Dandy-Walker syndrome involves a congenital brain malformation of the cerebellum and the fluid-filled spaces around it. Cerebellum is an area at the back of the brain that controls movement.
The cause for this syndrome is unknown in most cases while in others it is known to have resulted from autosomal recessive genes, meaning, getting an abnormal gene on one of the autosomal chromosomes from each parent.
Symptoms of Dandy-Walker Syndrome often occur in early infancy and can be as follows:
• slow motor development
• progressive enlargement of the skull.
In older children, symptoms of increased intracranial pressure include:
• signs of cerebellar dysfunction, such as unsteadiness, lack of muscle
co-ordination, or jerky movements of the eyes
• increased head circumference
• bulging at the back of the skull
• problems with the nerves that control the eyes, face and neck
• abnormal breathing patterns
• seizures (up to 15%).
The effect of this syndrome on intellectual development is variable, with some children having normal cognition and others never achieving normal intellectual development, even when the excess fluid build-up is treated both early and correctly. Life expectancy depends on the severity of the syndrome and associated malformations. The presence of multiple congenital defects may shorten a person’s life span.
The chance of survival of the patient is 75 percent to 100 percent. About half of patients have normal IQ. Difficulty with balance, stiffness and poor fine motor control is common. Seizures occur in 15 percent of patients.
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