Factors affecting outcome in ocular myasthenia gravis

Mazzoli, Marco; Ariatti, Alessandra; Valzania, Franco; Kaleci, Shaniko; Tondelli, Manuela; Nichelli, Paolo Frigio; Galassi, Giuliana

doi:10.1080/00207454.2017.1344237

Cited by 55 publications

(64 citation statements)

References 43 publications

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“…The chance of undergoing generalization was almost double in females compared with males. A previous study also found that female gender was a risk factor for generalization; this finding is consistent with several other studies ( 2 , 22 ). The specific role of gender in the pathogenesis of MG, however, remains unclear.…”

Section: Discussionsupporting

confidence: 92%

Clinical Characteristics of Juvenile Myasthenia Gravis in Southern China

Huang

Feng

et al. 2018

Front. Neurol.

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ObjectivesTo describe the clinical profile, clinical outcomes and factors that may affect the outcome of juvenile myasthenia gravis (JMG) patients in southern China.MethodsWe reviewed information relating to JMG patients treated and evaluated at the First Affiliated Hospital, Sun Yat-sen University, between 1998 and 2015. The study involved 327 JMG patients who had been followed up for ≥1 year.ResultsOverall, 77.4% patients showed initial symptoms in the prepubertal period (<12 years). 306 patients showed only ocular symptoms at onset. By the final follow-up, 61 ocular myasthenia gravis (OMG) patients (61/306, 19.9%) had developed generalized myasthenia gravis (GMG). Anti-acetylcholine receptor antibodies (AChR-Ab) titer was an independent risk factor for generalization. Eleven patients (3.4%) experienced spontaneous remission, but four relapsed. Low-dose oral prednisone (0.25 mg/kg) was administered when symptoms did not significantly improve after pyridostigmine treatment. Immunosuppressants were administered when prednisone was unsatisfactory. Optimal outcome was achieved in 59.6% of patients. Specifically, 60 patients (18.3%) attained complete stable remission (CSR), 12 (3.7%) attained pharmaceutical remission (PR), and 123 (37.6%) attained minimal manifestation (MM). In total, 53 OMG patients (21.5%) attained CSR, a significantly higher proportion than among the GMG patients (8.6%, P = 0.009). Moreover, 67.2% of patients with duration <2 years showed significant clinical improvement compared with 46.3% of those with duration >2 years (P < 0.001). Thymectomy did not exhibit definite efficacy for JMG patients.ConclusionThere was a low frequency of cases positive for AChR-Ab in the Chinese population. AChR-Ab titer was revealed as an independent risk factor for generalization. Low doses of prednisone could treat JMG effectively with few side effects.

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Section: Discussionsupporting

confidence: 92%

Clinical Characteristics of Juvenile Myasthenia Gravis in Southern China

Huang

Feng

et al. 2018

Front. Neurol.

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“…Female gender was considered as a risk factor in previous studies involving Asian juvenile-onset OMG [12,21] and Caucasian OMG [22]. However, the present study did not find any difference between the attribution of sex of pure OMG and converted OMG.…”

Section: Discussioncontrasting

confidence: 85%

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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“…The ethnic origin of the patients influences the acetylcholine receptor antibodies serum level and clinical outcome. Another adult MG study revealed that female sex, late onset and positivity for anti-acetylcholine receptor antibodies were associated with progression from ocular MG to generalized MG [14]. Some of seronegative patients had other antibodies, such as cortactin and lipoprotein receptor-related protein 4.…”

Section: Discussionmentioning

confidence: 99%

Correlation of anti-acetylcholine receptor antibody levels and long-term outcomes of juvenile myasthenia gravis in Taiwan: a case control study

Chou

et al. 2019

BMC Neurol

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Background Myasthenia gravis is the most common disease affecting the neuromuscular junction. The most common etiology among patients with juvenile myasthenia gravis is the production of antibodies against the acetylcholine receptor. However, the clinical outcome in relation to serum levels of anti-acetylcholine receptor antibodies in juvenile myasthenia gravis has rarely been discussed. We aimed to analyze the correlation between the presence of anti-acetylcholine receptor antibodies and outcome in juvenile myasthenia gravis. Methods Patients diagnosed with juvenile myasthenia gravis younger than of 20 years of age were retrospectively recruited from January 1995 to February 2017 in a tertiary referral medical center. According to the Myasthenia Gravis Foundation of America outcome scale, the primary outcome was complete symptom remission and cessation of medications for at least 1 year measured 2 years after diagnosis. Secondary outcome was complete symptom remission at the last outpatient clinic. Results A total of 54 patients were followed up for over 2 years. Nine patients (9/54, 16.7%) achieved complete remission without medication use at 2 years after diagnosis. Thirteen (24.1%) patients achieved complete remission during longer follow-up periods. Those with negative anti-acetylcholine receptor antibodies were more likely to achieve complete remission at 2 years (6/15 [40%] vs. 3/39 [7.7%], 95% Confidence interval [CI] 1.670 to 38.323) and at the last outpatient clinic follow-up (8/15 [53.3%] vs. 5/39 [12.8%], 95% CI 2.367 to 20.704). Thirteen patients with comorbid autoimmune thyroid diseases were older than those without disease (11.8 ± 5.8 years old vs. 8.0 ± 6.3 years old, 95% CI 0.018 to 7.33). Moreover, patients negative for anti-acetylcholine receptor antibodies were less likely comorbid with autoimmune thyroid disease (1/35 [2.9%] vs. 12/71 [16.9%], 95% CI 0.018 to 1.161). Conclusions Juvenile myasthenia gravis patients without anti-acetylcholine antibodies exhibited significantly increased complete remission rates and a reduced likelihood of comorbid autoimmune thyroid diseases compared with those with anti-acetylcholine receptor antibodies among Chinese. Electronic supplementary material The online version of this article (10.1186/s12883-019-1397-0) contains supplementary material, which is available to authorized users.

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Factors affecting outcome in ocular myasthenia gravis

Cited by 55 publications

References 43 publications

Clinical Characteristics of Juvenile Myasthenia Gravis in Southern China

Clinical Characteristics of Juvenile Myasthenia Gravis in Southern China

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

Correlation of anti-acetylcholine receptor antibody levels and long-term outcomes of juvenile myasthenia gravis in Taiwan: a case control study

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