RESUMO
Objective:To establish the reference values of stimulation single fiber electromyography (SFEMG) in orbicularis oculi, and to explore its sensitivity in repetitive nerve stimulation (RNS) negative ocular myasthenia gravis (OMG) patients, and the relationship between jitter and various clinical parameters.Methods:Thirty-two healthy volunteers were included to establish the reference value of normal controls from January 2019 to December 2019. From December 2019 to January 2023, 36 OMG patients with negative RNS were collected. Quantitative MG score (QMGS) was performed, neostigmine test and antibody titers as well as thymus CT results were recorded. One side of the orbicularis oculi muscle was tested with a disposable concentric needle electrode in stimulation SFEMG, and the mean consecutive difference (MCD) value was calculated, which was compared with the average MCD value and upper limit of individual values in normal controls to evaluate whether the jitter was abnormal. Spearman correlation analysis of abnormal mean MCD values with QMGS and antibody titer was conducted.Results:Among the 32 healthy volunteers, there were 13 males and 19 females, the age was (46.8 ±18.7) years, and the MCD was (19.0 ±4.4) μs. The upper limit of the reference value was 27.7 μs for average MCD, and 37.4 μs for 10% individual values. Among 36 OMG patients negative at RNS tests, 20 were male and 16 were female, with a age of (37.2 ±17.0) years. The MCD was (29.9 ±14.7) μs, and Jitter was abnormal in 29 patients (81%). Among them, 20 (20/25) patients were antibody positive, 6 (6/26) patients had thymic hyperplasia, and 7 (7/26) patients had thymoma. The QMGS was 3(2, 4). There were 7 patients (19%) with normal jitter, whose QMGS was 3(2, 4). Among the patients with normal Jitter, 5 (5/5) patients were antibody positive, 2 (2/6) patients had thymic hyperplasia. There was no statistically significant difference in clinical indicators between the two groups of patients with abnormal or normal jitter. There was no significant correlation in antibody titer or QMGS with abnormal mean MCD value. Conclusions:The upper limit of the mean MCD value in the normal controls is 27.7 μs. The upper limit of a single value is 37.4 μs. Its sensitivity for OMG patients with RNS negative is 81%, and the abnormal mean MCD value does not show a significant correlation with various clinical indicators. Abnormal jitter indicates dysfunction of neuromuscular junction transmission, which is an important neuroelectrophysiological indicator for MG patients and is suitable for RNS negative patients. Orbicularis oculi muscle stimulation SFEMG provides a reliable and sensitive electrophysiological means for functional evaluation of neuromuscular junction.
RESUMO
BACKGROUND: The axonal stimulation single fiber electromyography (S-SFEMG) is a relatively new electrophysio-logical technique and has several advantages over conventional voluntarily activated single fiber electromyography (V-SFEMG). This study was performed in patients with myasthenia gravis (MG) in order to analyze their neuromuscular transmission defects and thus to verify the usefulness of the S-SFEMG technique. METHODS: In 44 patients with MG, S-SFEMG was performed on the extensor digitorum communis muscle. The repetitive nerve stimulation test (RNST) on orbicularis oculi, trapezius, flexor carpi ulnaris, and abductor digiti quinti muscles was also performed at the same time. The results of the RNS and S-SFEMG were then analysed in detail. RESULTS: The S-SFEMG was found abnormal in 84.1%, while RNST was found abnormal in 75.0% of the patients tested. The normal S-SFEMG result was observed exclusively among 7 ocular type MG patients. The mean of the mean consecutive difference (MCD) value, % of fibers with blocking and % of fibers with abnormal jitter, was more increased in patients with generalized type MG than those with ocular type MG. This difference was statistically significant (P<0.01) in all 3 variables. CONCLUSIONS The S-SFEMG is a highly sensitive and useful diagnostic tool in MG. Although it demands more strict technical consideration than V-SFEMG, it is less time-consuming and applicable to uncooperative patients including children. Our study shows S-SFEMG to be especially useful in patients with ocular type MG whose RNST results do not show definite decremen-tal responses.