Abstract
Late-onset myasthenia gravis (LOMG) is a unique MG subgroup. More information is needed on its subgroups such as non-thymomatous generalized LOMG. We evaluated the effect of demographic, clinical, and serological factors as well as different immunosuppressive modalities on outcome in generalized non-thymomatous LOMG with onset ≥ 50 years. Myasthenia Gravis Foundation of America (MGFA) Clinical Classification, MGFA postintervention score (MGFA PIS) and MG Composite scores were obtained to define the severity of disease and clinical outcome. In 95 patients with generalized non-thymomatous LOMG, 60 (63%) were men, 45 (47%) had mild disease, 80 (84%) were anti-AChR, and 56 (61%) were anti-titin positive. In those who received immunosuppressives and provided the clinical scores (84 patients), 50 (60%) had favorable outcome (MGFA PIS categories of complete stable remission, pharmacological remission and minimal manifestations) at the end of 3 years. Use of prednisone + azathioprine had significantly positive effect on outcome. The presence of anti-titin antibodies had no significant effect on severity and outcome. Five anti-MuSK-positive patients had favorable outcome. In conclusion, the presence of neither anti-titin nor anti-MuSK antibodies points to unfavorable outcome. Prednisone and azathioprine combination has beneficial effects in non-thymomatous generalized LOMG.
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We thank Dr. Murat Kurtuncu for his help in statistical analysis.
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The study was supported by the Scientific Research Project, Istanbul University (#34256).
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SYC, HD, VY and GSD collected and integrated the data. SYC and FD conceived and designed the study. SYC, YGP, PSO and FD analyzed the data, wrote and reviewed the paper. All authors approved the final version of this paper for publication.
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All procedures performed in this study were in accordance with the ethical standards of the Medical Ethical Committee at the Istanbul Medical Faculty, Istanbul University (no.: 2013/95) and the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
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Yildiz Celik, S., Durmus, H., Yilmaz, V. et al. Late-onset generalized myasthenia gravis: clinical features, treatment, and outcome. Acta Neurol Belg 120, 133–140 (2020). https://doi.org/10.1007/s13760-019-01252-x
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DOI: https://doi.org/10.1007/s13760-019-01252-x