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Introduction

Hereditary Motor Sensory Neuropathy (HMSN) is a name given to a group of different neuropathies that are all characterized by their implications on both the afferent and efferent neural communication. HMSNs are characterized by atypical neural development and degradation of neural tissue. The two common forms of HMSNs 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, whereas the complete atrophy of neural tissues causes the breakdown of axons and neural cell bodies. Patients affected with this pathophysiological condition experience progressive muscle atrophy and sensory neuropathy of the extremities. Originally, the term “hereditary motor and sensory neuropathy” was historically used to denote the more common form called Charcot-Marie-Tooth (CMT) Disease. With the identification of a wide number of genetically and phenotypically distinct forms of CMT, the term HMSN is now used to describe a larger number of medical conditions (see Table H-3).

TABLE H-3Hereditary Motor Sensory Neuropathies (HMSN): An Overview

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