Hereditary motor and sensory neuropathy

Hereditary motor and sensory neuropathy
Onion bulb formations in a nerve biopsy in a case of HMSN type I
SpecialtyNeurology Edit this on Wikidata

Hereditary motor and sensory neuropathies (HMSN) is a name sometimes given to a group of different neuropathies which are all characterized by their impact upon both afferent and efferent neural communication. HMSN are characterised by atypical neural development and degradation of neural tissue. The two common forms of HMSN are either hypertrophic demyelinated nerves or complete atrophy of neural tissue. Hypertrophic condition causes neural stiffness and a demyelination of nerves in the peripheral nervous system, and atrophy causes the breakdown of axons and neural cell bodies.[1] In these disorders, a patient experiences progressive muscle atrophy and sensory neuropathy of the extremities.[2]

The term "hereditary motor and sensory neuropathy" was used mostly historically to denote the more common forms Charcot–Marie–Tooth disease (CMT). With the identification of a wide number of genetically and phenotypically distinct forms of CMT, the term HMSN is now used less frequently.

  1. ^ Charcot-Marie-Tooth and Other Hereditary Motor and Sensory Neuropathies at eMedicine
  2. ^ Vance, Jeffery M. (1991). "Hereditary motor and sensory neuropathies". Journal of Medical Genetics. 28 (1): 1–5. doi:10.1136/jmg.28.1.1. PMC 1016739. PMID 1999826.