It is now increasingly acknowledged that spectrin repeat-containing nuclear envelope protein (SYNE1) ataxia and autosomal-recessive spastic ataxia of Charlevoix-Saguenay (ARSACS; gene: SACS), which were long thought to be largely confined to a specific group of French-Canadian populations, are relatively frequent ARCAs distributed around the world [2, 7]. Here, SYNE1 is linked to Ataxia.