Worster-Drought syndrome. Neurological pattern (case report)

Abstract

L.M. Shchugareva1,2, O.V. Poteshkina1,2, V.D. Petrova2, P.A. Salamanov2 1Children’s City Hospital No. 1, St. Petersburg, Russian Federation 2I.I. Mechnikov North-Western State Medical University St. Petersburg, Russian Federation 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. Keywords: Worster-Drought syndrome, congenital pseudobulbar palsy, seizures, dysarthria, developmental delay, microcephalia, polymicrogyria. For citation: 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.