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Dandy-Walker Syndrome

What is Dandy-Walker syndrome?

Dandy-Walker syndrome is a condition that affects brain development, primarily development of the part of the brain that coordinates movement (the cerebellum). In individuals with this condition, various parts of the cerebellum develop abnormally, resulting in malformations that can be observed with medical imaging. The central part of the cerebellum, known as the vermis, is absent or very small. Cysts form in the fourth ventricle, which is a fluid-filled cavity between the brainstem and the cerebellum, causing the ventricle to enlarge. The part of the skull that contains the cerebellum and the brainstem, called the posterior fossa, is abnormally large. These abnormalities often result in problems with movement, coordination, intellect, and other neurological functions.

In 80 to 90 percent of individuals with Dandy-Walker syndrome, signs and symptoms caused by abnormal brain development appear within the first year of life. Most children have a buildup of fluid in the brain (hydrocephalus) that causes increased head size (macrocephaly). Affected individuals typically have intellectual disability that ranges from mild to severe, although some have a normal intellect. Children with Dandy-Walker syndrome often have delayed development, particularly a delay in motor skills such as crawling, walking, and coordinating movements. People with Dandy-Walker syndrome frequently experience muscle stiffness and paralysis of the lower limbs (spastic paraplegia), and they may also have seizures. Hearing and vision problems are sometimes a feature of this condition.

Less commonly, other brain malformations have been reported in people with Dandy-Walker syndrome. These malformations include a failure of the brain to divide properly into the right and left hemispheres (holoprosencephaly), underdeveloped or absent tissue connecting the left and right halves of the brain (agenesis of the corpus callosum), a sac-like protrusion of the brain through an opening at the back of the skull (occipital encephalocele), abnormal slits or clefts in the brain (schizencephaly), or a type of birth defect that occurs when the protective covering around the brain or spinal cord fails to close during development (neural tube defect). These additional brain malformations are associated with more severe signs and symptoms. It is estimated that 20 percent of individuals with Dandy-Walker syndrome survive past the first year of life. The most common causes of death in people with Dandy-Walker syndrome are problems related to hydrocephalus or complications of treatment.

Other signs and symptoms of Dandy-Walker syndrome can include heart defects, malformations of the urogenital tract, extra fingers or toes (polydactyly) or fused fingers or toes (syndactyly), or abnormal facial features.

In 10 to 20 percent of people with Dandy-Walker syndrome, signs and symptoms do not appear until late childhood or into adulthood. These individuals typically have a different range of signs and symptoms, including headaches, an unsteady walking gait, paralysis of facial muscles (facial palsy), increased muscle tone, muscle spasms, and mental and behavioral changes.

A milder form of Dandy-Walker syndrome, called Dandy-Walker variant, is associated with less severe brain abnormalities and, generally, less severe signs and symptoms. Rarely, people with brain malformations characteristic of Dandy-Walker syndrome have no symptoms.

How common is Dandy-Walker syndrome?

Dandy-Walker syndrome is estimated to affect 1 in 25,000 to 30,000 newborns.



PLEASE NOTE: This is by no means complete or authoritative and is provided only as a convenience to readers. The resources on this site should never be used as a substitute for professional medical care or advice. Information herein must not be used in any decision-making process regarding the wellness and/or health of your child. You must consult the appropriate professional regarding any such issues.

Narrative on several of these pages is presented as provided to the public domain by the National Institute of Neurological Disorders and Stroke and the National Institute of Health. For additional and more detailed information visit the NINDS Website.

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