The National Institute of Neurological Disorders and Stroke (NINDS) defines Dandy-Walker syndrome as a congenital brain condition that affect the cerebellum and ventricles. Ventricles are the fluid-filled areas of the brain that contains cerebrospinal fluid, or CSF. The cerebellum is towards the back of the brain, near the base and has an outer covering of gray matter; the University of Idaho states that the cerebellum is responsible for voluntary movements and learned motor behaviors.
Dandy-Walker syndrome results in abnormal brain formation. The NINDS states that the patient has an enlarged fourth ventricle, partial or complete lack of a cerebellar vermis (the area between the left and right hemsphere and a cyst close to the internal base of the skull. Due to the larger size of the ventricle, the patient has increased cranial pressure.
The symptoms of Dandy-Walker syndrome start as early as infancy, where the child has a slowed development of motor skills and a larger skull. As the child gets older, symptoms of intracranial pressure and cerebellar dysfunction start to show; the NINDS lists convulsions, vomiting, and irritability as symptoms of intracranial pressure; and jerky eye movements, unsteadiness, and lack of muscle coordination as symptoms of cerebellar dysfunction. Other symptoms of Dandy-Walker syndrome include abnormal breathing, nerve problems in the eye, face and neck, increased head circumference and bulging at the back of the head.
The NINDS adds that Dandy-Walker syndrome is often associated with other neurological disorders. Golden, Rorke and Bruce reviewed 28 Dandy-Walker syndrome, with 16 postmortem examinations and 12 clinical examinations. In the postmortem examinations, they found 11 patients had hydrocephalus; ten of them had other central nervous system malformations, and six had visceral anomalies.
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