Spinocerebellar ataxia types 19 (SCA19) and 22 (SCA22) are rare allelic channelopathies; linkage to chromosome region 1p21-q21 was reported first in two large Dutch and Chinese families [4, 5]; and the mutations in KCND3, which encodes the Shal-related voltage-gated potassium channel Kv4.3, associated with ataxia were discovered in 2012 by two independent research groups [6–8]. The gene discussed is KCND3; the disease is channelopathy.