Disease-specific measure of quality of life for myasthenia gravis

In 2000 a Task Force of the Myasthenia Gravis Foundation of America recommended development of a quality of life (QOL) measure specific for myasthenia gravis (MG). Extant investigations have relied solely on assessment of physical aspects of daily living in conceptualizing QOL, despite research that emphasizes the importance of including psychological factors. In the present study we developed a QOL questionnaire specific to MG (MG-QOL) that assesses both physical and psychological aspects of function. The MG-QOL questionnaire was administered as a secondary measure of efficacy in a recently completed prospective trial of mycophenolate mofetil involving 80 MG patients. Comparisons indicated that the MG-QOL performed better than a nondisease-specific measure of QOL, the SF-36, in demonstrating disease change as assessed by the primary measure, the Quantitative MG score (QMG). The MG-QOL correlated highly with the SF-36, and demonstrated stronger associations with independent physical ability ratings. Results from this study support the use of this new measure of QOL, both clinically and in treatment trials of MG.     

Quality of life and well-being of patients with myasthenia gravis

The cardinal symptom of myasthenia gravis (MG) is weakness of voluntary muscles, a feature that may restrict full participation in life activities. In turn, such limitations may negatively affect quality of life (QOL) and well-being among individuals with the disease. In the present study, we administered a measure of QOL to 27 patients with generalized MG. Results revealed that functional status was negatively impacted in the domains of physical functioning, energy, and general health. However, a clinically meaningful difference was evident only on perceived ability to accomplish physical tasks. The results suggest that although MG requires accommodations in physical activities, general QOL and well-being does not differ markedly from the general population.     

Quality of life in 188 patients with myasthenia gravis in China

Myasthenia gravis (MG) is a kind of chronic autoimmune disease which can weaken patients' motor function and, furthermore, produce negative impact on the health-related quality of life (HRQoL). The primary purpose of this research was to evaluate factors that might affect the HRQoL of MG patients. A cross-sectional clinical research was carried out including 188 successive patients with MG. Myasthenia Gravis Foundation of America (MGFA) classification and Quantitative Myasthenia Gravis (QMG) score were applied to assess the severity of the disease. The Medical Outcome Survey 36-Item Short-Form Health Survey (SF-36) was used to estimate the HRQoL. Hamilton Depression Rating Scale (HDRS) and Hamilton Anxiety Rating Scale (HARS) were utilized to measure the depression and anxiety symptom. Factors may influence the HRQoL of MG patients include age, educational level, occupation, the situation of the thymus, the type of MG and generalized myasthenia gravis (GMG), the severity of the disease and the psychological disorder. Higher QMG and HARS scores were two significant factors that can prognosticate lower Physical Composite Score (PCS) and Mental Composite Score (MCS), while older age was just a significant factor which has prognostic value for lower PCS. The results of this research may have a potential guiding significance for the clinical treatment strategy and improve the quality of life in patients with MG consequently. In addition to the treatment of physical symptoms, the psychological symptoms such as anxiety and depression should be concerned as well.     

The MG-QOL15 for following the health-related quality of life of patients with myasthenia gravis

The MG-QOL15 is helpful in informing the clinician about the patient's perception of the extent of and dissatisfaction with myasthenia gravis (MG)-related dysfunction. The aims of this study were to determine the usefulness of the MG-QOL15 for following individuals with MG and to guide clinical researchers who plan to use the MG-QOL15. We assessed sensitivity and specificity for detecting clinical change and evaluated test-retest reliability. Sensitivities and specificities of various cut-points of change in scores are presented. Also presented are means and standard deviations of MG-QOL15 scores for all patients and for subgroups of patients. The test-retest reliability coefficient was 98.6%. The MG-QOL15 has an acceptable longitudinal construct validity. We consider this instrument to be most useful for informing the clinician about the patient's perception and tolerance of MG-related dysfunction. More objective measures, such as the MG Composite, should also be used to follow disease severity.     

188例重症肌无力患者生活质量研究

目的分析重症肌无力(MG)患者的生活质量。方法纳入2013-03-2014-06在唐都医院神经内科就诊的MG患者188例,应用重症肌无力量化评分(QMGs)评估患者病情严重程度,采用36项简明健康状况调查表(SF-36)评估患者生活质量,采用汉密尔顿抑郁量表(HDRS)和汉密尔顿焦虑量表(HARS)评估抑郁和焦虑症状。比较不同教育水平、职业、眼肌型重症肌无力(ocular MG,OMG)症状、胸腺情况等患者间SF-36评分的差异,并对QMG评分、年龄、HARS和HDRS得分与SF-36两项复合得分进行多元线性回归分析。结果高级教育组在躯体疼痛项得分高于初级教育组(P0.05),学生组在生理机能项(P0.05)和生理角色功能项(P0.05)得分均高于脑力劳动组,学生组在生理角色功能项得分亦高于体力劳动组(P0.05);学生组在生理复合得分(PCS)项得分高于按照职业分组的其他3组(均P0.05);OMG组在精神复合得分(MCS)项得分高于全身型重症肌无力(generalized MG,GMG)组(P0.05)。较高的QMGs、HARS得分和高龄可以预测较低的PCS得分,较高的QMGs和HARS得分可预测较低的MCS得分。结论影响MG患者生活质量的因素包括年龄、教育水平、职业、胸腺情况、MG的类型和GMG的类型、疾病的严重程度和心理障碍。较高的QMGs和HARS得分可以预测较低的PCS和MCS得分,年龄大可预测较低的PCS得分。     

