January 1, 1999
Pages: 336
PubMed: 9932953

Publication History

Received: April 30, 1998
Accepted: October 10, 1998
Published online: January 1, 1999
Published in print: January 1, 1999

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Authors

Affiliations & Disclosures

S.M. Sisodiya, MRCP, PhD
From the Epilepsy Research Group (Drs. SisodiyaFree, Fish, Shorvon, and Everitt), University Department of Clinical Neurology and the Department of Neuropathology (Dr. Thom), Institute of Neurology, London, UK.
S.L. Free, PhD
From the Epilepsy Research Group (Drs. SisodiyaFree, Fish, Shorvon, and Everitt), University Department of Clinical Neurology and the Department of Neuropathology (Dr. Thom), Institute of Neurology, London, UK.
M. Thom, MRCPath
From the Epilepsy Research Group (Drs. SisodiyaFree, Fish, Shorvon, and Everitt), University Department of Clinical Neurology and the Department of Neuropathology (Dr. Thom), Institute of Neurology, London, UK.
A.E. Everitt, MRCP
From the Epilepsy Research Group (Drs. SisodiyaFree, Fish, Shorvon, and Everitt), University Department of Clinical Neurology and the Department of Neuropathology (Dr. Thom), Institute of Neurology, London, UK.
D.R. Fish, MD, FRCP
From the Epilepsy Research Group (Drs. SisodiyaFree, Fish, Shorvon, and Everitt), University Department of Clinical Neurology and the Department of Neuropathology (Dr. Thom), Institute of Neurology, London, UK.
S.D. Shorvon, MD, FRCP
From the Epilepsy Research Group (Drs. SisodiyaFree, Fish, Shorvon, and Everitt), University Department of Clinical Neurology and the Department of Neuropathology (Dr. Thom), Institute of Neurology, London, UK.

Notes

Address correspondence and reprint requests to Dr. S.M. Sisodiya, Institute of Neurology, Queen Square, London, UK WC1N 3BG.

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  1. Hippocampal deep brain stimulation: a therapeutic option in patients with extensive bilateral periventricular nodular heterotopia: a case report, Epileptic Disorders, 22, 5, (664-668), (2020).https://doi.org/10.1684/epd.2020.1206
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  3. Brain structural and neuroendocrine basis of sex differences in epilepsy, Sex Differences in Neurology and Psychiatry, (223-233), (2020).https://doi.org/10.1016/B978-0-444-64123-6.00016-3
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  5. The neuroendocrine basis of sex differences in epilepsy, Pharmacology Biochemistry and Behavior, 152, (97-104), (2017).https://doi.org/10.1016/j.pbb.2016.07.002
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  8. Sex differences in the neurobiology of epilepsy: A preclinical perspective, Neurobiology of Disease, 72, (180-192), (2014).https://doi.org/10.1016/j.nbd.2014.07.004
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  9. A case report of surgically treated drug resistant epilepsy associated with subependymal nodular heterotopia, Seizure, 21, 3, (223-226), (2012).https://doi.org/10.1016/j.seizure.2011.11.002
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  10. Malformations of Cortical Development, Swaiman's Pediatric Neurology, (202-231), (2012).https://doi.org/10.1016/B978-1-4377-0435-8.00026-3
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