Chorea-Acanthocytosis (ChAc), one of the core Neuroacanthocytosis syndromes, is an adult-onset rare neurodegenerative disorder caused by alteration in gene VPS13A, one of the four members of the human gene family presenting similarity to yeast VPS13 gene; all of these genes have now been associated with recessive disorders. This evidence concerns the gene VPS13A and Choreoacanthocytosis.