Prognosis of myasthenia gravis: A multicenter follow-up study of 844 patients

doi:10.1016/0022-510X(91)90260-E

Journal of the Neurological Sciences

Volume 106, Issue 2, December 1991, Pages 213-220

https://doi.org/10.1016/0022-510X(91)90260-E Get rights and content

Abstract

The prognosis of myasthenia gravis (MG) was assessed retrospectively using life-table analysis in 844 patients followed up for a mean period of 5 years in 3 major Italian centers. The chance of achieving at least a 1-year remission after treatment withdrawal (complete remission) was assessed as a specific end-point in the whole population and in selected subgroups with reference to the principal prognostic variables. The cumulative probability of complete remission was 1% by 1 year, 8% by 3 years. 13% by 5 years, and 21% by 10 years. The only variables correlated to the chance of complete remission were younger age at onset of MG, lower severity of symptoms at onset and nadir, and shorter disease duration at diagnosis. In addition, thymectomy and early surgery seemed to influence the chance of remission. Other factors (including the presence of thymoma) did not significantly influence the outcome of the disease.

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The aim of this study was to investigate the surgical and long-term neurological outcomes of patients with acetylcholine-receptor-antibody-associated myasthenia gravis (AChR-MG) who underwent robotic thymectomy (RATS). We retrospectively analyzed the clinical-pathological data of all patients with AChR-MG who underwent RATS using the DaVinci® Robotic System at the MUMC+ between April 2004 and December 2018. Follow-up data were collected from 60 referring Dutch hospitals. In total, 230 myasthenic patients including 76 patients with a thymoma (33.0%) were enrolled in this study. Mean follow-up time, procedure time and hospitalization were, respectively 65.7 ± 43.1 months, 111±52.5 min and 3.3 ± 2.2 days. Thymomatous patients had significantly more frequently and more severe complications than nonthymomatous patients (18.4% vs. 3.9%, p<0.001). Follow up data was available in 71.7% of the included patients. The Myasthenia Gravis Foundation of America postintervention score showed any kind of improvement of MG-symptoms after RATS in 82.4% of the patients. Complete stable remission (CSR) or pharmacological remission (PR) of MG was observed in 8.4% and 39.4% of the patients, respectively. Mean time till CSR/PR remission after thymectomy was 26.2 ± 29.2 months. No statistical difference was found in remission or improvement in MGFA scale between thymomatous and nonthymomatous patients. RATS is safe and feasible in patients with MG. The majority of the patients (82.4%) improved after thymectomy. CSR and PR were observed in 8.4% and 39.4% of the patients, respectively, with a mean of 26.2 months after thymectomy. Thymomatous patients had more frequently and more severe complications compared to nonthymomatous patients.
Novel treatment strategies for acetylcholine receptor antibody-positive myasthenia gravis and related disorders
2022, Autoimmunity Reviews
The presence of autoantibodies directed against the muscle nicotinic acetylcholine receptor (AChR) is the most common cause of myasthenia gravis (MG). These antibodies damage the postsynaptic membrane of the neuromuscular junction and cause muscle weakness by depleting AChRs and thus impairing synaptic transmission. As one of the best-characterized antibody-mediated autoimmune diseases, AChR-MG has often served as a reference model for other autoimmune disorders. Classical pharmacological treatments, including broad-spectrum immunosuppressive drugs, are effective in many patients. However, complete remission cannot be achieved in all patients, and 10% of patients do not respond to currently used therapies. This may be attributed to production of autoantibodies by long-lived plasma cells which are resistant to conventional immunosuppressive drugs. Hence, novel therapies specifically targeting plasma cells might be a suitable therapeutic approach for selected patients. Additionally, in order to reduce side effects of broad-spectrum immunosuppression, targeted immunotherapies and symptomatic treatments will be required. This review presents established therapies as well as novel therapeutic approaches for MG and related conditions, with a focus on AChR-MG.
Comparison of Conservative Treatment and Thymectomy on Myasthenia Gravis Outcome
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A systematic review of the literature was performed to compare long-term outcomes of thymectomy and medical treatment for generalized myasthenia gravis (MG).
A Medline search through June 2015 resulted in 71 studies, 27 of which were selected (10,140 patients: 5,275 thymectomies, 4,865 medication).
The pooled proportion of remission with thymectomy was 0.31 (95% CI, 0.25–0.37), with conservative treatment it was 0.15 (95% CI, 0.12–0.18). The odds ratio (OR) of remission with thymectomy in comparison with medication alone was 2.44 (95% CI, 1.91–3.12) overall and according to medication type and remission definitions.
Thymectomy is superior to conservative treatment with solely medication on remission in MG.
Repetitive nerve stimulation often fails to detect abnormal decrement in acute severe generalized Myasthenia Gravis
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We assessed the diagnostic pattern of repetitive nerve stimulation (RNS) test and concentric electrode (CNE) jitter analysis between patients with generalized myasthenia gravis (GMG) with acute versus slow onset.
All examinations that established the diagnosis of GMG at the department of Clinical Neurophysiology, Uppsala University Hospital, were retrospectively analyzed from January 2012 to December 2014. Patients were grouped according to disease duration at neurophysiological evaluation: acute onset (<4 weeks) or slow onset (⩾4 weeks).
We identified 41 patients diagnosed with GMG. Of the nine patients with acute onset GMG (5 women) only one patient had abnormal decrement, whereas of the 32 patients with slow onset (13 women) 26 patients (84%) had abnormal decrement. CNE jitter was abnormal in all. AChR antibody status was comparable (78% versus 84%) whereas the MGFA class was higher in the acute onset group (range: 3A–5) compared to the slow onset group (range: 2A–3B).
RNS test is frequently normal in cases of acute severe GMG, including myasthenic crisis. Performing CNE jitter analysis is therefore of crucial importance for a correct early diagnosis.
MG patients with acute severe onset of bulbar or generalized fatigue often have normal findings on RNS test in proximal muscles.
Efficacy of ravulizumab in patients with generalized myasthenia gravis by time from diagnosis: A post hoc subgroup analysis of the CHAMPION MG study
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The diagnostic and prognostic utility of repetitive nerve stimulation in patients with myasthenia gravis
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^☆: Presented in part at the 41st Annual Meeting of the American Academy of Neurology, Chicago. IL, U.S.A., April 13–19, 1989, and at the XIVth World Congress of Neurology. New Delhi, India. October 22–27, 1989.

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Research paperPrognosis of myasthenia gravis: A multicenter follow-up study of 844 patients☆

Abstract

Am. J. Med.

J. Thorac. Cardiovasc. Surg.

J. Thorac. Cardiovasc. Surg.

Am. J. Surg.

J. Neurol. Sci.

Prognosis of ocular myasthenia

Ann. Neurol.

The value of thymectomy in myasthenia gravis

Ann. Surg.

Clinical, pathological, HLA antigen and immunological evidence of disease heterogeneity in myasthenia gravis

Brain

Regression models and life-tables

J.R. Statist. Soc. B

Evaluations of results of thymectomy in myasthenia gravis

J. Neurol.

Course and management of myasthenia gravis

JAMA

The course of myasthenia gravis and therapies affecting outcome

Ann. N.Y. Acad. Sci.

Heterogeneity in myasthenia gravis: HLA phenotypes and autoantibody responses in ocular and generalized type

Ann. Neurol.

Research paper
Prognosis of myasthenia gravis: A multicenter follow-up study of 844 patients☆