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

2020 ◽  
Vol 83 (2) ◽  
pp. 182-188
Author(s):  
Xuelin Feng ◽  
Xiao Huan ◽  
Chong Yan ◽  
Jie Song ◽  
Jun Lu ◽  
...  

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%.

2020 ◽  
Vol 5 (7) ◽  
pp. 190-193
Author(s):  
Dr. Usha BR. ◽  
◽  
Dr. Nandhini K ◽  
Dr. Chaitra MC ◽  
◽  
...  

Myasthenia gravis (MG) is a rare autoimmune disorder affecting neuromuscular junction by muscleweakness. Myasthenia gravis can be generalized or localized as ocular myasthenia gravis. Casepresentation: We report an 8-year-old boy who presented with 10 days history of drooping of botheyelids and 8 days history of diplopia. Examination revealed bilateral ptosis. A diagnosis of JuvenileOcular Myasthenia gravis was made when symptoms improved with intramuscular Edrophoniumadministration. He was commenced on oral Neostigmine at a dose of 2mg/Kg/ day,4 hourly individed doses and is on regular follow up and had a good response. Conclusion: Ocular Myastheniagravis (OMG) is a rare disease in itself. A high index of suspicion is required in a juvenile as it iseven rarer.


2018 ◽  
Vol 79 (3-4) ◽  
pp. 113-117 ◽  
Author(s):  
Florencia Aguirre ◽  
Andres M. Villa

Background: We investigated the prognosis of patients with myasthenia gravis (MG) initially presenting with only ocular symptoms in an Argentinian population. Summary: We performed a retrospective analysis of 61 patients with MG with pure ocular involvement at onset. Generalized MG (gMG) developed in 73.7% of patients, while the rest only exhibited ocular symptoms throughout the course of the disease (ocular MG [oMG]). The AChR antibody (AChR-ab) was found in 81.4% of the gMG and 50% of the oMG cases. AChR-ab titers were also significantly higher in the gMG than in the oMG group. Patients with oMG more frequently required treatment only with acetylcholinesterase inhibitors and showed more remissions. Key Messages: Our study showed high generalization in patients with MG debuting with ocular symptoms. Patients with AChR seropositivity and higher AChR-ab titers had an increased risk for gMG, while patients with oMG showed lower corticosteroid requirements and a higher remission rate.


2017 ◽  
Vol 37 (2) ◽  
Author(s):  
Hong-Wei Yang ◽  
Ying-Xue Wang ◽  
Jie Bao ◽  
Shu-Hui Wang ◽  
Ping Lei ◽  
...  

The present study aims to explore the correlation of human leucocyte antigen (HLA)-DQ and tumour necrosis factor (TNF)-α gene polymorphisms with ocular myasthenia gravis (OMG) combined with thyroid-associated ophthalmopathy (TAO). From March 2009 to March 2015, 56 OMG patients complicated with TAO (OMG + TAO group), 134 patients diagnosed with OMG only (OMG group) and 236 healthy individuals (control group) were enrolled in the present study. PCR-sequence specific primer (PCR-SSP) was used for HLA-DQ genotyping and PCR-restriction fragment length polymorphism (PCR-RFLP) for TNF-α genotyping. ELISA kit was applied to detect acetylcholine receptor antibody (AchRAb) level and chemiluminescence immunoassay (CLIA) to measure thyroid-associated antibody (T-Ab) level. Logistic regression analysis was carried out to analyse the risk factors for OMG combined with TAO. DQA1*0103 showed lower frequency in the OMG group than in the control group. DQA1*0301 showed increased and DQB1*0601 showed decreased frequency in the OMG + TAO group. DQB1*0501 showed higher frequency in the OMG and OMG + TAO groups than in the control group. Patients carrying TNF-α -863C > A (CA + AA) might confront with greater risks of OMG combined with TAO. Frequency of DQA1*0103/*0301 and DQB1*0501/*0601, and TNF-α -863C > A, -238G > A and -308G > A were associated with the levels of AchRAb and T-Ab. TNF-α -863C > A (CA + AA) and high level of T-Ab were risk factors for OMG combined with TAO. Our results demonstrate that TNF-α -863 polymorphism is possibly correlated with the risk of OMG combined with TAO.


2016 ◽  
Vol 22 ◽  
pp. 277-278
Author(s):  
Lima Lawrence ◽  
Aleida Rodriguez ◽  
Tahira Yasmeen ◽  
Erin Drever

Author(s):  
Gordon Plant

Ocular myasthenia is not the most severe form of myasthenia but it might cause certain difficulties to diagnose. In this article, we shall discuss the clinical presentations of ocular myasthenia, its differential diagnostics, examinations and management. Keywords: ocular myasthenia gravis, diagnosis, examination, management.


2021 ◽  
Vol 118 ◽  
pp. 12-19
Author(s):  
Kristen S. Fisher ◽  
Jason Gill ◽  
Hannah F. Todd ◽  
Michael B. Yang ◽  
Michael A. Lopez ◽  
...  

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