Research Article
Year 2023,
Volume: 50 Issue: 4 - Cilt 50, Sayı 4, 482 - 489, 29.12.2023
Abstract
References
- 1.Lazaridis K, Tzartos SJ. Autoantibody Specificities inMyasthenia Gravis; Implications for ImprovedDiagnostics and Therapeutics. Front Immunol2020;11:212. doi:10.3389/fimmu.2020.00212
- 2.Nic Hehir MK, Silvestri NJ. Generalized MyastheniaGravis: Classification, Clinical Presentation, NaturalHistory, and Epidemiology. Neurol Clin2018;36(2):253-60. doi:10.1016/j.ncl.2018.01.002
- 3.AAEM Quality Assurance Committee. AmericanAssociation of Electrodiagnostic Medicine. Literaturereview of the usefulness of repetitive nerve stimulation and single fiber EMG in the electrodiagnosticevaluation of patients with suspected myastheniagravis or Lambert-Eaton myasthenic syndrome.Muscle Nerve 2001;24(9):1239-47.doi:10.1002/mus.1140
- 4.Amandusson Å, Elf K, Grindlund ME, Punga AR.Diagnostic Utility of Repetitive Nerve Stimulation in aLarge Cohort of Patients With Myasthenia Gravis. J Clin Neurophysiol 2017;34(5):400-7.doi:10.1097/WNP.0000000000000398
- 5.Abraham A, Alabdali M, Alsulaiman A, et al.Repetitive nerve stimulation cutoff values for thediagnosis of myasthenia gravis. Muscle Nerven2017;55(2):166-70. doi:10.1002/mus.25214
- 6.Stålberg E, van Dijk H, Falck B, et al. Standards forquantification of EMG and neurography. ClinNeurophysiol 2019;130(9):1688-729.doi:10.1016/j.clinph.2019.05.008
- 7.Sanders DB, Arimura K, Cui L, et al. Guidelines forsingle fiber EMG. Clin Neurophysiol2019;130(8):1417-39.doi:10.1016/j.clinph.2019.04.005
- 8.Benatar M. A systematic review of diagnostic studies in myasthenia gravis. Neuromuscul Disord2006;16(7):459-67. doi:10.1016/j.nmd.2006.05.006
- 9.Juel VC. Single fiber electromyography. Handb ClinNeurol 2019;160:303-10. doi:10.1016/B978-0-444-64032-1.00019-9
- 10.Giannoccaro MP, Paolucci M, Zenesini C, et al.Comparison of ice pack test and single-fiber EMGdiagnostic accuracy in patients referred formyasthenic ptosis. Neurology 2020;95(13):e1800-e1806. doi:10.1212/WNL.0000000000010619
- 11.Mercelis R, Merckaert V. Diagnostic utility ofstimulated single-fiber electromyography of theorbicularis oculi muscle in patients with suspectedocular myasthenia. Muscle Nerve 2011;43(2):168-70.doi:10.1002/mus.21853
- 12.Yildirim A, Adatepe T, Gunduz A, Yagiz O, Uzun N.Occipitalis muscle: using for repetitive facial nervestimulation in myasthenia gravis. Acta Neurol Belg2020;120(3):609-13. doi:10.1007/s13760-018-0889-9
- 13.Jaretzki A 3rd, Barohn RJ, Ernstoff RM, et al.Myasthenia gravis: recommendations for clinicalresearch standards. Task Force of the MedicalScientific Advisory Board of the Myasthenia GravisFoundation of America. Ann Thorac Surg2000;70(1):327-34. doi:10.1016/s0003-4975(00)01595-2
- 14.Costa J, Evangelista T, Conceição I, de Carvalho M.Repetitive nerve stimulation in myasthenia gravis--relative sensitivity of different muscles. ClinNeurophysiol 2004;115(12):2776-82.doi:10.1016/j.clinph.2004.05.024
- 15.Pike-Lee T, Higginbotham D, Li Y. Directcomparison of median and ulnar repetitive nervestimulation in generalized myasthenia gravis. MuscleNerve 2021;64(4):490-3. doi:10.1002/mus.27366
- 16.Niks EH, Badrising UA, Verschuuren JJ, Van Dijk JG.Decremental response of the nasalis and hypothenarmuscles in myasthenia gravis. Muscle Nerve2003;28(2):236-8. doi:10.1002/mus.10411
- 17.Witoonpanich R, Dejthevaporn C, SriphrapradangA, Pulkes T. Electrophysiological and immunologicalstudy in myasthenia gravis: diagnostic sensitivity andcorrelation. Clin Neurophysiol 2011;122(9):1873-7.doi:10.1016/j.clinph.2011.02.026
- 18.Sommer N, Sigg B, Melms A, et al. Ocularmyasthenia gravis: response to long-termimmunosuppressive treatment. J Neurol NeurosurgPsychiatry 1997;62(2):156-62.doi:10.1136/jnnp.62.2.156
