PSN Issue
WHAT IS DANDY-WALKER SYNDROME?
Dandy-Walker Syndrome is a congenital birth defect where a part of the
back of the brain called the vermis is underdeveloped or missing. It is
named after neurosurgeons Arthur E. Walker (1907–1995) and Walter E. Dandy (1883–1941), who first described the disorder in 1914.
Diagnosing Dandy-Walker Syndrome:
Dandy-Walker Syndrome may be diagnosed via computerized tomography (CT) or magnetic resonance imaging (MRI) or in utero via ultrasonography, provided that appropriate prenatal care is available and received. The
Metropolitan Atlanta Congenital Defects Program, Centers for Disease
Control and Prevention reports that Dandy-Walker Syndrome is occurring
in at least 1 in every 5000 live born infants (Molecular Genetics and
Metabolism, vol 80, 2003, pp 36-54, Parisi et al., “Human malformations of
the midbrain and hindbrain: review and proposed classification scheme”)
with as many as 40,000 cases in the United States.
Most Common Characteristics
Individuals with Dandy-Walker many times present with developmental delay, lethargy, vomiting, enlarged head circumference, or signs and symptoms of a condition called hydrocephalus. Approximately 70 to 90 percent of individuals with Dandy-Walker have or will develop hydrocephalus. Seizures will manifest in somewhere between 15 and 30 percent of patients with Dandy-Walker. It affects males and females equally.
Treating Dandy-Walker Syndrome
The treatments for individuals with Dandy-Walker Syndrome generally
consist of treating the associated problems versus the syndrome itself.
The long term prognosis is variable depending on the degree of other
abnormalities with cognitive abilities ranging from normal to varying
degrees of intellectual disability. Individuals with specific questions
or concerns about Dandy-Walker Syndrome should consult a neurosurgeon.
The Dandy-Walker Alliance is a 501(c)(3) non-profit corporation committed
to educational and informational activities, programs and publications and
supporting non-partisan research and events to increase public awareness
for Dandy-Walker Syndrome. We support all efforts to determine the
cause(s) of, find the cure for, and to ameliorate the effects of
Dandy-Walker Syndrome.
The Dandy-Walker Alliance has testified before the United States Congress and state legislatures. They have worked with lawmakers to pass national and state legislation, participated in the National Institutes of Health 2008 Nonprofit Forum to discuss prioritization of research needs with the National Institute of Neurological Disorders and Stroke. In addition, they have met with President George W. Bush in the Oval Office to discuss intellectual disability needs, participated in a Dandy-Walker documentary, provided free educational material in 15 languages and receive up to 90,000 visits a month to their website from all 50 states and 73 countries.
For more information please visit www.dandy-walker.org
Disclaimer: All information on this site is of a general nature and is furnished for knowledge and understanding only.