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Updated: Aug 17, 2026

In Vivo Electrophysiological Measurement of Compound Muscle Action Potential from the Forelimbs in Mouse Models of Motor Neuron Degeneration
Published on: June 15, 2018
Pathology of peroneal muscular atrophy (Charcot-Marie-Tooth disease)
Abstract:
Four cases of peroneal muscular atrophy (Charcot-Marie-Tooth disease) are described in which observations were made from the necropsy examination of the central nervous system, the peripheral nervous system, and the skeletal muscles. The pattern of nerve degeneration in these various locations is described and the conclusion is drawn that the basis of the disease is a very chronic progressive neuropathy. To the pathological descriptions of previous observers is added the finding of fibrosis affecting the muscle spindles which was present in all four of the cases. The literature of the case reports with necropsy examination is reviewed.
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