Generalization after ocular onset in myasthenia gravis: a case series in Germany

Li, Feng; Hotter, Benjamin; Swierzy, Marc; Ismail, Mahmoud; Meisel, Andreas; Rückert, Jens-C.

doi:10.1007/s00415-018-9056-8

Cited by 39 publications

(47 citation statements)

References 39 publications

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“…A small platform was found between 17 and 24 months after disease onset, and the conversion occurred slower after that. Similar deceleration was also found in the study of Li et al [19].…”

Section: Discussionsupporting

confidence: 90%

“…Second, most of the medical data were obtained retrospectively and the follow-up period of patients was not long enough. Third, several studies reported that steroid use is a protective factor and may delay time to the conversion [13,19], but we could not effectively evaluate it because of the small number of patients whose immunosuppressant treatment could be evaluated and the design of this retrospective study. A prospective and controlled study or a well-designed real-world study will provide more valuable information.…”

Section: Discussionmentioning

confidence: 98%

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Adult Ocular Myasthenia Gravis Conversion: A Single-Center Retrospective Analysis in China

et al. 2020

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Introduction: The conversion rate from ocular myasthenia gravis (OMG) to generalized myasthenia gravis (GMG) was reported to be much lower in Asian population since most OMG patients are juvenile onset. However, the exact conversion rate for adult-onset OMG to GMG is still unknown. Objective: We aimed to delineate the conversion rate and risk factors for adult patients with ocular onset to GMG. Methods: Adult myasthenia gravis (MG) patients with ocular onset (age > 18 years) were retrospectively reviewed. Patients with confined ocular involvement lasting more than 2 years (pure OMG group) and those who converted into GMG (converted OMG group) were enrolled for subsequent analysis. We then analyzed 5 clinical variables, including onset age, sex, onset symptoms, anti-acetylcholine receptor antibody (AChR Ab), and thymus CT. Survival analysis was applied to all enrolled patients to explore risk factors associated with conversion. Results: In a total number of 249 ocular-onset MG patients initially enrolled, we excluded 122 patients with OMG lasting less than 2 years. The remaining 127 patients were enrolled, including 106 converted OMG and 21 pure OMG patients. Converted OMG patients had an older onset age (threshold: 43 years) and higher anti-AChR Ab titer (threshold: 6.13 nmol/L). The estimated conversion rate was 70.64%. Moreover, 67% of conversion occurred within 2 years after onset. Cox regression of survival analysis revealed that higher anti-AChR Ab titer and bilateral ptosis were associated with a higher conversion rate. Conclusions: The conversion of adult OMG was associated with anti-AChR Ab titer, onset age, and bilateral ptosis. The estimated conversion rate of Chinese adult OMG patients was 70%.

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“…A small platform was found between 17 and 24 months after disease onset, and the conversion occurred slower after that. Similar deceleration was also found in the study of Li et al [19].…”

Section: Discussionsupporting

confidence: 90%

Section: Discussionmentioning

confidence: 98%

Adult Ocular Myasthenia Gravis Conversion: A Single-Center Retrospective Analysis in China

et al. 2020

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“…In as "ocular or bulbar plus limb muscle weakness", but in the definition of Li et al the presence of limb muscle weakness was not necessary and bulbar weakness alone was sufficient ("symptoms other than ocular symptoms, such as dysarthria, dysphagia, dyspnea or weakness of the jaw, neck or arms and legs"). [9,17] For this reason we avoided the term 'generalized weakness' in our phenotype classification and opted to describe different muscle regions separately.…”

