NINDS Dandy-Walker Syndrome Information Page

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What 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 area of the brain between the two cerebellar hemispheres (cerebellar vermis), 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 area made up of nerve fibers connecting the two cerebral hemispheres (corpus callosum) and malformations of the heart, face, limbs, fingers and toes.

Is there any treatment?

What is the prognosis?

What research is being done?

NIH Patient Recruitment for Dandy-Walker Syndrome Clinical Trials

• At NIH Clinical Center
• Throughout the U.S. and Worldwide

Organizations

Dandy-Walker Alliance, Inc. 4422 Clearbrook Lane Kensington, MD   20895 submission@dandy-walker.org http://www.dandy-walker.org Tel: 301-919-2653	Guardians of Hydrocephalus Research Foundation 2618 Avenue Z Brooklyn, NY   11235-2023 GHRF2618@aol.com http://ghrf.Homestead.com/ghrf.html Tel: 718-743-GHRF (4473) Fax: 718-743-1171
Hydrocephalus Association 870 Market Street Suite 705 San Francisco, CA   94102 info@hydroassoc.org http://www.hydroassoc.org Tel: 415-732-7040 888-598-3789 Fax: 415-732-7044	March of Dimes Foundation 1275 Mamaroneck Avenue White Plains, NY   10605 askus@marchofdimes.com http://www.marchofdimes.com Tel: 914-428-7100 888-MODIMES (663-4637) Fax: 914-428-8203
National Hydrocephalus Foundation 12413 Centralia Road Lakewood, CA   90715-1623 debbifields@nhfonline.org http://nhfonline.org Tel: 562-924-6666 888-857-3434 Fax: 562-924-6666	National Organization for Rare Disorders (NORD) P.O. Box 1968 (55 Kenosia Avenue) Danbury, CT   06813-1968 orphan@rarediseases.org http://www.rarediseases.org Tel: 203-744-0100 Voice Mail 800-999-NORD (6673) Fax: 203-798-2291

Prepared by:
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
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Last updated September 16, 2008