Table 4.
Classification of the distal hereditary motor neuropathies
| Type | Inheritance | Gene/locus | Specific phenotype |
| HMN I | AD | unknown | juvenile-onset dHMN |
| HMN II | AD | HSP27 (HSPB1) | Adult-onset typical dHMN/+/− minor sensory (CMT4F) |
| HMN II | AD | HSP22 (HSPB8) | Adult-onset typical dHMN/+/− minor sensory (CMT2L) |
| HMN III | AR | 11q13 | Early-onset, slowly progressive |
| HMN IV | AR | 11q13 | Juvenile-onset, diaphragmatic involvement |
| HMN V | AD | GARS | Upper limb onset, slowly progressive/CMT2D |
| HMN V | AD | BSCL2 | Upper limb onset, +/−spasticity lower limbs/Silver syndrome |
| HMN VI | AR | IGHMBP2 | Spinal muscle atrophy with respiratory distress (SMARD1), infantile-onset respiratory distress |
| HMN VIIA | AD | 2q14 | Adult-onset, vocal cord paralysis |
| HMN VIIB | AD | DCTN1 | Adult-onset/vocal cord paralysis/facial weakness |
| HMN/ALS4 | AD | SETX | Early-onset, pyramidal signs |
| HMN-J | AR | 9p21.1-p12 | Juvenile-onset, pyramidal features, Jerash |
| Congenital distal SMA | AD | 12q23-12q24 | Antenatal-onset, arthrogryposis |
HSP 27 = heat shock 27kDa protein 1; HSP 22 = heat shock 22kDa protein 8; GARS = glycyl-tRNA synthetase; BSCL2 = Berardinelli-Seip congenital lipodystrophy 2 (Seipin); IGHMBP2 = immunoglobulin mu binding protein 2; DCTN1 = dynactin1; SETX = sentaxin