Recessive RNF170 mutations have recently been confirmed as a cause of HSP [14, 39]**, but a heterozygous mutation in RNF170 (p.Arg199Cys) was found to cause autosomal dominant late-onset progressive sensory ganglionopathy as a cerebellar ataxia, neuropathy, and vestibular areflexia syndrome (CANVAS) mimic [40]. This evidence concerns the gene RNF170 and cerebellar ataxia.