COQ7 and neuronopathy, distal hereditary motor, autosomal recessive: Biallelic pathogenic variants of SIGMAR1 (HGNC:8157), encoding a transmembrane ER protein involved in ER-mitochondrial signalling, have recently been associated with autosomal recessive dHMN and Silver-like syndrome in several families.21-23 The phenotype associated with SIGMAR1, and one of the most frequent HSP subtypes, SPG11 (HGNC:11226), can also mimic juvenile amyotrophic lateral sclerosis and might be misdiagnosed as such.21,24 Furthermore, biallelic COQ7 (HGNC:2244) variants were also recently described to cause dHMN with and without pyramidal signs.25