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.
(c) 2007 Wiley-Liss, Inc.
Publication types
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Comparative Study
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Research Support, Non-U.S. Gov't
MeSH terms
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Behavioral Symptoms / diagnosis
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Behavioral Symptoms / diagnostic imaging
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Behavioral Symptoms / etiology
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Behavioral Symptoms / genetics*
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Diseases in Twins
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Epilepsy / diagnosis
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Epilepsy / diagnostic imaging
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Epilepsy / etiology
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Epilepsy / genetics*
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Epilepsy / physiopathology
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Family*
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Female
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Humans
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Karyotyping
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Learning Disabilities / diagnosis
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Learning Disabilities / diagnostic imaging
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Learning Disabilities / etiology
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Learning Disabilities / genetics*
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Magnetic Resonance Imaging
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Male
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Malformations of Cortical Development / diagnosis
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Malformations of Cortical Development / diagnostic imaging
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Malformations of Cortical Development / etiology
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Malformations of Cortical Development / genetics*
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Phenotype
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Pseudobulbar Palsy / diagnostic imaging
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Pseudobulbar Palsy / genetics*
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Pseudobulbar Palsy / pathology
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Pseudobulbar Palsy / physiopathology
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Quadriplegia / diagnostic imaging
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Quadriplegia / genetics*
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Quadriplegia / pathology
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Quadriplegia / physiopathology
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Radiography
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Siblings
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Syndrome