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peroneal muscular atrophy n.
A hereditary form of muscular atrophy characterized by progressive wasting of the distal muscles of the extremities, usually affecting the legs before the arms. Also called Charcot-Marie-Tooth disease.
a group of inherited nerve diseases characterized by slowly progressive weakness and wasting of the muscles of the lower parts of the extremities. In Charcot-Marie-Tooth (CMT), the myelin sheath that surrounds motor and sensory nerves gradually deteriorates, blocking the conduction of nerve impulses to the muscles. Onset usually occurs in childhood or in adolescence, with the early-childhood form of the disease being the most rapidly progressive and disabling. CMT manifests with weakness of the leg muscles below the knee (stork leg); this usually results in a foot drop (i.e., the inability to hold the foot in a horizontal position), which causes a disturbance in gait. It occasionally affects the hands. Although the peripheral nerves may be enlarged in some persons with CMT, sensory loss is usually absent or minimal. Bone abnormalities such as curvature of the spine, high arches of the feet (pes cavus), and hammertoes are common. Longevity is not affected, though the disease is progressive. CMT is named for the French neurologists Jean-Martin Charcot and Pierre Marie and the English physician Howard Henry Tooth, who first described the disease in the late 19th century.