What is Dandy Walker Syndrome?

What is Dandy Walker Syndrome?

Publications. Definition. Dandy-Walker Syndrome is a congenital brain malformation involving the cerebellum (an area of the back of the brain that coordinates movement) and the fluid-filled spaces around it.

Can you live with Dandy Walker syndrome?

There are no two people in the world with Dandy-Walker that experience the same symptoms. Some people live their entire lives never knowing they have it. Babies die from unknown causes because they were born seemingly healthy at full term with never a suspicion that something was wrong.

What causes hydrocephalus in Dandy Walker syndrome?

About Dandy Walker Syndrome The Dandy-Walker syndrome is hydrocephalus associated with a posterior fossa cyst and abnormal development of the cerebellum, a portion of the brain located near the base of the skull and important to voluntary muscle movement, balance and posture.

How did Dandy Walker syndrome get its name?

The syndrome is named after physicians Walter Dandy and Arthur Walker, who described associated signs and symptoms of the syndrome in the 1900s. The malformations often develop during embryonic stages.

Is Dandy-Walker a disability?

Dandy-Walker malformation with intellectual disability, basal ganglia disease and seizures is a genetic disease, which means that it is caused by one or more genes not working correctly.

Is Dandy-Walker syndrome fatal?

Rarely, people with Dandy-Walker malformation have no health problems related to the condition. Problems related to hydrocephalus or complications of its treatment are the most common cause of death in people with Dandy-Walker malformation.

Is Dandy-Walker Syndrome considered a disability?

Some children have a buildup of fluid in the brain (hydrocephalus) that may cause increased head size (macrocephaly ). Up to half of affected individuals have intellectual disability that ranges from mild to severe, and those with normal intelligence may have learning disabilities.

Is Dandy-Walker malformation fatal?

Is Dandy Walker Syndrome considered a disability?

How common is Dandy-Walker?

The frequency of Dandy Walker malformation in the US is approximately 1 per 25,000 -35,000 live births and affects more females than males.

Is Dandy-Walker fatal?

Like any condition affecting the brain, a Dandy-Walker syndrome diagnosis can be scary. It’s important to remember that most people with Dandy-Walker syndrome can lead happy, healthy lives with the help of educational, occupational, and physical therapies.

What is the difference between Dandy-Walker malformation and variant?

Dandy-Walker variant (DWv) is a less severe posterior fossa anomaly than the classic Dandy-Walker malformation (DWM), characterized as partial vermian hypoplasia with partial obstruction to the fourth ventricle. It is considered on the lesser end of the disease spectrum in the Dandy-Walker continuum.

How is Dandy-Walker diagnosed?

Dandy-Walker complex can be diagnosed by imaging of the brain such as ultrasound, CT scan, or MRI scan.

Is Dandy-Walker a chromosomal abnormality?

Some people with Dandy-Walker have chromosomal conditions, such as extra or missing pieces of chromosomes. Chromosomes are packages of DNA that carry genes. Dandy-Walker syndrome may also happen as part of a genetic disorder that includes several birth defects (congenital conditions).

How common is Dandy-Walker malformation?

Is Dandy-Walker hereditary?

These genetic changes may cause Dandy-Walker complex alone, or they may cause Dandy-Walker complex as well as other health problems. In these situations, the complex can run in families. However, in most situations, Dandy-Walker complex is caused by a combination of genetic and environmental factors.