Document Type : Original Article
Authors
- Zahra Vahabi 1
- Ferdos Nazari 2
- Farzad Fatehi 2
- Valiolah Bayegi 3
- Zahra Saffarian 4
- Fatemeh Saffarian 5
- Shahriar Nafissi 2
1 Department of Geriatric Medicine, Ziaeian Hospital, Tehran University of Medical Sciences, Tehran, Iran Division of Memory and Behavioral Neurology, Roozbeh Hospital, Tehran University of Medical Sciences, Tehran, Iran
2 Department of Neurology, Neuromuscular Research Center, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
3 Department of Epidemiology and Biostatistics, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran Sina Trauma and Surgery Research Center, Tehran University of Medical Sciences, Tehran, Iran
4 Department of Dermatology, Imam Khomeini Hospital Complex, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
5 Department of Cardiology, School of Medicine, Qazvin University of Medical Sciences, Qazvin, Iran
Abstract
Background: Myasthenia gravis (MG) affects the neuromuscular transmission, causing fluctuating muscle weakness and fatigue. This study is carried out with the aim to study the electrophysiologic findings of different subtypes of MG referred to our center in Tehran, Iran.
Methods: All patients with MG presenting to neurology department of Shariati Hospital, Tehran University of Medical Sciences were enrolled. Clinically, patients with MG were categorized as ocular vs. generalized. The acetylcholine receptor (Ach-R) and muscle-specific receptor tyrosine kinase (anti-MuSK) antibodies were performed. Repetitive Nerve Stimulation (RNS) was performed using the standard method, with supramaximal stimulation of muscles at the 3 Hz frequency by surface electrode at rest. Abductor pollicis brevis (APB) (median nerve), anconeus (radial nerve), trapezius (accessory nerve), and nasalis (facial nerve) muscles were studied in all patients. Single fiber electromyography (SFEMG) was performed by standard method.
Results: 196 seropositive patients with MG were included in the study. In electrophysiological studies, RNS was performed for 146 patients of Ach-R-Ab positive MG, with positive results in 110 patients. In addition, SFEMG was conducted for 8 patients with negative RNS, which resulted in 7 positive tests.
Among 23 patients with anti-MuSK-positive MG, RNS was performed for 16 patients, with positive results in 11 patients. The 5 remaining patients with negative RNS test were studied by SFEMG, 4 of whom had positive results. APB compound muscle action potential (CMAP) decrementation significantly correlated with Ach-R-Ab positive MG (P < 0.03).
Conclusion: This finding can support the hypothesis that the selection of muscles in electrodignostic study would be important. The electrodiagnostic studies are a good and non-invasive diagnostic tool for MG, and a combination of different distal, proximal, and facial muscles can increase the overall sensitivity of the test.
Keywords
- Sieb JP. Myasthenia gravis: An update for the clinician. Clin Exp Immunol 2014; 175(3): 408-18.
- Gilhus NE, Verschuuren JJ. Myasthenia gravis: Subgroup classification and therapeutic strategies. Lancet Neurol 2015; 14(10): 1023-36.
- Jing F, Cui F, Chen Z, Yang F, Ling L, Huang X. Clinical and electrophysiological markers in myasthenia gravis patients. Eur Neurol 2015; 74(1-2): 22-7.
- Abraham A, Alabdali M, Alsulaiman A, Breiner A, Barnett C, Katzberg HD, et al. Repetitive nerve stimulation cutoff values for the diagnosis of myasthenia gravis. Muscle Nerve 2017; 55(2): 166-70.
- Kouyoumdjian JA, Stalberg EV. Reference jitter values for concentric needle electrodes in voluntarily activated extensor digitorum communis and orbicularis oculi muscles. Muscle Nerve 2008; 37(6): 694-9.
- Pasnoor M, Wolfe GI, Nations S, Trivedi J, Barohn RJ, Herbelin L, et al. Clinical findings in MuSK-antibody positive myasthenia gravis: A U.S. experience. Muscle Nerve 2010; 41(3): 370-4.
- Liang T, Boulos MI, Murray BJ, Krishnan S, Katzberg H, Umapathy K. Detection of myasthenia gravis using electrooculography signals. Annu Int Conf IEEE Eng Med Biol Soc 2016; 2016: 896-9.
- Bou Ali H., Salort-Campana E, Grapperon AM, Gallard J, Franques J, Sevy A, et al. New strategy for improving the diagnostic sensitivity of repetitive nerve stimulation in myasthenia gravis. Muscle Nerve 2017; 55(4): 532-8.
- Yan C, Song J, Pang S, Yi F, Xi J, Zhou L, et al. Palpebral portion of the orbicularis oculi muscle to repetitive nerve stimulation testing: A potential assessment indicator in patients with generalized myasthenia gravis. J Clin Neurosci 2018; 48: 238-42.
- Patil SA, Bokoliya SC, Nagappa M, Taly AB. Diagnosis of myasthenia gravis: Comparison of anti-nicotinic acetyl choline receptor antibodies, repetitive nerve stimulation and Neostigmine tests at a tertiary neuro care centre in India, a ten-year study. J Neuroimmunol 2016; 292: 81-4.
- Witoonpanich R, Dejthevaporn C, Sriphrapradang A, Pulkes T. Electrophysiological and immunological study in myasthenia gravis: Diagnostic sensitivity and correlation. Clin Neurophysiol 2011; 122(9): 1873-7.
- Nikolic A, Basta I, Stojanovic VR, Stevic Z, Lavrnic D. Electrophysiological profile of the patients with MuSK positive myasthenia gravis. Neurol Res 2014; 36(11): 945-9.
- Kim SW, Sunwoo MK, Kim SM, Shin HY, Sunwoo IN. Repetitive nerve stimulation in musk-antibody-positive myasthenia gravis. J Clin Neurol 2017; 13(3): 287-92.
- Zambelis T, Kokotis P, Karandreas N. Repetitive nerve stimulation of facial and hypothenar muscles: Relative sensitivity in different myasthenia gravis subgroups. Eur Neurol 2011; 65(4): 203-7.