<症例>末梢神経および筋肉に著しい変化を認めた原発性amyloidosisの一生検例

概要

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Non-familial amyloidneuropathy is known as the lower incidence than familial one. Pathomorphological reports on amyloidneuropathy have mostly referred to the sural nerve. very few concerned with muscle and the intramuscular nerve. We have studied thoroughly not only the sural nerve but also the intramuscular nerve which might contain motor nerve axons. We summarize the results as follows. 1) Changes of the sural nerve a. Deposition of amyloid around vascular wall b. Decrease in the number of both myelinated and unmyelinated nerve fibers (especially large nerve fibers) 2) Changes of the muscle a. Deposition of amyloid in the connective tissue and pericapillary region b. Especially grouping of small angular fibers which means one of neurogenic changes 3) Changes of the intramuscular nerve a. Degenerative changes with the appearance of the Schwann cell pocket in the intramuscular nerves b. Existence of Schwann cell processes instead of the axon terminal in the presynaptic cleft ("empty folds of basement membrane"). Although we could not find the exact cause of changes of the intramuscular nerve, we presume the same process as the sural nerve degeneration. In conclusion we confirmed that the changes of the motor nerve fibers may occur in non-familial amyloidneuropathy.
近畿大学の論文
1983-06-25