Unfortunately, Dandy-Walker Syndrome (DWS) belies its sprightly sounding name. In actuality, it is a malformation in the cerebellum and the area around it.
The cerebellum is located in the back part of the brain. It controls movement and cognition (ability to think).
Dandy-Walker Syndrome affects the fourth ventricle which is a channel for fluid to move between certain areas of the brain and the spinal cord.
The space for the fluid around the brain is abnormally large and pressure within from an overabundance of cerebrospinal fluid is the result.
The corpus callosum which connects the cerebral hemispheres, and the cerebellar vermis which lies between the hemispheres of the cerebellum, may be partially or totally missing.
Dandy-Walker Syndrome has its origins during brain development before the baby is born. Approximately 1 in 25,000 babies will have DWS.
The disorder can develop slowly or can appear suddenly. Symptoms generally will begin to show up in infancy or childhood.
A variety of malfunctions may emerge which affect the central nervous system.
As the circumference of the skull increases the back of the skull may bulge out. As the child becomes older, and their skull is under greater pressure from within, they may experience convulsions and vomiting, deteriorating muscle coordination, and unsteadiness.
Motor development is slow. Balance will become shaky and fine motor control will decrease.
Hearing and vision may be negatively affected, with eye movements becoming jerky and poorly controlled. Breathing problems can appear.
Heart malformations and problems with the kidneys and urinary tract may occur. Deformities of the face, such as a cleft lip, and abnormalities in the arms and legs are not uncommon. The individual may have extra fingers or toes.
Over half of all children with DWS will have diminished intellectual development to the point of mental retardation, while others will develop normal cognitive abilities.
There is no cure for Dandy-Walker Syndrome at the present time. But some treatments are available to help handle some of its symptoms. For example, a shunt (tube to drain off excess fluid) when inserted into the skull will reduce the internal pressure and swelling.
Life expectancy will depend in large part on the severity of symptoms and accompanying defects.
Some of those with DWS will never develop any of these disabling symptoms, their only indicator then being the abnormally large size of the head. These individuals will be able to go on to live normal lives.
Resources:
Dandy-Walker Malformation
http://emedicine.medscape.com/article/408059-overview
Dandy-Walker Syndrome
http://www.doctorsofusc.com/condition/document/230528
What Is DWS?
http://www.dandywalker.net/index.php?option=com_content&task=view&id=2&Itemid=1
Dandy walker malformation
http://www.medcyclopaedia.com/library/topics/volume_vii/d/DANDY_WALKER_MALFORMATION.aspx
NINDS Dandy-Walker Syndrome Information Page
http://www.ninds.nih.gov/disorders/dandywalker/dandywalker.htm
Search Medica: Dandy-Walker Syndrome
http://www.searchmedica.com/search.html?q=Dandy-Walker%20Syndrome
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