Dandy-Walker syndrome
Dandy-Walker Syndrome (pronounced: dan-dee-wok-er sin-drome) is a congenital brain malformation involving the cerebellum and the fluid filled spaces around it. The etymology of the term is derived from the names of the two American physicians, Walter Dandy and Arthur Walker, who first described the syndrome in the 20th century.
Definition
Dandy-Walker Syndrome is a group of related conditions that affect the development and function of the cerebellum, an area at the back of the brain that plays a crucial role in coordinating movement. The syndrome is characterized by a triad of abnormalities: 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 cerebellar vermis (the area between the two cerebellar hemispheres), and cyst formation near the internal base of the skull.
Symptoms
The symptoms of Dandy-Walker Syndrome can include slow motor development, progressive enlargement of the skull, irritability, vomiting, unsteadiness, lack of muscle coordination, or jerky movements of the eyes. Symptoms can occur in early infancy but in some cases, they may not become noticeable until late childhood or adulthood.
Causes
The exact cause of Dandy-Walker Syndrome is unknown. However, it is believed to result from a genetic mutation or environmental factors that interfere with the normal development of the brain during the early stages of pregnancy.
Diagnosis
Diagnosis of Dandy-Walker Syndrome is usually made through prenatal ultrasound or after birth by using imaging techniques such as MRI or CT scan.
Treatment
There is no cure for Dandy-Walker Syndrome. Treatment is symptomatic and supportive, and may include physical therapy to help with movement problems, medication to control seizures, and in some cases, surgery to control fluid accumulation in the brain.
Prognosis
The prognosis for individuals with Dandy-Walker Syndrome varies. Some children have normal cognition and others may have intellectual disability. Longevity depends on the severity of the syndrome and associated malformations.
See also
External links
- Medical encyclopedia article on Dandy-Walker syndrome
- Wikipedia's article - Dandy-Walker syndrome
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