DBN is also frequently found in patients with spinocerebellar ataxia type 6 (SCA6) as well as in episodic ataxia type 2 (EA2) [14], whereas DBN was not found in other genetic CA such as SCA1, SCA2, SCA3, and SCA 31 [15–17]. Here, ATXN3 is linked to spinocerebellar ataxia type 6.