ACKNOWLEDGEMENTS This study was supported in part by a grant from Sport Aiding Medical Research for Kids (SPARKS). The parent support group has been a significant source of information and encouragement. Professor S Reilly helped with the initial concept and Mike Clarke helped with data collection. Great Ormond Street Hospital for Children NHS Trust received research and development funding from the NHS Executive.
Worster-Drought syndrome: poorly recognized despite severe and persistent difficulties with feeding and speech
Article first published online: 12 OCT 2009
© The Authors. Journal compilation © Mac Keith Press 2009
Developmental Medicine & Child Neurology
Volume 52, Issue 1, pages 27–32, January 2010
How to Cite
CLARK, M., HARRIS, R., JOLLEFF, N., PRICE, K. and NEVILLE, B. G. (2010), Worster-Drought syndrome: poorly recognized despite severe and persistent difficulties with feeding and speech. Developmental Medicine & Child Neurology, 52: 27–32. doi: 10.1111/j.1469-8749.2009.03475.x
- Issue published online: 18 DEC 2009
- Article first published online: 12 OCT 2009
- PUBLICATION DATA Accepted for publication 17th June 2009. Published online.
Aim 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.
Method 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.
Interpretation 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.