Worster-Drought Syndrome: Poorly Recognized despite Severe and Persistent Difficulties with Feeding and Speech

Autor/inn/en	Clark, Maria; Harris, Rebecca; Jolleff, Nicola; Price, Katie; Neville, Brian G. R.
Titel	Worster-Drought Syndrome: Poorly Recognized despite Severe and Persistent Difficulties with Feeding and Speech
Quelle	In: Developmental Medicine & Child Neurology, 52 (2010) 1, S.27-32 (6 Seiten) PDF als Volltext Verfügbarkeit
Sprache	englisch
Dokumenttyp	gedruckt; online; Zeitschriftenaufsatz
ISSN	0012-1622
DOI	10.1111/j.1469-8749.2009.03475.x
Schlagwörter	Attention Deficit Hyperactivity Disorder; Psychology; Cerebral Palsy; Criticism; Identification; Neurology; Brain; Profiles; Children; Speech Impairments; Augmentative and Alternative Communication; Epilepsy; Mental Retardation; Severity (of Disability); Clinical Diagnosis; Intervention; Interpersonal Communication + Suchen Sie Ihr Suchwort? Psychologie; Hirnlähmung; Kritik; Identifikation; Identifizierung; Neurologie; Gehirn; Charakterisierung; Profilanalyse; Child; Kind; Kinder; Speech impairment; Speech handicap; Speech handicaps; Language handicps; Language impairments; Sprachbehinderung; Epilepsie; Geistige Behinderung; Schweregrad; Interpersonale Kommunikation
Abstract	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. (As Provided).
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Erfasst von	ERIC (Education Resources Information Center), Washington, DC
Update	2017/4/10