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The late diagnosis of double cortex syndrome in a 36-year-old woman with resistant atonic seizures

https://doi.org/10.14412/2074-2711-2015-3-40-45

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Abstract

Subcortical laminar heterotopia (double cortex syndrome) is an orphan disease with an incidence of 1 to 200,000 people in the population. The cause of the disease is mutation of the gene DCX (synonyms: DBCN, XLIS) in chromosome Xq22.3-q23. The type of inheritance is X-linked dominant. Correct diagnosis requires a high degree of skills of a neurologist/epileptologist and a radiologist. The paper describes a clinical case of the late diagnosis of double cortex syndrome in a 36-year-old woman with a long history of resistant atonic seizures and mental retardation.

About the Authors

N. A. Shnayder
Prof. V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, Ministry of Health of Russia
Russian Federation

Neurological Centre of Epileptology, Neurogenetics and Brain Research, University Clinic,

Partisan Zheleznyak St., Krasnoyarsk 660022



D. V. Dmitrenko
Prof. V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, Ministry of Health of Russia
Russian Federation

Neurological Centre of Epileptology, Neurogenetics and Brain Research, University Clinic,

Partisan Zheleznyak St., Krasnoyarsk 660022



Yu. B. Govorina
Prof. V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, Ministry of Health of Russia
Russian Federation

Neurological Centre of Epileptology, Neurogenetics and Brain Research, University Clinic,

Partisan Zheleznyak St., Krasnoyarsk 660022



E. A. Kantimirova
Prof. V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, Ministry of Health of Russia
Russian Federation

Neurological Centre of Epileptology, Neurogenetics and Brain Research, University Clinic,

Partisan Zheleznyak St., Krasnoyarsk 660022



O. V. Alekseeva
Prof. V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, Ministry of Health of Russia
Russian Federation

Neurological Centre of Epileptology, Neurogenetics and Brain Research, University Clinic,

Partisan Zheleznyak St., Krasnoyarsk 660022



A. A. Molgachev
Medical Diagnostic Centre, S.M. Berezin International Institute of Biological Systems
Russian Federation
26, Kolomenskaya St., Build. 3, Krasnoyarsk 660037


A. A. Makarkin
Medical Diagnostic Centre, S.M. Berezin International Institute of Biological Systems
Russian Federation
26, Kolomenskaya St., Build. 3, Krasnoyarsk 660037


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For citation:


Shnayder N.A., Dmitrenko D.V., Govorina Y.B., Kantimirova E.A., Alekseeva O.V., Molgachev A.A., Makarkin A.A. The late diagnosis of double cortex syndrome in a 36-year-old woman with resistant atonic seizures. Neurology, Neuropsychiatry, Psychosomatics. 2015;7(3):40-45. (In Russ.) https://doi.org/10.14412/2074-2711-2015-3-40-45

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ISSN 2074-2711 (Print)
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