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Alberto Rivero Luis Crovetto Lidia Lopez Ricardo Maselli Martín Nogus 《Muscle & nerve》1995,18(9):943-947
We performed single fiber electromyography (SFEMG) in the superior rectus and levator palpebralis (SR-LP) muscles of 17 patients with pure ocular myasthenia gravis (MG) and 9 controls. Thirteen patients were also assessed with SFEMG in the orbicularis oculi (OO) muscle. All the MG patients but none of the control subjects showed abnormal SFEMG jitter in the SR-LP muscles. On the other hand, only 62% of the MG patients had abnormal SFEMG jitter in the OO muscle. The procedure was well tolerated by the patients, and complications were minor. We conclude that SFEMG of the SR–LP muscles is a safe and highly sensitive technique for the diagnosis of ocular MG. © 1995 John Wiley & Sons, Inc. 相似文献
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To define the underlying mechanism of neuromuscular transmission failure induced by anticholinesterases, we simultaneously performed surface recordings of compound muscle action potentials (CMAPs) and intracellular recordings of miniature end-plate potentials (MEPPs), miniature end-plate current (MEPCs), and end-plate potential (EPPs) in rat diaphragms exposed in vitro to 1 × 10?4 to 2 × 10?2 mmol/L neostigmine methylsulfate. At low concentrations of neostigmine, repetitive stimulation of the phrenic nerve resulted in decrement followed by complete recuperation of CMAP amplitudes. This bimodal pattern was associated with maximal end-plate depolarization at the beginning of the stimulation period, increased MEPP amplitudes, and prolonged time constants of MEPC decays. Higher concentrations of neostigmine resulted in a unimodal decline of amplitudes of CMAPs and EPPS, reduced MEPP amplitudes, and a double exponential time course of MEPC decays. These results indicate that low concentrations of anticholinesterases impaired neuromuscular transmission by producing transient depolarization of the end-plate region. Higher concentrations induced desensitization and direct blockade of the end-plate receptor channel, probably in its open conformation. © 1993 John Wiley & Soncs, Inc. 相似文献
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Adverse reaction to intravenous gadoteridol 总被引:1,自引:0,他引:1
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J S Appelbaum R P Roos E F Salazar-Grueso A Buchman S Iannaccone R Glantz T Siddique R Maselli 《Neurology》1992,42(8):1488-1492
Although there are varied inheritance patterns in motor neuron disease (MND), the phenotype of MND is reported to be constant within these families, ie, cases of amyotrophic lateral sclerosis or primary lateral sclerosis do not occur in pedigrees with cases of spinal muscular atrophy. We describe four pedigrees whose members diverged in the phenotype of MND expressed. The intrafamilial variation of phenotype suggests a similar pathogenesis for some of the varied types of familial MND and the need for careful inquiry of family history in all patients with MND. 相似文献
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Neuromuscular transmission as a function of motor unit size in patients with prior poliomyelitis. 总被引:2,自引:0,他引:2
We studied neuromuscular transmission in 16 patients with prior poliomyelitis by measuring single fiber electromyographic (SFEMG) jitter. This was compared with 3 indirect methods of assessing reinnervation: SFEMG fiber density, macro EMG, and the presence of fiber type grouping on muscle biopsy. In patients with acute poliomyelitis before the age of 10, there was a positive correlation between the extent of neuromuscular transmission impairment, demonstrated by increased SFEMG jitter, and the enlargement of the motor unit, as indicated by increased fiber density, increased macro EMG signals, and fiber type grouping on muscle biopsy. However, there was no correlation between any of these parameters and the presence or absence of new symptoms of weakness. These findings suggest that impaired neuromuscular transmission is most common in patients with prior poliomyelitis whose motor units have been maximally enlarged by axonal sprouting, but is independent of the presence or absence of new symptoms of weakness. 相似文献
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Sinotubular junction reconstruction in reimplantation type of valve-sparing aortic procedure can present some problem when a Valsalva graft is used. Since in the Valsalva graft the sinotubular junction height is predetermined, correct matching with native commissures height can be difficult. We propose a method by which it is possible to create a new sinotubular junction in Valsalva graft without altering its original configuration. 相似文献