Epilepsy in Sturge-Weber syndrome: a literature review and description of a clinical case

The Struge-Weber syndrome is the third most common neurodermal disease after neurofibromatosis and tuberous sclerosis. This disease is not inherited, but occurs exclusively sporadically, both in men and in women, and in all races and ethnic groups. In 90% of cases, this syndrome is diagnosed in pedi...

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Main Authors: N. I. Shova, V. A. Mikhailov, S. A. Korovina, D. V. Alekseeva
Format: Article
Language:Russian
Published: IRBIS LLC 2020-04-01
Series:Эпилепсия и пароксизмальные состояния
Subjects:
Online Access:https://www.epilepsia.su/jour/article/view/520
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author N. I. Shova
V. A. Mikhailov
S. A. Korovina
D. V. Alekseeva
author_facet N. I. Shova
V. A. Mikhailov
S. A. Korovina
D. V. Alekseeva
author_sort N. I. Shova
collection DOAJ
description The Struge-Weber syndrome is the third most common neurodermal disease after neurofibromatosis and tuberous sclerosis. This disease is not inherited, but occurs exclusively sporadically, both in men and in women, and in all races and ethnic groups. In 90% of cases, this syndrome is diagnosed in pediatric practice. Adult cases of this syndrome are sporadic, since they remain unrecognized due to the polymorphism of this disease. Comorbidity between epilepsy and the Sturge-Weber syndrome was noted.Aim. To conduct a literature analysis and describe the clinical observation of epilepsy with the Sturge-Weber syndrome.Materials and methods. Patient M., 50 years old has been observed for a long time due to complaints on episodes of numbness according to the hematotype with transient paresis. The examination (MRI) revealed changes characteristic for Sturge-Weber syndrome. The presence of specific paroxysmal changes according to the results of electroencephalography helped to verify the genesis of paroxysmal conditions.Results. The patient underwent a comprehensive examination. As a result, the diagnosis of epilepsy was verified. The prescription of adequate antiepileptic therapy allowed for stabilization and jugulation of epileptic seizures.Conclusion. In this clinical observation, we focus on the differential diagnosis between transient ischemic attack and epilepsy. Unfortunately, at the moment, adults with a previously undetectable syndrome undergo multiple hospitalizations and do not receive antiepileptic therapy, since the prior disease (epilepsy) is not verified.
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spelling doaj.art-34a476959731402283e7ce8173666e602023-03-13T07:44:17ZrusIRBIS LLCЭпилепсия и пароксизмальные состояния2077-83332311-40882020-04-01121677310.17749/2077-8333.2020.12.1.67-73482Epilepsy in Sturge-Weber syndrome: a literature review and description of a clinical caseN. I. Shova0V. A. Mikhailov1S. A. Korovina2D. V. Alekseeva3Bekhterev National Medical Research Center of Psychiatry and NeurologyBekhterev National Medical Research Center of Psychiatry and NeurologyBekhterev National Medical Research Center of Psychiatry and NeurologyBekhterev National Medical Research Center of Psychiatry and NeurologyThe Struge-Weber syndrome is the third most common neurodermal disease after neurofibromatosis and tuberous sclerosis. This disease is not inherited, but occurs exclusively sporadically, both in men and in women, and in all races and ethnic groups. In 90% of cases, this syndrome is diagnosed in pediatric practice. Adult cases of this syndrome are sporadic, since they remain unrecognized due to the polymorphism of this disease. Comorbidity between epilepsy and the Sturge-Weber syndrome was noted.Aim. To conduct a literature analysis and describe the clinical observation of epilepsy with the Sturge-Weber syndrome.Materials and methods. Patient M., 50 years old has been observed for a long time due to complaints on episodes of numbness according to the hematotype with transient paresis. The examination (MRI) revealed changes characteristic for Sturge-Weber syndrome. The presence of specific paroxysmal changes according to the results of electroencephalography helped to verify the genesis of paroxysmal conditions.Results. The patient underwent a comprehensive examination. As a result, the diagnosis of epilepsy was verified. The prescription of adequate antiepileptic therapy allowed for stabilization and jugulation of epileptic seizures.Conclusion. In this clinical observation, we focus on the differential diagnosis between transient ischemic attack and epilepsy. Unfortunately, at the moment, adults with a previously undetectable syndrome undergo multiple hospitalizations and do not receive antiepileptic therapy, since the prior disease (epilepsy) is not verified.https://www.epilepsia.su/jour/article/view/520sturge-weber syndromeepilepsyphacomatosistransient ischemic attackclinical case
spellingShingle N. I. Shova
V. A. Mikhailov
S. A. Korovina
D. V. Alekseeva
Epilepsy in Sturge-Weber syndrome: a literature review and description of a clinical case
Эпилепсия и пароксизмальные состояния
sturge-weber syndrome
epilepsy
phacomatosis
transient ischemic attack
clinical case
title Epilepsy in Sturge-Weber syndrome: a literature review and description of a clinical case
title_full Epilepsy in Sturge-Weber syndrome: a literature review and description of a clinical case
title_fullStr Epilepsy in Sturge-Weber syndrome: a literature review and description of a clinical case
title_full_unstemmed Epilepsy in Sturge-Weber syndrome: a literature review and description of a clinical case
title_short Epilepsy in Sturge-Weber syndrome: a literature review and description of a clinical case
title_sort epilepsy in sturge weber syndrome a literature review and description of a clinical case
topic sturge-weber syndrome
epilepsy
phacomatosis
transient ischemic attack
clinical case
url https://www.epilepsia.su/jour/article/view/520
work_keys_str_mv AT nishova epilepsyinsturgewebersyndromealiteraturereviewanddescriptionofaclinicalcase
AT vamikhailov epilepsyinsturgewebersyndromealiteraturereviewanddescriptionofaclinicalcase
AT sakorovina epilepsyinsturgewebersyndromealiteraturereviewanddescriptionofaclinicalcase
AT dvalekseeva epilepsyinsturgewebersyndromealiteraturereviewanddescriptionofaclinicalcase