Section: Discussionmentioning

confidence: 99%

Heterogeneity and shifts in distribution of muscle weakness in myasthenia gravis

Meel

Tannemaat

Verschuuren

2019

Neuromuscular Disorders

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The distribution of muscle weakness in myasthenia gravis (MG) patients with acetylcholine receptor (AChR) antibodies is highly variable. As muscle groups respond differently to therapeutic interventions, it is important to acknowledge this variability. We analysed the distribution of muscle weakness in 225 AChR MG patients over time. On the basis of combinations of muscle weakness, seven phenotypes were defined: 'ocular' (O), 'bulbar' (B), 'neck/limbs/respiratory' (NLR), or a combination (O + B, O + NLR, B + NLR and O + B + NLR). MG remained restricted to ocular weakness in 5%, whereas 7% never had ocular weakness. At last follow-up, ocular or bulbar weakness had resolved more frequently than NLR weakness (40%, 38% and 25%; p = 0.003, respectively). Patients with O, B or OB phenotype at baseline had a higher age at onset and were more frequently male than patients with NLR, ONLR, BNLR or OBNLR phenotype (52.7 ± 17.5 vs. 44.0 ± 18.9; p = 0.007 and 64% vs. 37%; p = 0.002, respectively). MG patients have heterogeneous distributions of muscle weakness and frequently shift between phenotypes. The phenotypic variations found in AChR MG suggest that also other factors aside from the AChR antibody mediated immune response are of importance in determining the disease expression in MG.

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“…Some scholars indicated positive AChR-Ab, abnormal RNS tests and abnormal SFEMG tests in the early stages of disease could predict more GMG conversion [9,23,24]. While other researches pointed out these factors could not be predictors [8,25].Due to restrictions of earlier objective condition, we did not carry out the AChR-Ab titer detection. In further study, we will take it into consideration.…”

Section: Discussionmentioning

confidence: 95%

“…Both in Korean and Singapore, repetitive nerve stimulation (RNS) tests and antiacetylcholine receptor antibody (AChR-Ab) could be predictive factors for conversion of OMG to GMG [6,7]. While in a Germany study, the scholar deemed the presence of thymoma was the only risk factor for secondary generalization [8].…”

Section: Introductionmentioning

confidence: 99%

Prediction of myasthenia gravis generalization under immunosuppressive therapy in Northwest China

Ding

Zhao

Ren

et al. 2020

Preprint

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Objective It is well demonstrated that immunosuppressants can reduce, but not eliminate the risk of generalized development in ocular myasthenia gravis (OMG). Herein, we explored the potential predictors of conversion of OMG patients receiving immunosuppressive treatments. Methods OMG patients under immunosuppressive treatments in Tangdu Hospital from June 2008 to June 2012 were retrospectively reviewed. Baseline clinical characteristics were documented. Patients were followed up regularly by face-to-face interview to evaluate clinic status and treatment efficacy. Main outcome measure was generalized conversion and the relative data were collected and analyzed by logistic regression. Results 223 eligible OMG patients completed the final follow-up visit and 38 (17.0%) progressed to generalized MG (GMG) at a median interval of 0.9 year. Patients with adult onset and positive repetitive nerve stimulation (RNS) of facial or axillary nerve had higher conversion rate than those with juvenile onset and negative RNS ( p = 0.001; p = 0.019; p = 0.015, respectively). Adult-onset patients converted earlier than juvenile-onset OMG patients ( p = 0.014). Upon multivariate logistic regression analysis, age of onset (Odds ratio [OR] 1.023, 95% confidence interval [CI] 1.006–1.041, p = 0.007) and positive facial nerve RNS (OR 2.826, 95%CI 1.045–5.460, p = 0.038) were found to be positively associated with generalized development. Moreover, an obviously negative association was found for disease duration (OR 0.603, 95%CI 0.365–0.850, p = 0.019). Conclusions Age of onset, disease duration and facial nerve RNS test can predict conversion of OMG under immunosuppressive treatment. Adult-onset, shorter disease duration and facial nerve RNS-positive patients have a higher risk of generalized development.

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Generalization after ocular onset in myasthenia gravis: a case series in Germany

Abstract: The conversion rate in ocular myasthenia was high in our series, predicted by the presence of a thymoma. Our findings suggest that corticosteroids can prevent secondary generalization in ocular myasthenia patients with thymic hyperplasia, which requires further research.

Cited by 39 publications

References 39 publications

Adult Ocular Myasthenia Gravis Conversion: A Single-Center Retrospective Analysis in China

Adult Ocular Myasthenia Gravis Conversion: A Single-Center Retrospective Analysis in China

Heterogeneity and shifts in distribution of muscle weakness in myasthenia gravis

Prediction of myasthenia gravis generalization under immunosuppressive therapy in Northwest China

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