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Worster-Drought syndrome

Also known as: Congenital Suprabulbar Paresis

Background

Worster-Drought syndrome is a form of cerebral palsy. The main problems occur with the mouth, tongue and swallowing (bulbar) muscles being affected. There are usually no obvious causes in the pregnancy or birth but some varieties are genetically determined. Because of the range of problems, the diagnosis is often made quite late.

Credits

Last updated October 2013 by Professor Brian Neville, Emeritus Professor of Paediatric Neurology, UCL Institute of Child Health and Great Ormond Street Hospital, London, UK.

What are the symptoms?

Worster-Drought syndrome is a non-progressive condition. The signs and symptoms include difficulties with voluntary lip, tongue, and palate movements. Sometimes there are changes to the shape of the jaw and tooth alignment, so that dental care is important.

The first indication of the condition is usually when the baby has difficulties with feeding; those most severely affected have sucking difficulties and may require tube feeding for several months. Problems with chewing and swallowing can occur when solid foods are introduced, which may lead to inhalation of food. These difficulties may gradually improve over the first two to three years, but may also persist for many years. Dribbling is very common, but may show steady improvement during childhood. There is also severe speech delay (see entry Speech and Language Impairment). As a result of the swallowing problems, middle ear infections can be quite frequent, and may cause conductive hearing loss. A mild delay in walking and running with clumsiness of the hands with mild spasticity (stiffness) are common. Most children have mild or moderate learning and behaviour difficulties (see entry Learning Disability), which may include hyperactivity and autism spectrum disorder. In a significant minority, epilepsy can also occur.

What are the causes?

Worster-Drought syndrome is caused by damage to the perisylvian area of the brain, which controls the mouth and throat muscles. The damage appears to occur in weeks 12 to 16 of pregnancy, and may be due a result of problems with blood supply to the area, or genetic vulnerability.

How is it diagnosed?

Diagnosis of Worster-Drought syndrome is based on the findings of clinical examination by a neurologist.

How is it treated?

The management of Worster-Drought syndrome is very much dependent on the degree of feeding and speech problems. In some cases, speech therapy, medication and occasionally surgical submandibular gland diversion to treat dribbling are required. Where epilepsy is present, it is treated with medication in the usual way. It is very important that cognitive development being ahead of speech is recognised.

Although separately described as a syndrome of quite severe epilepsy, bilateral perisylvian syndrome (see entry Congenital Bilateral Perisylvian syndrome) overlaps almost totally with Worster-Drought syndrome. In this latter condition, magnetic resonance imaging (MRI) shows the characteristic abnormality called perisylvian polymicrogyria. All of the clinical problems are similar to those of the Worster-Drought syndrome with the exception that the rate of epilepsy is higher. There are families with one child with this MRI abnormality and another child with typical Worster-Drought findings and a normal MRI scan. There are some genetic clues now to perisylvian polymicrogyria which are being actively explored.

Inheritance patterns and prenatal diagnosis

Inheritance patterns 
Most cases are sporadic but there have been families with more than one affected member for which the inheritance is still unclear.

Prenatal diagnosis 
None.

Is there support?

Worster-Drought Syndrome Support Group

Email: national.contact@wdssg.org.uk
www.wdssg.org.uk

The Group is a Registered Charity in England and Wales No. 1095290. It provides information and support to families affected by Worster-Drought Syndrome or Congenital Bilateral Perisylvian Polymicrogyria. 

Group details last updated December 2014.

Talk to other families about your child's condition

Visit our online Worster-Drought syndrome group

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