Neuroimaging revealed cavitations in the basal ganglia area with brain atrophy, a mandatory differential diagnosis with CI deficiency by NADH:ubiquinone oxidoreductase core subunit S1 (NDUFS1) and NADH:ubiquinone oxidoreductase core subunit V1 (NDUFV1) mutations [34], as biotin responsive basal ganglia disorders (BBGDs) are treatable. The gene discussed is NDUFV1; the disease is basal ganglia disorder.