- 19.Benatar M, Kaminski HJ; Quality StandardsSubcommittee of the American Academy of Neurology.Evidence report: the medical treatment of ocularmyasthenia (an evidence-based review): report of theQuality Standards Subcommittee of the AmericanAcademy of Neurology. Neurology 2007;68(24):2144-149.doi:10.1212/01.wnl.0000263481.14289.90
- 20.Sanders DB, Wolfe GI, Narayanaswami P.Developing treatment guidelines for myastheniagravis. Ann N Y Acad Sci 2018;1412(1):95-101.doi:10.1111/nyas.13537
- 21.Rubin DI, Hentschel K. Is exercise necessary withrepetitive nerve stimulation in evaluating patientswith suspected myasthenia gravis?. Muscle Nerve2007;35(1):103-6. doi:10.1002/mus.20667
Year 2023,
Volume: 50 Issue: 4 - Cilt 50, Sayı 4, 482 - 489, 29.12.2023
Abstract
Objective: This research offers a comprehensive analysis of Myasthenia Gravis (MG), uncovering the remarkable accuracy of spinal accessory, ulnar, and facial nerve repetitive nerve stimulation (RNS), along with the precision of single fiber electromyography (SF-EMG) in MG diagnosis. We also embark on an exploration of clinical features and autoantibody test results in generalized MG patients.
Methods: In this prospective study, we welcomed 31 individuals definitively diagnosed with generalized MG into our quest. The categorization of patients was conducted in accordance with the criteria set by the Myasthenia Gravis Foundation of America (MGFA). We examined patients' trapezius, nasalis, and abductor digiti minimi (ADM) muscles using RNS. We meticulously recorded the presence of MG autoantibodies, clinical subtypes based on affected muscle groups, and SF-EMG jitter rates.
Results: The mean age of the 31 patients of whom 19 (61.3%) were male, was 64 ± 13.9 years. Among them, 20 showed positivity in the Anti-AChR antibody test. In 28 patients, accounting for 90.3% of the study group, single fiber electromyography (EMG) displayed increased jitter. There were 4 (12.9%), 24 (77.4%) and 12 (38.7%) patients featuring a decremental response of exceeding 10% in ADM, trapezius and nasalis muscles, respectivelyOur investigation revealed notable findings, such as the absence of substantial correlations between decremental response rates and age, gender, duration of complaints, antibody test results, thymus abnormalities, affected muscle types, familial history, or increased jitter rates in SF-EMG (p>0.05).
Conclusion: As our findings clearly show, we can confidently attest to the remarkable sensitivity of RNS in MG diagnosis when muscle selection is precise. A gem discovered on our study is the high sensitivity of the spinal accessory nerve, a revelation that should guide the course of routine RNS studies, particularly for those facing ocular-onset myasthenia.
References
- 1.Lazaridis K, Tzartos SJ. Autoantibody Specificities inMyasthenia Gravis; Implications for ImprovedDiagnostics and Therapeutics. Front Immunol2020;11:212. doi:10.3389/fimmu.2020.00212
- 2.Nic Hehir MK, Silvestri NJ. Generalized MyastheniaGravis: Classification, Clinical Presentation, NaturalHistory, and Epidemiology. Neurol Clin2018;36(2):253-60. doi:10.1016/j.ncl.2018.01.002
- 3.AAEM Quality Assurance Committee. AmericanAssociation of Electrodiagnostic Medicine. Literaturereview of the usefulness of repetitive nerve stimulation and single fiber EMG in the electrodiagnosticevaluation of patients with suspected myastheniagravis or Lambert-Eaton myasthenic syndrome.Muscle Nerve 2001;24(9):1239-47.doi:10.1002/mus.1140
- 4.Amandusson Å, Elf K, Grindlund ME, Punga AR.Diagnostic Utility of Repetitive Nerve Stimulation in aLarge Cohort of Patients With Myasthenia Gravis. J Clin Neurophysiol 2017;34(5):400-7.doi:10.1097/WNP.0000000000000398
