While ATXN-2 (SCA2) and ATXN-1 (SCA1) PolyQ intermediate expansions have been previously reported to be independently associated with an increased risk for ALS [32–34], co-occurrence of ALS and SCA1 within a family carrying an intermediate ATXN-1 poly-Q expansion has been recently reported, reinforcing the putative pathogenic link between the two disorders [35]. The gene discussed is ATXN2; the disease is amyotrophic lateral sclerosis.