The part of the brain located between the two cerebellar hemispheres called cerebral vermis becomes partially or completely absent. Other key features include an enlargement of the fourth ventricle (one of four connected cavities in the brain) and cyst formation near the internal base of the skull. The three features form a triad.
It is a genetically sporadic disorder. Dandy-Walker syndrome occurs one in every 25,000 live births. Females are mostly affected.
References:1. National Institute of Neurological Disorders and Stroke, "NINDS Dandy-Walker Syndrome Information Page
2. Harrison's Principles of Internal Medicine, 17th Ed.
3. Volpe, Joseph, J.Neurology of the Newborn, 4th edition.
Latest Publications and Research on Dandy Walker SyndromeDandy-Walker Syndrome with Giant Cell Lesions and Cherubism. - Published by PubMed
Fetal cerebellar disorders. - Published by PubMed
Ablative stereotactic neurosurgery for irreducible neuroaggressive disorder in pediatric patients. - Published by PubMed