Amyotrophic choreoacanthocytosis: is it really a very rare disease?

Amyotrophic choreoacanthocytosis (ACA) is a hereditary disease characterized by adult onset, hyperkinesias (with tongue and/or lip biting), neurogenic muscular atrophies, acanthocytosis and normal blood lipoprotein pattern. In this paper we cite 8 patients belonging to three families, describing 6 patients of two of the families. We query the supposed rarity of the disease.