KEGG   DISEASE: Congenital suprabulbar paresis
Entry
H01837                      Disease                                
Name
Congenital suprabulbar paresis;
Worster Drought syndrome
Description
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.
Category
Nervous system disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
 08 Diseases of the nervous system
  Cerebral palsy
   8D23  Worster-Drought syndrome
    H01837  Congenital suprabulbar paresis
Other DBs
ICD-11: 8D23
MeSH: C536747
OMIM: 185480
Reference
  Authors
Clark M, Harris R, Jolleff N, Price K, Neville BG
  Title
Worster-Drought syndrome: poorly recognized despite severe and persistent difficulties with feeding and speech.
  Journal
Dev Med Child Neurol 52:27-32 (2010)
DOI:10.1111/j.1469-8749.2009.03475.x
Reference
PMID:3955865
  Authors
Patton MA, Baraitser M, Brett EM
  Title
A family with congenital suprabulbar paresis (Worster-Drought syndrome).
  Journal
Clin Genet 29:147-50 (1986)
DOI:10.1111/j.1399-0004.1986.tb01239.x
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