Dandy-Walker Syndrome
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What is Dandy-Walker Syndrome?Is there any treatment?What is the prognosis?What research is being done?OrganizationsWhat is Dandy-Walker Syndrome?
Dandy-Walker Syndrome is a congenital brain malformation involving the cerebellum (an area at the back of the brain that controls
movement) and the fluid filled spaces around it. The key features of this syndrome are an enlargement of the fourth ventricle
(a small channel that allows fluid to flow freely between the upper and lower areas of the brain and spinal cord), a partial
or complete absence of the cerebellar vermis (the area between the two cerebellar hemispheres), and cyst formation near the internal base of the skull. An increase in
the size of the fluid spaces surrounding the brain as well as an increase in pressure may also be present. The syndrome can
appear dramatically or develop unnoticed. Symptoms, which often occur in early infancy, include slow motor development and
progressive enlargement of the skull. In older children, symptoms of increased intracranial pressure such as irritability,
vomiting, and convulsions, and signs of cerebellar dysfunction such as unsteadiness, lack of muscle coordination, or jerky
movements of the eyes may occur. Other symptoms include increased head circumference, bulging at the back of the skull, problems
with the nerves that control the eyes, face and neck, and abnormal breathing patterns. Dandy-Walker Syndrome is frequently
associated with disorders of other areas of the central nervous system including absence of the corpus callosum (the connecting area between the two cerebral hemispheres, and malformations of the heart, face, limbs, fingers and toes.
Is there any treatment?
Treatment for individuals with Dandy-Walker Syndrome generally consists of treating the associated problems, if needed. A
special tube to drain off excess fluid may be placed inside the skull. This will reduce intracranial pressure and help control
swelling. Parents of children with Dandy Walker Syndrome may benefit from genetic counseling if they intend to have more children.
What is the prognosis?
The effect of Dandy-Walker Syndrome on intellectual development is variable, with some children having normal cognition and
others never achieving normal intellectual development even when the hydrocephalus is treated early and correctly. Longevity
depends on the severity of the syndrome and associated malformations. The presence of multiple congenital defects may shorten
life span.
What research is being done?
The NINDS conducts and supports a wide range of studies that explore the complex mechanisms of normal brain development. The
knowledge gained from these fundamental studies provides the foundation for understanding abnormal brain development and offers
hope for new ways to treat and prevent developmental brain disorders such as Dandy-Walker Syndrome.
Select this link
to view a list of studies currently seeking patients.
Organizations
Prepared by:
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National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892
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on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined
that patient or is familiar with that patient's medical history.
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Last updated February 12, 2007
