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Definition

An autosomal dominant, axonal form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies (designated CMT1 when they are dominantly inherited) and primary peripheral axonal neuropathies (CMT2). Neuropathies of the CMT2 group are characterized by signs of axonal degeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy.

Acronym

CMT2Z

Synonyms

Charcot-Marie-Tooth disease, axonal, autosomal dominant, type 2Z
Charcot-Marie-Tooth disease, axonal, type 2Z
Charcot-Marie-Tooth neuropathy, type 2Z

Keywords

Charcot-Marie-Tooth disease
Neurodegeneration