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Myasthenia in patients with dermato myositis clinical electro physiological and ultrastructural studies

, : Myasthenia in patients with dermato myositis clinical electro physiological and ultrastructural studies. Journal of the Neurological Sciences 38(2): 129-144

In 4 patients with clinical signs of dermatomyositis, confirmed by electromyography and muscle biopsy, a form of muscle fatigue was detected which was expressed clinically by predominantly proximal motor deficit, wity phonation and deglutition disturbances, slightly influenced by prostigmine. In all patients, stimulation of the ulnar nerve at 3-10 Hz induced a decrement of muscle-evoked potentials in abductor digiti minimi and at 15-50 Hz and increment at the end of the trains (1.2 s in duration) of repetitive stimulation (preceded in 2 cases by a decrement in the response to the 5th stimulus in the train). Stimulation at 30 Hz for 10 s resulted in a transient facilitaiton, followed (at 3 Hz stimulation) by postactivation exhaustion which disappeared after 5-15 min. The post-tetanic facilitation, the incremental response and the myasthenic symptoms reverted to normal under treatment with corticosteroids, an immunosuppressor agent and guanidine hydrochloride. A mixed, pre- and postsynaptic mechanism may underlie the muscle fatigue in these patients. EM of muscle biopsies disclosed zones of necrosis and, in incipient stages, large agglomerations of glycogen that disorganized the structure of myofibrils. The end-plates in the biopsies were larger than normal and the cholinesterase reaction was hyperactive. Serum immunoelectrophoretic and electrophoretic data.sbd.increase of Ig[immunoglobulin]G and IgM, decrease of IgA and hypergammaglobulinemia.sbd.point to a possible autoimmune mechanism of the neuromuscular disorders in these patients.

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