RFC1-positive patients showed more often a positive Romberg sign (85% vs 60%, POR 3.95 CI 95% 1.47–10.6, P = 0.004) associated with sensory ataxia (τb = 0.254, P < 0.001) and relatively spared DTR (median total score 18.0/24 vs 11.5/24, R = 0.275, P = 0.001) compared to non-mutated, even though clinical examination did not disclose a relevant difference in tested sensory modalities (median SMSS 28.5/40 vs 30.0/40, R = − 0.054, P = 0.80). The gene discussed is RFC1; the disease is sensory ataxia.