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[Terminal polineuropathy (independent clinical syndrome or variant course of myasthenia)]
Abstract:
The clinical data and findings of thorough electrophysiological and morphohistochemical examinations of the muscles of 13 patients with the myasthenic syndrome associated with terminal polyneuropathy are presented. Correlation of the clinical and electrophysiological data to the clinical manifestations of the disease and the magnitude of the block of the neuromuscular transmission disclosed a number of characteristic features of the clinical course of the disease (a predominantly oculodistal type of damage, a diminution or the absence of tendon and periosteal reflexes, the presence of atrophies of distal muscular groups) and of the EMG pattern (the presence of enlarged PDDE, an increase in residual latency, the inverse ratio of the decrement of electric and mechanical responses upon indirect stimulation of the muscle at a frequency of 3/sec) and in the histochemical picture (the presence of groupings of type I and II muscular fibrils, which are not characteristic of myasthenia). On the basis of the evidence obtained, a conclusion is drawn that the described syndrome is an independent clinical form rather than a myasthenia variant.