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Surgical treatment of drug resistant epilepsy in Sturge-Weber syndrome: review of the literature and clinical case presentation

https://doi.org/10.17749/2077-8333.2019.11.1.53-62

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Abstract

The paper addresses the relatively rare inherited neurodermal disorder – Sturge-Weber syndrome that can manifest in epileptic seizures. We describe updated concepts, epidemiology, etiology, pathogenesis, clinical manifestations, and surgical treatment of the disease. We examined medical records of 21 patients (aged from 1 to 11 years) with Sturge-Weber syndrome treated over the period of 1996-2016. After surgical treatment of 10 patients (five cases with hemispheretomy and five – with multifocal resection), positive outcomes (Engel class I, II) were found in 70% of cases, and negative (Engel class III, IV) – in 20% of cases. Оne child suddenly died during epileptic seizures. In non-operated children (age from 2 to 5 years) under our observation, an improvement was noted in six cases, no changes – in three cases, and a further progression of the disease – in three cases. In this article, we analyze two of these cases in detail.

About the Authors

G. S. Ibatova
National Center of Neurosurgery
Kazakhstan

MD, PhD, Neurologist, Department of Pediatric Neurosurgery

34/1 Turan Str., Astana 010000, Republic of Kazakhstan



S. K. Akshulakov
National Center of Neurosurgery
Kazakhstan

PhD, MD, Professor of Neurosurgery, Head

34/1 Turan Str., Astana 010000, Republic of Kazakhstan

+7(701)1113271



S. M. Malyshev
Almazov National Medical Research Center
Russian Federation

MD, PhD Student, Department of Nervous Diseases and Psychiatry

2 Akkuratova Str., St Petersburg 197341, Russia

Scopus ID: 57192810951; Researcher ID: N-7251-2016



R. G. Khachatryan
Almazov National Medical Research Center
Russian Federation

MD, PhD Student, Department of Nervous Diseases and Psychiatry

2 Akkuratova Str., St Petersburg 197341, Russia

Researcher ID: C-6929-2019.



T. M. Alekseeva
Almazov National Medical Research Center
Russian Federation

MD, PhD, Head of the Department of Nervous Diseases and Psychiatry

2 Akkuratova Str., St Petersburg 197341, Russia



A. S. Shershever
Sverdlovsk Regional Oncology Center
Russian Federation

MD, Professor at the Department of Nervous Diseases, Neurosurgery and Medical Genetics, Ural State Medical University; Neurologist and Neurosurgeon, Sverdlovsk Regional Oncological Hospital

29 Soboleva Str., Ekaterinburg 620036, Russia



W. A. Khachatryan
Almazov National Medical Research Center
Russian Federation

MD, PhD, Professor of Neurosurgery, Head of the Department of Pediatric Neurosurgery

2 Akkuratova Str., St Petersburg 197341, Russia

Scopus ID: 55633841500; Researcher ID: G-4002-2018



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For citation:


Ibatova G.S., Akshulakov S.K., Malyshev S.M., Khachatryan R.G., Alekseeva T.M., Shershever A.S., Khachatryan W.A. Surgical treatment of drug resistant epilepsy in Sturge-Weber syndrome: review of the literature and clinical case presentation. Epilepsy and paroxysmal conditions. 2019;11(1):53-62. (In Russ.) https://doi.org/10.17749/2077-8333.2019.11.1.53-62

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ISSN 2077-8333 (Print)
ISSN 2311-4088 (Online)