Worster-Drought syndrome. Neurological pattern (case report)

<p> <b>L.M. Shchugareva<sup>1,2</sup>, O.V. Poteshkina<sup>1,2</sup>, V.D. Petrova<sup>2</sup>, P.A. Salamanov<sup>2</sup></b> </p> <p> <sup>1</sup>Children’s City Hospital No. 1, St. Petersburg, Russian Fede...

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Main Authors: O.V. Poteshkina, V.D. Petrova, P.A. Salamanov
Format: Article
Language:Russian
Published: LCC «Medicine-Inform» 2022-02-01
Series:РМЖ. Мать и дитя
Online Access:http://wchjournal.com/upload/iblock/266/266c15ae942fc25ca45d1ce23755ede4.pdf
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author O.V. Poteshkina
O.V. Poteshkina
V.D. Petrova
P.A. Salamanov
author_facet O.V. Poteshkina
O.V. Poteshkina
V.D. Petrova
P.A. Salamanov
author_sort O.V. Poteshkina
collection DOAJ
description <p> <b>L.M. Shchugareva<sup>1,2</sup>, O.V. Poteshkina<sup>1,2</sup>, V.D. Petrova<sup>2</sup>, P.A. Salamanov<sup>2</sup></b> </p> <p> <sup>1</sup>Children’s City Hospital No. 1, St. Petersburg, Russian Federation </p> <p> <sup>2</sup>I.I. Mechnikov North-Western State Medical University St. Petersburg, Russian Federation </p> <p> Worster-Drought syndrome (WDS) is a rare neurological disorder characterized by dissociation of voluntary and involuntary movements of the face, mouth, tongue, dysarthria, and oromotor dyspraxia. WDS rate varies from 1–9 per 100,000 to 1 per 25,000–30,000 newborns. Anterior (frontal) operculum, including the lower Rolandic area. WDS is diagnosed in the neonatal period based on a typical phenotype and neuroimaging findings. The prognosis is determined by medical care, epilepsy management, and rehabilitation. We describe a child with a severe WDS phenotype. In this child, abnormal intrauterine neuronal migration was associated with established cytomegalovirus meningoencephalitis in the neonatal period. Moreover, phenotypic signs (dissociation of voluntary and involuntary movements, dysarthria, severe salivation, microcephaly, and epilepsy) were coupled with neuroimaging findings (bilateral polymicrogyria of the opercular area and microcephalia). Pediatricians should be awa re of WDS clinical polymorphism. The knowledge base is upgraded annually. </p> <p> <b>Keywords</b>: Worster-Drought syndrome, congenital pseudobulbar palsy, seizures, dysarthria, developmental delay, microcephalia, polymicrogyria. </p> <p> <i><b>For citation: </b>Shchugareva L.M., Poteshkina O.V., Petrova V.D., Salamanov P.A. Worster-Drought syndrome. Neurological pattern (case report). Russian Journal of Woman and Child Health. 2022;5(1):90–96 (in Russ.). DOI: 10.32364/2618-8430-2022-5-1-90-96.</i> </p>
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spelling doaj.art-14d072ff31534c31bacc3ae3cf7d30b02024-02-26T10:13:33ZrusLCC «Medicine-Inform»РМЖ. Мать и дитя2618-84302686-71842022-02-015133556Worster-Drought syndrome. Neurological pattern (case report)O.V. Poteshkina0O.V. Poteshkina1V.D. Petrova2P.A. Salamanov3Russian Journal of Woman and Child Health, Publisher of «Medicina-Inform» Address for correspondence: Russia, 105064, Moscow, P.O. Box 399Russian Journal of Woman and Child Health, Publisher of «Medicina-Inform» Address for correspondence: Russia, 105064, Moscow, P.O. Box 399Russian Journal of Woman and Child Health, Publisher of «Medicina-Inform» Address for correspondence: Russia, 105064, Moscow, P.O. Box 399Russian Journal of Woman and Child Health, Publisher of «Medicina-Inform» Address for correspondence: Russia, 105064, Moscow, P.O. Box 399<p> <b>L.M. Shchugareva<sup>1,2</sup>, O.V. Poteshkina<sup>1,2</sup>, V.D. Petrova<sup>2</sup>, P.A. Salamanov<sup>2</sup></b> </p> <p> <sup>1</sup>Children’s City Hospital No. 1, St. Petersburg, Russian Federation </p> <p> <sup>2</sup>I.I. Mechnikov North-Western State Medical University St. Petersburg, Russian Federation </p> <p> Worster-Drought syndrome (WDS) is a rare neurological disorder characterized by dissociation of voluntary and involuntary movements of the face, mouth, tongue, dysarthria, and oromotor dyspraxia. WDS rate varies from 1–9 per 100,000 to 1 per 25,000–30,000 newborns. Anterior (frontal) operculum, including the lower Rolandic area. WDS is diagnosed in the neonatal period based on a typical phenotype and neuroimaging findings. The prognosis is determined by medical care, epilepsy management, and rehabilitation. We describe a child with a severe WDS phenotype. In this child, abnormal intrauterine neuronal migration was associated with established cytomegalovirus meningoencephalitis in the neonatal period. Moreover, phenotypic signs (dissociation of voluntary and involuntary movements, dysarthria, severe salivation, microcephaly, and epilepsy) were coupled with neuroimaging findings (bilateral polymicrogyria of the opercular area and microcephalia). Pediatricians should be awa re of WDS clinical polymorphism. The knowledge base is upgraded annually. </p> <p> <b>Keywords</b>: Worster-Drought syndrome, congenital pseudobulbar palsy, seizures, dysarthria, developmental delay, microcephalia, polymicrogyria. </p> <p> <i><b>For citation: </b>Shchugareva L.M., Poteshkina O.V., Petrova V.D., Salamanov P.A. Worster-Drought syndrome. Neurological pattern (case report). Russian Journal of Woman and Child Health. 2022;5(1):90–96 (in Russ.). DOI: 10.32364/2618-8430-2022-5-1-90-96.</i> </p>http://wchjournal.com/upload/iblock/266/266c15ae942fc25ca45d1ce23755ede4.pdf
spellingShingle O.V. Poteshkina
O.V. Poteshkina
V.D. Petrova
P.A. Salamanov
Worster-Drought syndrome. Neurological pattern (case report)
РМЖ. Мать и дитя
title Worster-Drought syndrome. Neurological pattern (case report)
title_full Worster-Drought syndrome. Neurological pattern (case report)
title_fullStr Worster-Drought syndrome. Neurological pattern (case report)
title_full_unstemmed Worster-Drought syndrome. Neurological pattern (case report)
title_short Worster-Drought syndrome. Neurological pattern (case report)
title_sort worster drought syndrome neurological pattern case report
url http://wchjournal.com/upload/iblock/266/266c15ae942fc25ca45d1ce23755ede4.pdf
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AT pasalamanov worsterdroughtsyndromeneurologicalpatterncasereport