The generator site in acquired autoimmune neuromyotonia

摘要: Abstract Objective To investigate the origin of ectopic activity in neuromyotonia (NMT). Methods We studied two patients. In addition to routine studies, we tested synchronicity spontaneous discharges different motor units simultaneous recordings made with needle electrodes first dorsal interosseus muscle. Time-locked fasciculations these double would represent abnormal initiated a nerve trunk ephaptic stimulation nearby axon. patient 1, this research protocol was applied once, 15 years after regular intravenous immunoglobulin (IvIg) treatment. Patient 2 investigated before and 1 year IvIg. Results Both patients improved IVIg, mirrored by striking decrease amount on electromyography. Moreover, our technique did not detect synchronous (time-locked fasciculations) second assessment, although predominant treatment 2. Conclusions NMT, originate both distal axonal branches more proximal segments. It appears that IvIg is effective blocking antibody segments, perhaps related factors such as blood-nerve barrier, temperature or differing ion channel distributions. Significance Treatment effects can shed light NMT.