Contribution of anti-ryanodine receptor antibody to impairment of excitation-contraction coupling in myasthenia gravis
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摘要

Objective

The aim of this study was to elucidate the relationship between the impairment of excitation-contraction (E-C) coupling and anti-ryanodine receptor (RyR) antibody in patients with myasthenia gravis (MG).

Methods

Masseteric compound muscle action potential (CMAP) and mandibular movement-related potentials (MRPs) were recorded simultaneously after stimulating the trigeminal motor nerve with a needle electrode. The E-C coupling time (ECCT) was calculated as the latency difference between CMAP and MRP. For each patient, we selected a representative data set when there was no abnormal decrement in response to repetitive nerve stimulation. The 26 data sets were divided into an anti-RyR-positive group (n = 12) and an anti-RyR-negative group (n = 14).

Results

Masseteric ECCT was significantly longer (p = 0.017) in anti-RyR-positive group (median, mean, range; 3.6, 3.8, 3.0-5.9 ms) than in anti-RyR-negative group (3.1, 3.1, 2.7-4.0) although there were no significant differences in masseteric CMAP amplitude and%decrement between the two groups. The bite force was significantly lower in anti-RyR-positive group than in normal controls.

Conclusions

Presence of anti-RyR antibodies is associated with significantly prolonged masseteric ECCT compared to absence of the antibodies in MG.

Significance

Anti-RyR antibody contributes to E-C coupling impairment in the masseter muscle in patients with MG.

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