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.
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.
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 excess fluid buildup 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.
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.
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 2640 East 28th Street Brooklyn, NY 11235 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 |
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Last updated September 16, 2008