The cerebellum arises from two anatomically and molecularly different proliferative compartments: the cerebellar ventricular zone and the rhombic lip. The protracted development makes the cerebellum vulnerable to a broad spectrum of developmental disorders, of which the more frequent (the Dandy-Walker and related malformations and the pontocerebellar hypoplasias) are discussed in this article. Several genes for congenital malformations of the human cerebellum have recently been identified, including genes causing Joubert syndrome, the Dandy-Walker malformation, and pontocerebellar hypoplasias.
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