Question
What is Worster-Drought Syndrome, and what are its clinical signs and symptoms? What are the causes of Worster-Drought Syndrome? What are the speech and hearing problems and other problems faced by the child with Worster-Drought Syndrome? What tests ca
Answer
Worster-Drought Syndrome (WDS) is a nonprogressive type of cerebral palsy originally described by Dr. Cecil Worster-Drought in 1956 which affects approximately 200,000 individuals in the United States. WDS is a syndrome of variable expression characterized by suprabulbar paresis, selective weakness and impairment of the tongue, lips, jaw, soft palate, and pharynx. Speech and swallowing are ostensibly affected. Males predominate 3:1.
More specifically, affected children have difficulty lateralizing and protruding their tongues, and lip movements are abnormal. An exaggerated jaw jerk may be present. Expressive language is significantly delayed in comparison with receptive language. Speech production is characteristically dysarthric: lingual and bilabial sounds are disordered and accompanied by hypernasality. In severe cases phonation is impaired. Nutritional intake and dysphagia are ongoing concerns. Persistent drooling may improve with oral-motor therapy, medications, or surgery. Middle ear infections, sometimes secretory otitis media, and conductive hearing loss can arise from swallowing problems which affect Eustachian tube function. Micrognathia and dental fusing occur occasionally.
Infants often present with sucking difficulties which result in choking and aspiration. Supplemental tube feeding is often necessary. Irritability and sleep difficulties may arise.
Most children experience additional complex impairments such as, mild pyramidal tetraplegia, learning difficulties, ADHD, neuropsychiatric problems, and in a minority of children, epilepsy. As the limbs are affected by mild spasticity and incoordination, motor milestones are achieved late, and fine motor problems are likely.
WDS is said to arise from a bilateral lesions of the corticobulbar tract. Cranial nerves VII, X, and XII are implicated. In a small number of cases WDS may be transmitted as an autosomal dominant inheritance trait.
There are no definitive diagnostic criteria. Instead, diagnosis is determined from history and examination. Mean age of diagnosis is 6 years.
Although the speech-language pathologist plays a major role on the multidisciplinary team, research detailing evidence based practices is lacking. The constellation of problems associated with this syndrome calls for a speech-language pathologist experienced in the treatment of dysphagia, dysarthria, augmentative/alternative communication, and language/learning disorders. Working knowledge of behavior management is advisable as well.
The Worster-Drought Syndrome Support Group formed in 1994 in the United Kingdom offers support to families and raises awareness among professions. For more information contact www.wdssg.org.uk.
Karen E. Crockett, Ph.D., CCC-SLP is a clinical speech-language pathologist who owns a private practice in Tampa, Florida. Areas of particular interest to her are processing disorders, pervasive developmental disorders, social language difficulties, reading disorders, and developmental delays in adults.