Construct and concurrent validation of the MG‐QOL15 in the practice setting

Health‐related quality of life (HRQOL) estimates can play an important role in patient care by providing information about the patient's perception of impairment and disability and the degree to which the patient tolerates disease manifestations. The 15‐item myasthenia gravis quality of life scale (MG‐QOL15) is an HRQOL evaluative instrument specific to patients with myasthenia gravis (MG) that was designed to be easy to administer and interpret. In this multicenter study we demonstrate the construct validity of the MG‐QOL15 in the practice setting. To assess the construct validity, score distributions were examined for test items in different MG patient groups that represent the clinical spectrum of the disease. For example, patients in remission more frequently scored test items as normal than did patients in other groups. Patients with lower (better) MG composite scores also more frequently scored items as normal than did patients with higher (worse) scores. There was also appropriate correlation between the MG‐QOL15 and the other MG‐specific scales studied. The study findings shed light on what troubles MG patients. The MG‐QOL15 has construct validity in the clinical practice setting and represents an efficient and valuable tool for assessing HRQOL for patients with MG. Muscle Nerve, 2010     

Predicting the course of myasthenic weakness following double filtration plasmapheresis

OBJECTIVE: To evaluate the clinical course of patients with myasthenia gravis (MG) up to 3 months after double filtration plasmapheresis (DFP). MATERIAL AND METHODS: We recorded the MG score and measured the level of acetylcholine receptor antibody (AchRAb) at baseline and day 1 (D1), week 1 (W1), 1 month (M1), 2 (M2) and 3 months (M3) after DFP in 16 MG patients. Based on the difference in score during follow-up, we divided our patients into clinical improvement (CI) and clinical worsening (CW) groups. RESULTS: The MG score decreased in all courses from a mean of 8.1 at baseline to 5.6 at D1, and to 4.7, 4.0, 3.8, and 3.7 at W1, M1, M2, and M3, respectively. In the CW group, AchRAb levels were significantly higher at M1 (P = 0.022). The AchRAb level at W1 correlated significantly with the MG score at M3 (P = 0.027) and the changes of MG score from W1 to M1 (P = 0.029). The ratio of AchRAb levels of M1 to W1 correlated well with MG score at W1 (P = 0.032), at M3 (P = 0.001), and the changes of MG score from W1 to M1 (P = 0.004). CONCLUSION: Excessive rebounds of AchRAb level at W1 may suggest clinical worsening and further increases in AchRAb level at M1 predict poorer outcome after DFP.     

Validation of Serbian version of the disease‐specific myasthenia gravis questionnaire

Basta I, Pekmezovi? T, Padua L, Stojanovi? V, Stevi? Z, Nikoli? A, Peri? S, Lavrni? D. Validation of Serbian version of the disease‐specific myasthenia gravis questionnaire.
Acta Neurol Scand: 2010: 122: 110–114.
© 2009 The Authors Journal compilation © 2009 Blackwell Munksgaard. Aim – The aim of this study was to validate translated and cross‐cultural adapted Italian version of myasthenia gravis‐specific questionnaire (MGQ) in Serbian MG patients. Materials and Methods – The questionnaire was validated in 140 consecutive MG patients from Belgrade. In each patient association between the total MGQ score and form and severity of the disease was determined. Also, correlation between regional domain scores of MGQ and main clinical findings according to Besinger’s clinical score was analyzed. Results – Patients’ participation in the assessment was satisfactory with excellent internal consistency and reproducibility. Total MGQ score, as well as domain scores, correlated with highly significant inverse relationship with the disease severity and clinical status of patients at the moment of completing the questionnaire. Furthermore, the bulbar domain of the questionnaire appeared more specific and sensitive than clinical history and examination. Conclusion – We concluded that the Serbian version of the MGQ may be useful as a measure of clinical outcome in patients with MG.     

