HSAN type | Age onset | Mutation | Onset symptom | Insensitivity to pain | Mental retardation | Temperature sensation | Hidrosis | Autonomic symptoms | Fractures | Multiple infection or ulcer | Auto amputation | Charcot joints | Nerve biopsy | CTS | Reference |
HSAN I | Adult | SPTLC1 | U/SP | Y | N | ↓ | N | Y/N | N | Y | Y | Y | Aα ↓ Aδ ↓↓ C ↓↓ | ? | Bejaoui,20 Houlden21 |
HSAN II | Childhood | HSNII | SP | Y | N | ↓ | ↓ | Y/N | N | Y | Y | Y | Aα N Aδ ↓↓ C N | ? | Lafreniere,22 Auer-Grumback23 |
HSAN III | Congenital | IKBKAP | A | Y | Y/N | ↓ | ↓ | Y | N | Y/N | N | Y/N | Aα ↓↓ Aδ N C ↓↓ | ? | Auer-Grumback,23 Slaugenhaupt24 |
HSAN IV TRKA | Congenital | TRKA | A/F | Y | Y | ↓ | ↓↓ | Y | Y | Y | Y | Y | Aα N Aδ ↓↓ C ↓ | N | Mardy,6 Shatzky25 |
HSAN V NGFB | Childhood | NGFB | AR/F | Y | N | ↓/N | N | Y/N | Y | Y | N | Y | Aα N Aδ ↓ C ↓↓ | N | Einarsdottir,8 Minde9 |
NGFB heterozygous | Adult | NGFB | AR | N | N | ↓/N | N | Y/N | N | N | N | Y | Aα N Aδ ↓ C ↓ | Y |
A, autonomic symptom; AR, arthropathy; CTS, carpal tunnel syndrome; F, fractures; HSAN, hereditary sensory and autonomic neuropathies; N, no or normal; SP, sensory neuropathy; U, ulcus skin; Y, yes.