Which malformation is characterized by cerebellar involvement and a high rate of intellectual disability?

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Dandy-Walker malformation is characterized by a specific developmental abnormality of the cerebellum and the fourth ventricle of the brain. This condition involves cystic enlargement of the fourth ventricle and an absence or hypoplasia of the cerebellar vermis, which connects the two hemispheres of the cerebellum. Patients with Dandy-Walker malformation often exhibit a range of neurological symptoms, including motor delays, and they have a higher prevalence of intellectual disabilities compared to other malformations. The presence of these associated features, particularly the cerebellar involvement and the correlated incidence of cognitive impairment, makes Dandy-Walker malformation a prominent condition in the context of developmental disorders.

In contrast, Holoprosencephaly is primarily characterized by incomplete separation of the cerebral hemispheres and can also lead to intellectual disability, but it is not primarily a malformation of the cerebellum. Chiari malformation involves the downward displacement of cerebellar tissue through the foramen magnum and can lead to a variety of symptoms, but its association with intellectual disability is less direct than in Dandy-Walker malformation. Spina bifida involves neural tube defects affecting the spinal cord and is associated with physical and functional impair

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