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Worster-Drought Syndrome

Last updated May 2, 2018

Approved by: Maulik P. Purohit MD, MPH

Worster-Drought Syndrome (WDS) is a condition that affects the muscles of the mouth and throat; it is considered a type of cerebral palsy.


What are the other Names for this Condition? (Also known as/Synonyms)

  • Congenital Suprabulbar Paresis
  • WDS (Worster-Drought Syndrome)

What is Worster-Drought Syndrome? (Definition/Background Information)

  • Worster-Drought Syndrome (WDS) is a condition that affects the muscles of the mouth and throat; it is considered a type of cerebral palsy
  • The main symptoms of WDS include difficulties with drooling, swallowing and speaking
  • Some affected individuals may also have challenges with intelligence, behavior, or seizure
  • The cause of WDS is currently unknown, though it is believed that both genetic and environmental factors influence the development of this condition

(Source: Worster Drought Syndrome; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Who gets Worster-Drought Syndrome? (Age and Sex Distribution)

  • Worster Drought Syndrome is a rare congenital disorder, occurring at an estimated prevalence of 1:30,000
  • The onset of symptoms may occur in childhood; the average age of diagnosis is around 6 years
  • Although both genders may be affected, WDS affects males more frequently than females (in a 3:1 ratio)
  • Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Worster-Drought Syndrome? (Predisposing Factors)

  • A positive family history may be an important risk factor, since Worster Drought Syndrome can be inherited
  • Currently, no other risk factors have been clearly identified for the syndrome

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Worster-Drought Syndrome? (Etiology)

  • The exact cause of Worster Drought Syndrome is not known at the present time
  • Research scientists believe that the disorder may develop from both genetic and environmental factors that are currently unknown
  • The inherited form of Worster Drought Syndrome is transmitted in an autosomal dominant manner with variable penetrance and expressivity

Autosomal dominant: Autosomal dominant conditions are traits or disorders that are present when only one copy of the mutation is inherited on a non-sex chromosome. In these types of conditions, the individual has one normal copy and one mutant copy of the gene. The abnormal gene dominates, masking the effects of the correctly function gene. If an individual has an autosomal dominant condition, the chance of passing the abnormal gene on to their offspring is 50%. Children, who do not inherit the abnormal gene, will not develop the condition or pass it on to their offspring.

When an autosomal dominant trait has variable expressivity and penetrance, it indicates that the onset of disease symptoms in an affected individual is not determined by the mutated gene alone, but is influenced by environmental and lifestyle choices as well.

What are the Signs and Symptoms of Worster-Drought Syndrome?

The signs and symptoms of Worster-Drought Syndrome may include:

  • Drooling
  • Dysarthria
  • EEG abnormality

Very frequently present symptoms in 80-99% of the cases:

  • Abnormality of the cranial nerves
  • Neurological speech impairment

Frequently present symptoms in 30-79% of the cases:

  • Cognitive impairment
  • Hyperreflexia

Occasionally present symptoms in 5-29% of the cases:

  • Microcephaly
  • Seizures
  • Tetraplegia
  • Sensorineural hearing impairment

(Source: Worster Drought Syndrome; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

How is Worster-Drought Syndrome Diagnosed?

Worster-Drought Syndrome is diagnosed on the basis of the following information:

  • Complete physical examination
  • Thorough medical history evaluation
  • Assessment of signs and symptoms
  • Laboratory tests
  • Imaging studies
  • Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Worster-Drought Syndrome?

The potential complications of Worster-Drought Syndrome may include:

  • Feeding difficulties
  • Learning disabilities; speech impediment
  • Recurrent infections in airways and lungs
  • Behavioral problems
  • Risk of falls and injury due to seizures

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Worster-Drought Syndrome Treated?

  • The treatment for Worster Drought Syndrome is geared toward managing the signs and symptoms of the condition and preventing complications
  • Speech therapy and special education may be necessary for the affected child
  • Anti-epileptic drugs may be prescribed to control seizures

How can Worster-Drought Syndrome be Prevented?

Worster Drought Syndrome may not be preventable, if the condition runs in the family.

  • If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
  • Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders
  • Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Worster-Drought Syndrome? (Outcomes/Resolutions)

  • The prognosis of Worster Drought Syndrome is dependent upon the severity of the signs and symptoms and associated complications, if any
  • Individuals with mild conditions have better prognosis than those with severe symptoms and complications
  • Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Worster-Drought Syndrome:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/

What are some Useful Resources for Additional Information?


References and Information Sources used for the Article:


Helpful Peer-Reviewed Medical Articles:


Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: May 2, 2018
Last updated: May 2, 2018