Synofzik et al. [4] screened 434 European ataxia patients, demonstrating a frequency of SYNE1 mutation higher than previously thought (5.3% after exclusion of Friedreich’s ataxia and the most common repeat expansion SCAs), and that it commonly presents with a multisystemic neurodegenerative disease. The gene discussed is SYNE1; the disease is Friedreich ataxia.