The second most common ARCA diagnosed was the ataxia with isolated vitamin E deficiency (AVED), with 19 patients (16 families) genetically confirmed: a unique mutation (c.744delA) in the TTPA gene was found at a homozygous state in all the patients, which further confirms its high frequency in the Mediterranen basin [22] and its strong founder effect as suggested by Ouahchi et al. [12] and supported by the recent report of El Euch-Fayache et al., 2014 [23] or our personal data (unpublished results). The gene discussed is TTPA; the disease is Ataxia.