Abstract
Publisher Summary Distal myopathies comprise a heterogeneous group of primary muscle disorders characterized by onset in the hands or feet and by myopathic changes in both the electromyography (EMG) and histopathologic examinations. The chapter discusses some major types of hereditary distal myopathies: Welander distal myopathy (WDM), tibial muscular dystrophy (TMD), Nonaka DM, Miyoshi DM, and Laing DM. All well-established forms of distal myopathy are genetic in origin. The mode of inheritance is known for all these myopathies, and each has been linked to a specific chromosome region. Histopathological findings in distal myopathies show dystrophic changes of variable severity. Rimmed vacuoles are seen frequently in WDM. In all distal myopathies, sensory nerve conduction velocities (SNCVs) and motor nerve conduction velocities (MNCVs) are normal as are the compound sensory nerve action potentials (SNAPs). The compound muscle action potential (CMAP) of distal muscles may be decreased in correspondence to the degree of atrophy of the muscle.
Published Version
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