Hereditary motor-sensitive neuropathy
| Classification according to ICD-10 | |
|---|---|
| G60.0 | Hereditary sensorimotor neuropathy - Charcot-Marie-Tooth-Hoffmann syndrome - Déjerine-Sottas disease - Peroneal muscular atrophy |
| G60.1 | Refsum disease |
| ICD-10 online (WHO version 2019) | |
Hereditary motor-sensitive neuropathies , abbreviated to HMSN , are a group of inherited and chronically progressing nerve diseases ( neuropathies ) that only affect the peripheral nerves and predominantly their motor component. The common characteristic is the weakness beginning distally (away from the trunk), gradually spreading centrally, with accompanying muscle wasting ( atrophy ). The old names with proper names are increasingly being abandoned in favor of the following classification (according to Dyck).
- HMSN I - Charcot-Marie-Tooth Disease
- HMSN II - Neuronal Peroneal Muscular Atrophy
- HMSN III - Déjerine Sottas Disease
- HMSN IV - Refsum Syndrome
- HMSN V - Hereditary Spastic Paraplegia
- HMSN VI - HMSN with optic atrophy
- HMSN VII - HMSN with retinitis pigmentosa
Individual evidence
- ^ PJ Dyck. P. Chance, R. Lebo, JA Carney: Hereditary motor and sensory neuropathies. In: PJ Dyck, PK Thomas, JW Griffin, P. A Low, JF Poduslo (Eds.): Peripheral Neuropathy. 3. Edition. Saunders, 1993, pp. 1094-1136.