A missense mutation in RNF170 (c.595C>T, p.Arg199Cys), going back to a common founder in the Eastern Canadian population, has previously been reported to cause autosomal dominant sensory ataxia (ADSA, MIM #608984 [https://www.omim.org/entry/608984]). This evidence concerns the gene RNF170 and autosomal dominant sensory ataxia 1.