Late-onset generalized myasthenia gravis: clinical features, treatment, and outcome

Çelik, Şenay Yıldız; Durmuş, Hacer; Yılmaz, Vuslat; Direskeneli, Güher Saruhan; Parman, Yeşim; Oflazer, Piraye; Deymeer, Feza

doi:10.1007/s13760-019-01252-x

Cited by 20 publications

(9 citation statements)

References 23 publications

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“…The majority of LOMG had ocular phenotype. The presence of neither anti-titin nor anti-MuSK antibodies points to an unfavorable outcome [ 32 ]. Still the diversity in peripheral immune profile between EOMG and LOMG remained unelucidated.…”

Section: Discussionmentioning

confidence: 99%

Increased serum IL-2, IL-4, IL-5 and IL-12p70 levels in AChR subtype generalized myasthenia gravis

et al. 2022

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Background Myasthenia gravis (MG) is an autoimmune disorder affecting neuromuscular junctions. Cytokines play important roles in facilitating the immune response and augmenting the pathogenic antibody production. The current study aims to sensitively characterize the serum levels of cytokines with very low concentration in generalized MG (gMG). Methods Using ultrasensitive single-molecule arrays (SIMOA), we measured serum IL-2, IL-4, IL-5 and IL-12p70 in 228 participants including 152 immunotherapy-naïve anti-acetylcholine receptor (AChR) subtype gMG from Huashan MG registry and 76 age-matched healthy controls. Subgroup analysis was then performed by stratifying patients according to the onset ages, MGFA classification, disease duration at baseline. Results Serum IL-2, IL-4, IL-5 and IL-12p70 levels were significantly elevated in gMG compared to controls (0.179 pg/mL versus 0.011 pg/mL, P < 0.0001; 0.029 pg/mL versus 0.018 pg/mL, P = 0.0259; 0.215 pg/mL versus 0.143 pg/mL, P = 0.0007; 0.132 pg/mL versus 0.118 pg/mL, P = 0.0401). Subgroup analysis revealed that IL-2 levels were slightly elevated in gMG with MGFA II compared to MGFA III/IV (0.195 pg/mL versus 0.160 pg/mL, P = 0.022), as well as elevated levels of IL-2 (0.220 pg/mL versus 0.159 pg/mL, P = 0.0002) and IL-5 (0.251 pg/mL versus 0.181 pg/mL, P = 0.004) in late-onset gMG compared with the early-onset gMG. gMG patients with a long duration had a significant increased serum IL-12p70 than those with a short duration (0.163 pg/mL versus 0.120 pg/mL, P = 0.011). Conclusion Serum IL-2, IL-4, IL-5 and IL-12p70 levels were increased in AChR subtype gMG using ultrasensitive measurement. Serum cytokines with very low concentrations may provide as potential biomarkers in stratifying gMG patients in future prospective cohort studies.

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Section: Discussionmentioning

confidence: 99%

Increased serum IL-2, IL-4, IL-5 and IL-12p70 levels in AChR subtype generalized myasthenia gravis

et al. 2022

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“…The scale, originally in English, is available in Polish, Italian, Arabic, and Korean translations (Alanazy et al, 2019; Lee et al, 2017; Raggi et al, 2017; Rozmilowska et al, 2018). Although the number of clinical trials is increasing in MG Turkish population (Salci et al, 2018; Tascilar et al, 2017; Yildiz Celik et al, 2019), the Turkish version of MG-ADL has not been yet prepared. Therefore, a Turkish version of the scale is necessary both for international standardization in clinical trials and adequate evaluation of the patients.…”

Section: Introductionmentioning

confidence: 99%

Reliability and Validity of Turkish Myasthenia Gravis-Activities of Daily Living Scale

