NINDS Dandy-Walker Syndrome Information Page: NINDS


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Article title: NINDS Dandy-Walker Syndrome Information Page: NINDS
Conditions: Dandy-Walker Syndrome
What is Dandy-Walker Syndrome?
Dandy-Walker syndrome is a congenital brain malformation involving the fourth ventricle and cerebellum. It is defined as an enlargement of the fourth ventricle, an absence (partial or complete) of the cerebellar vermis (the narrow middle area between the two cerebral hemispheres), and cyst formation in the posterior fossa (the internal base of the skull). Hydrocephalus (increased intracranial pressure) may also be present. Symptoms which often occur in early infancy include slow motor development and progressive macrocrania (an abnormally enlarged skull). In older children symptoms of increased intracranial pressure such as irritability, vomiting and convulsions, and/or signs of cerebellar dysfunction such as ataxia and nystagmus (jerky eyes) may occur. The syndrome can appear dramatically or be totally asymptomatic. Other symptoms include increased head circumference, bulging occiput (the back of the head), cranial nerve dysfunction, and abnormal breathing patterns. Of importance is the high association of Dandy-Walker syndrome with other central nervous system structural anomalies including agenesis of the corpus callosum and malformations of the face, limbs, digits and heart.

Is there any treatment?
Treatment for individuals with Dandy-Walker syndrome generally consists of treating the associated anomalies, if needed. Also, a ventriculoperitoneal shunt may be inserted to control the hydrocephalus. Genetic counselling may also be needed.

What is the prognosis?
The prognosis for patients with Dandy-Walker syndrome is only moderately favorable, even when the hydrocephalus is treated early and correctly. The presence of multiple congenital defects may adversely affect survival. Prognosis for normal intellectual development is variable depending on the severity of the syndrome and associated malformations.

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 how this process can go awry and, thus, offers hope for new means to treat and prevent developmental brain disorders, including Dandy-Walker syndrome.

Selected references

James, H.
Hydrocephalus in Infancy and Childhood. American Family Physician, 45:2; 733-742 (February 1992).

Castroviejo, I, Velez, A, Pascual-Pascual, S, Roche, M, and Villarejo, F.
Dandy-Walker Malformation: Analysis of 38 Cases. Child's Nervous System, 7:2; 88-97 (1991).

Bordarier, C, and Aicardi, J.
Dandy-Walker Syndrome and Agenesis of the Cerebellar Vermis: Diagnostic Problems and Genetic Counselling. Developmental Medicine and Child Neurology, 32; 285-294 (1990).

Russ, P, Pretorius, D, and Johnson, M.
Dandy-Walker Syndrome: A Review of Fifteen Cases Evaluated by Prenatal Sonography. American Journal of Obstetrics and Gynecology, 161:2; 401-406 (1989).

 Organizations

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
hydroassoc@aol.com
http://www.hydroassoc.org/
Tel: 415-732-7040 888-598-3789
Fax: 415-732-7044

Hydrocephalus Support Group, Inc.
P.O. Box 4236
Chesterfield, MO 63006-4236
dhydrobuff@aol.com
Tel: 636-532-8228

March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
resourcecenter@modimes.org
http://www.modimes.org/
Tel: 914-428-7100 888-MODIMES (663-4637)
Fax: 914-428-8203

National Organization for Rare Disorders (NORD)
P.O. Box 8923
(100 Route 37)
New Fairfield, CT 06812-8923
orphan@rarediseases.org
http://www.rarediseases.org/
Tel: 203-746-6518 800-999-NORD (6673)
Fax: 203-746-6481

This fact sheet is in the public domain. You may copy it.Provided by:
The National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892



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