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 lowest part 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 and vomiting, 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 surgical procedure called a shunt may be required to drain off excess fluid within the brain. 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. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?CMD=search&term=dandy+walker+syndrome+AND+human[mh]+AND+english[la]&db=PubMed&orig_db=PubMed&filters=on&pmfilter_EDatLimit=5+Years 1 http://clinicaltrials.gov/ct2/results?term= /disorders/dandywalker/pubs_dandywalker.htm /disorders/dandywalker/xml_dandywalker.xml xml_dandywalker.xml V877 Dandy-Walker Alliance, Inc.

10325 Kensington Pkwy.

Suite 384 Kensington MD 20895 comments@dandy-walker.org http://www.dandy-walker.org 877-Dandy-Walker (326-3992) Nonprofit organization committed to educational and informational activities, programs and publications, and supporting non-partisan research and events to increase public awareness for Dandy-Walker. Supports all efforts to determine the cause(s) of, find the cure for and to ameliorate the effects of Dandy-Walker. V147 Hydrocephalus Association

4340 East West Highway

Suite 905 Bethesda MD 20814 info@hydroassoc.org http://www.hydroassoc.org 301-202-3811 888-598-3789 301-202-3813 Non-profit association that provides support, education, and advocacy programs for families and professionals. V41 March of Dimes

1275 Mamaroneck Avenue

White Plains NY 10605 askus@marchofdimes.com http://www.marchofdimes.com 914-997-4488 888-MODIMES (663-4637) 914-428-8203 Works to improve the health of babies by preventing birth defects and infant mortality through programs of research, community services, education, and advocacy. V707 National Hydrocephalus Foundation

12413 Centralia Road

Lakewood CA 90715-1653 debbifields@nhfonline.org http://nhfonline.org 562-924-6666 888-857-3434 Non-profit organization helping the unborn to the elderly. Provides educational material and information, physician referrals (based on patients & family), a communication network, support groups and advocacy. V91 National Organization for Rare Disorders (NORD)

55 Kenosia Avenue

Danbury CT 06810 orphan@rarediseases.org http://www.rarediseases.org 203-744-0100 Voice Mail 800-999-NORD (6673) 203-798-2291 Federation of voluntary health organizations dedicated to helping people with rare "orphan" diseases and assisting the organizations that serve them. Committed to the identification, treatment, and cure of rare disorders through programs of education, advocacy, research, and service. V1011 Pediatric Hydrocephalus Foundation

2004 Green Hollow Drive

Iselin NJ 08830 info@hydrocephaluskids.org http://www.HydrocephalusKids.org 732-634-1283 National all-volunteer organization dedicated to funding a cure for hydrocephalus, educating and raising awareness about the condition and offering support to the hydrocephalus community.