Karanfil

Salcı

Fil-Balkan

et al. 2020

OTJR: Occupation, Participation and Health

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Linguistic, reliable, and valid secondary efficacy measures are important in clinical settings and studies. The aim of the study is to report test–retest reliability and construct validity of Turkish version of Myasthenia Gravis-Activities of Daily Living Scale (MG-ADL-T) in Myasthenia Gravis (MG) patients. Fifty-two ocular and generalized individuals with MG, applying to rehabilitation center, were included in the study. MG-ADL-T, MG quality-of-life questionnaire (MG-QoL), MG composite (MGC), quantitative MG score (QMGS), and pulmonary function test were administered. Reliability was assessed with intraclass correlation coefficient (ICC) and Cronbach’s alpha. Spearman correlation test and receiver operating characteristic (ROC) analysis were performed for construct validity. MG-ADL-T had fair internal consistency (Cronbach’s α = .67), excellent test–retest reliability (ICC = 0.96) and moderate construct validity (MG-QoL, r = 0.59; QMGS, r = .58; MGC, r = .68). MG-ADL, a unique scale that evaluates activities of daily living (ADL), has good test–retest reliability and construct validity in Turkish MG patients.

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“…In this study, a mild male predominance in a small sample of Chinese patients, together with distinct male-to-female ratios in OMG (2.3:1) and GMG (1:1.3) groups further indicate potential gender predominance dependent on clinical subtypes ( 23 ). Previous studies enrolling elderly onset MG showed a high prevalence of AChR antibodies ranging from 80% to nearly 93% ( 4 , 6 , 19 , 21 ). Similarly, our study showed a comparable positive rate of 85.7%.…”

Section: Discussionmentioning

confidence: 99%

Lack of Immunotherapy as the Only Predictor of Secondary Generalization in Very-Late-Onset Myasthenia Gravis With Pure Ocular Onset

Zhao

Ding

et al. 2022

Front. Neurol.

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During the past two decades, an increasing number of patients with very-late-onset myasthenia gravis (v-LOMG) with an onset age of 65 years or older have been identified. However, few studies explore the predictors of secondary generalization in patients with v-LOMG with pure ocular onset. In this retrospective cohort study, 69 patients with v-LOMG were divided into ocular MG (OMG) and generalized MG (GMG), and the clinical characteristics and outcomes were compared. Cox regression analysis was performed to explore the predictors of generalization. The average onset age of the study population was 73.1 ± 4.2 years and the median disease duration was 48.0 months (interquartile range, 32.5–64.5 months). Serum acetylcholine receptor (AChR) antibody was detected in up to 86% of patients and concomitant diseases in approximately half of the patients. Male predominance was seen in OMG group while female predominance in GMG group (p = 0.043). Patients with OMG showed a lower positive rate of repetitive nerve stimulation (RNS) than those with GMG (p = 0.014), and favorable outcomes were obtained in more patients with OMG than those with GMG (p < 0.001). Of the 51 patients with pure ocular onset, 25 (49.0%) underwent secondary generalization. A higher probability of generalization was found in patients with positive RNS results and without immunotherapy (p = 0.018 and <0.001). Upon Cox regression analysis, immunotherapy was negatively associated with secondary generalization [HR (hazard ratio) 0.077, 95%CI [0.024–0.247], p < 0.001]. Altogether, compared to the patients with very-late-onset GMG, the counterparts with OMG exhibit a significantly higher female predominance and a lower positive rate of RNS tests, especially on facial and accessory nerves. Lack of immunotherapy is the only predictor of secondary generalization in those with pure ocular onset.

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Late-onset generalized myasthenia gravis: clinical features, treatment, and outcome

Cited by 20 publications

References 23 publications

Increased serum IL-2, IL-4, IL-5 and IL-12p70 levels in AChR subtype generalized myasthenia gravis

Increased serum IL-2, IL-4, IL-5 and IL-12p70 levels in AChR subtype generalized myasthenia gravis

Reliability and Validity of Turkish Myasthenia Gravis-Activities of Daily Living Scale

Lack of Immunotherapy as the Only Predictor of Secondary Generalization in Very-Late-Onset Myasthenia Gravis With Pure Ocular Onset

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