Worcester drought syndrome - a form of bulbar cerebral palsy.

摘要

A 7-y-old boy presented with non-progressive speech disturbances, drooling and difficulty in swallowing food (liquids > solids) since early childhood. His motor and social milestones were age-appropriate. He had no convulsions, behavioral disturbances, hearing or visual deficits. Family and birth history were non-significant. He had normal head circumference. Neurological examination revealed normal cognition, spastic dysarthria and pseudobulbar palsy. Motor tone, power and muscle stretch reflexes were normal. Rest of the systemic examination was unremarkable. Magnetic Resonance Imaging of the brain revealed bilateral perisyl-vian polymicrogyria (Fig. 1). The clinico-radiological presentation was consistent with Worcester Drought Syndrome (WDS). WDS, congenital suprabulbar paresis, may be considered a form of bulbar cerebral palsy. It causes permanent bulbar dysfunction characterized by problems with swallowing, feeding, drooling, speech and airway protection. It may be associated with mild spastic quadriplegia. The neuroimag-ing may show bilateral perisylvian polymicrogyria [1]. Clark et al. studied 121 children with WDS and found unilateral or bilateral perisylvian polymicrogyria in 42 % children. WDS may lie on a spectrum of disorders of congenital perisylvian dysfunction [2].