How to cite this article: Clark M, Neville BGR. 2007. Familial and genetic associations in Worster-Drought syndrome and perisylvian disorders. Am J Med Genet Part A 146A:35–42.
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Research Article
Familial and genetic associations in Worster-Drought syndrome and perisylvian disorders†
Article first published online: 9 NOV 2007
DOI: 10.1002/ajmg.a.32015
Copyright © 2007 Wiley-Liss, Inc.
Additional Information
How to Cite
Clark, M. and Neville, B. G.R. (2008), Familial and genetic associations in Worster-Drought syndrome and perisylvian disorders. Am. J. Med. Genet., 146A: 35–42. doi: 10.1002/ajmg.a.32015
- †
Publication History
- Issue published online: 19 DEC 2007
- Article first published online: 9 NOV 2007
- Manuscript Accepted: 19 JUL 2007
- Manuscript Received: 26 JUN 2006
- Abstract
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Keywords:
- Worster-Drought syndrome;
- congenital bilateral perisylvian polymicrogyria syndrome
Abstract
Worster-Drought syndrome (WDS) is a distinct clinical phenotype, comprising a congenital pseudobulbar palsy usually in association with a mild tetraplegia and often additional impairments. The phenotype is identical to that described in congenital bilateral perisylvian polymicrogyria syndrome (CBPS) and appears to have several different causes and a significant familial incidence. This study draws from a database of children with WDS phenotype or perisylvian polymicrogyria, held at a tertiary center. The findings suggest that genetic factors are important for a significant proportion of children and points to considerable genetic heterogeneity. There are grounds for considering WDS and perisylvian polymicrogyria as a spectrum of perisylvian malfunction. © 2007 Wiley-Liss, Inc.