- 5.Abraham A, Alabdali M, Alsulaiman A, et al.Repetitive nerve stimulation cutoff values for thediagnosis of myasthenia gravis. Muscle Nerven2017;55(2):166-70. doi:10.1002/mus.25214
- 6.Stålberg E, van Dijk H, Falck B, et al. Standards forquantification of EMG and neurography. ClinNeurophysiol 2019;130(9):1688-729.doi:10.1016/j.clinph.2019.05.008
- 7.Sanders DB, Arimura K, Cui L, et al. Guidelines forsingle fiber EMG. Clin Neurophysiol2019;130(8):1417-39.doi:10.1016/j.clinph.2019.04.005
- 8.Benatar M. A systematic review of diagnostic studies in myasthenia gravis. Neuromuscul Disord2006;16(7):459-67. doi:10.1016/j.nmd.2006.05.006
- 9.Juel VC. Single fiber electromyography. Handb ClinNeurol 2019;160:303-10. doi:10.1016/B978-0-444-64032-1.00019-9
- 10.Giannoccaro MP, Paolucci M, Zenesini C, et al.Comparison of ice pack test and single-fiber EMGdiagnostic accuracy in patients referred formyasthenic ptosis. Neurology 2020;95(13):e1800-e1806. doi:10.1212/WNL.0000000000010619
- 11.Mercelis R, Merckaert V. Diagnostic utility ofstimulated single-fiber electromyography of theorbicularis oculi muscle in patients with suspectedocular myasthenia. Muscle Nerve 2011;43(2):168-70.doi:10.1002/mus.21853
- 12.Yildirim A, Adatepe T, Gunduz A, Yagiz O, Uzun N.Occipitalis muscle: using for repetitive facial nervestimulation in myasthenia gravis. Acta Neurol Belg2020;120(3):609-13. doi:10.1007/s13760-018-0889-9
- 13.Jaretzki A 3rd, Barohn RJ, Ernstoff RM, et al.Myasthenia gravis: recommendations for clinicalresearch standards. Task Force of the MedicalScientific Advisory Board of the Myasthenia GravisFoundation of America. Ann Thorac Surg2000;70(1):327-34. doi:10.1016/s0003-4975(00)01595-2
- 14.Costa J, Evangelista T, Conceição I, de Carvalho M.Repetitive nerve stimulation in myasthenia gravis--relative sensitivity of different muscles. ClinNeurophysiol 2004;115(12):2776-82.doi:10.1016/j.clinph.2004.05.024
- 15.Pike-Lee T, Higginbotham D, Li Y. Directcomparison of median and ulnar repetitive nervestimulation in generalized myasthenia gravis. MuscleNerve 2021;64(4):490-3. doi:10.1002/mus.27366
- 16.Niks EH, Badrising UA, Verschuuren JJ, Van Dijk JG.Decremental response of the nasalis and hypothenarmuscles in myasthenia gravis. Muscle Nerve2003;28(2):236-8. doi:10.1002/mus.10411
- 17.Witoonpanich R, Dejthevaporn C, SriphrapradangA, Pulkes T. Electrophysiological and immunologicalstudy in myasthenia gravis: diagnostic sensitivity andcorrelation. Clin Neurophysiol 2011;122(9):1873-7.doi:10.1016/j.clinph.2011.02.026
- 18.Sommer N, Sigg B, Melms A, et al. Ocularmyasthenia gravis: response to long-termimmunosuppressive treatment. J Neurol NeurosurgPsychiatry 1997;62(2):156-62.doi:10.1136/jnnp.62.2.156
- 19.Benatar M, Kaminski HJ; Quality StandardsSubcommittee of the American Academy of Neurology.Evidence report: the medical treatment of ocularmyasthenia (an evidence-based review): report of theQuality Standards Subcommittee of the AmericanAcademy of Neurology. Neurology 2007;68(24):2144-149.doi:10.1212/01.wnl.0000263481.14289.90
- 20.Sanders DB, Wolfe GI, Narayanaswami P.Developing treatment guidelines for myastheniagravis. Ann N Y Acad Sci 2018;1412(1):95-101.doi:10.1111/nyas.13537
- 21.Rubin DI, Hentschel K. Is exercise necessary withrepetitive nerve stimulation in evaluating patientswith suspected myasthenia gravis?. Muscle Nerve2007;35(1):103-6. doi:10.1002/mus.20667
There are 21 citations in total.
Details
| Primary Language | English |
|---|---|
| Subjects | Medical Education |
| Journal Section | Original Articles |
| Authors | |
| Publication Date | December 29, 2023 |
| Submission Date | October 21, 2023 |
| Acceptance Date | December 8, 2023 |
| Published in Issue | Year 2023 Volume: 50 Issue: 4 - Cilt 50, Sayı 4 |