Worster-Drought syndrome: poorly recognized despite severe and persistent difficulties with feeding and speech.
OBJECTIVE
Worster-Drought syndrome (WDS), or congenital suprabulbar paresis, 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. As such, it falls within the cerebral palsies. The aim of this study was to describe the physical and neuropsychological profiles of children with WDS.


METHODS
Forty-two children with WDS (26 males, 16 females; mean age 7y 10mo, SD 3y 1mo; range 2y 6mo to 16y 5mo) were studied prospectively using a standard protocol.


RESULTS
All of the children had severe bulbar dysfunction; 36 out of 42 had feeding difficulties and 23 of 38 had unintelligible speech, which was poorly compensated for by augmentative communication. There were accompanying disturbances in cognition (mean non-verbal IQ 59), behaviour (12/40 attention-deficit-hyperactivity disorder [ADHD]), social communication (8/42 autism), and epilepsy (12/39). The severity of bulbar dysfunction and impact of additional impairments made it difficult to use formal assessments.


CONCLUSIONS
WDS causes severe and persistent bulbar dysfunction that is often accompanied by additional impairments, as in other cerebral palsies. Speech prognosis is particularly poor. Early diagnosis with appreciation of the underlying neurology would encourage critical evaluation of interventions and long-term planning to improve outcome.
Clark
Maria
M
Great Ormond Street Hospital for Children NHS Trust, London, UK.
Harris
Rebecca
R
Jolleff
Nicola
N
Price
Katie
K
Neville
Brian G R
BG
D000293:Adolescent; D001289:Attention Deficit Disorder with Hyperactivity / Q000175:diagnosis / Q000235:genetics / Q000534:rehabilitation; D002547:Cerebral Palsy / Q000175:diagnosis* / Q000235:genetics / Q000534:rehabilitation; D002648:Child; D002659:Child Development Disorders, Pervasive / Q000175:diagnosis / Q000235:genetics / Q000534:rehabilitation; D002675:Child, Preschool; D015897:Comorbidity; D003680:Deglutition Disorders / Q000175:diagnosis / Q000235:genetics / Q000534:rehabilitation; D003937:Diagnosis, Differential; D004827:Epilepsy / Q000175:diagnosis / Q000235:genetics / Q000534:rehabilitation; D019959:Feeding and Eating Disorders of Childhood / Q000175:diagnosis* / Q000235:genetics / Q000534:rehabilitation; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000175:diagnosis / Q000235:genetics / Q000534:rehabilitation; D007805:Language Development Disorders / Q000175:diagnosis* / Q000235:genetics / Q000534:rehabilitation; D007807:Language Tests; D008297:Male; D009460:Neurologic Examination; D013064:Speech Disorders / Q000175:diagnosis* / Q000235:genetics / Q000534:rehabilitation; D013068:Speech Production Measurement; D013577:Syndrome