Congenital suprabulbar paresis

Definition:

Congenital suprabulbar paresis (Worster- Drought syndrome) is a permanent movement disorder of the bulbar muscles causing persistent difficulties with swallowing, feeding, speech, and saliva control owing to a non-progressive disturbance in early brain development. Owing to the paresis of the orbicularis oris the patient is unable to round the lips or to blow, but as other facial muscles are not affected he can smile normally and close his eyes. An increased jaw jerk and a positive snout reflex are sometimes found, suggesting an upper motor neurone lesion. Congenital suprabulbar paresis has not yet been recognised in the literature as a genetic entity but occasional familial cases suggest that it may have a genetic basis.

References:

[1]. M A Patton, et al. A family with congenital suprabulbar paresis (Worster-Drought syndrome). Clin Genet. 1986 Feb;29(2):147-50. [Content Brief]

[2]. Maria Clark, et al. Worster-Drought syndrome: poorly recognized despite severe and persistent difficulties with feeding and speech. Dev Med Child Neurol. 2010 Jan;52(1):27-32. [Content Brief]

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