International clinimetric evaluation of the MG‐QOL15, resulting in slight revision and subsequent validation of the MG‐QOL15r

Introduction: The MG‐QOL15 is a validated, health‐related quality of life (HRQOL) measure for myasthenia gravis (MG). Widespread use of the scale gave us the opportunity to further analyze its clinimetric properties. Methods: We first performed Rasch analysis on >1,300 15‐item Myasthenia Gravis Quality of Life scale (MG‐QOL15) completed surveys. Results were discussed during a conference call with specialists and biostatisticians. We decided to revise 3 items and prospectively evaluate the revised scale (MG‐QOL15r) using either 3, 4, or 5 responses. Rasch analysis was then performed on >1,300 MG‐QOL15r scales. Results: The MGQOL15r performed slightly better than the MG‐QOL15. The 3‐response option MG‐QOL15r demonstrated better clinimetric properties than the 4‐ or 5‐option scales. Relative distributions of item and person location estimates showed good coverage of disease severity. Conclusions: The MG‐QOL15r is now the preferred HRQOL instrument for MG because of improved clinimetrics and ease of use. This revision does not negate previous studies or interpretations of results using the MG‐QOL15. Muscle Nerve 54 : 1015–1022, 2016     

The modified rankin scale to assess disability in myasthenia gravis: Comparing with other tools

Introduction: We explored the modified Rankin scale (mRS) as a tool to quantitate disability in myasthenia gravis (MG). Our aim was to correlate patients' perception of their disability with that of the care provider and determine its relationship with other MG‐related scores. Methods: We evaluated 107 MG patients at 2 neurological centers. Patients were assessed over the telephone before and after clinic visits using the 15‐item Myasthenia Gravis Quality‐of‐Life index (MG‐QOL15) and mRS. At the clinic, patients were assessed using the MG‐QOL15, MG Composite (MGC), and mRS. Results: The MG‐QOL15 correlated with the MGC, mRS, and assessors' scores of patients. Assessors' perception of disease burden was in line with that of the patients' scoring. MG‐QOL15 scores obtained over the telephone were consistent with those obtained in the clinic. Scores were generally higher in patients receiving steroids at >5 mg/day and in those receiving or seeking benefits. Conclusion: The MG‐QOL15 and mRS are useful for estimating disability in MG. Muscle Nerve 50 : 501–507, 2014     

A health-related quality of life questionnaire for multiple sclerosis Patients

OBJECTIVE: The construction of a brief, valid and reliable HRQoL questionnaire for use in multiple sclerosis patients based on generic and disease-specific HRQoL measures. MATERIAL AND METHODS: The Medical Outcomes Study 36-item Short Form Health Survey (SF-36), COOP/ WONCA Charts, and Disability & Impact Profile (DIP) were used in a longitudinal study in 162 patients with multiple sclerosis. RESULTS: Factor analyses identified 2 underlying dimensions of HRQoL, relating to "physical functioning" and "psychological functioning". Selection of the 3 highest loading reliable scales on each factor resulted in a final questionnaire consisting of 3 scales of the SF-36 and 3 scales of the DIP. In total 40 items were selected; completion time is about 10 min. CONCLUSION: The final questionnaire adequately measured 2 dimensions of HRQoL. The length of the questionnaire is acceptable for patients with MS in view of respondent burden.     

Correlation between clinical/neurophysiological findings and quality of life in Charcot-Marie-Tooth type 1A

Abstract   Quality of life (QoL), as defined by the Short Form 36, has previously been shown to be abnormal in patients with Charcot-Marie-Tooth disease (CMT), both for Physical Composite Scores (PCS) and Mental Composite Scores (MCS). We have now extended these observations in a multicenter evaluation of 89 patients with Charcot-Marie-Tooth disease type 1A, the most common form of CMT. Both the PCS and MCS were abnormal also in this cohort, compared with the Italian population at large. In particular, the ability to ambulate independently as well as toe and heel walk correlated well with QoL measures in our patients.     

Assessing quality of life for adolescents in Taiwan

This study was to evaluate the psychometric properties of the Taiwanese quality of life questionnaire for adolescents and the factors affecting the quality of life of Taiwanese adolescents. The survey involved 5538 junior high school students, aged 13-15 years. An initial 90-item questionnaire was shortened to 38 items by means of principal component analyses. Quality of life assessment involved seven factors: family, residential environment, personal competence, social relationships, physical appearance, psychological well-being, and pain. The rate of missing data was low. The Cronbach alpha coefficient remained above the 0.75 threshold criterion for the global scale and seven subdomain scales. A lower quality of life score was evident for female adolescents in higher grades in school, those living with a single parent or other relatives, and those living in rural areas. This 38-item questionnaire should serve as a reliable tool for future studies.     

Validation of the Swedish version of the disease-specific Myasthenia Gravis Questionnaire

Abstract The aim of this study was to translate and validate the disease-specific patient-derived Myasthenia Gravis (MG) Questionnaire to enable use among Swedish MG patients. The original Italian version of the MG Questionnaire (MGQ) was translated into Swedish and transculturally adapted. The validity and reliability was tested on 48 Swedish MG patients. We correlated MGQ scores with disease severity and with the Swedish version of the Short-Form 36-item general health survey (SF-36). Reproducibility was assessed on 18 clinically stable MG patients. A significant correlation regarding the MGQ scores was seen when correlated with physical scores of the SF-36 and the overall clinical status. Internal consistency and reproducibility was excellent. We conclude that the evaluation capacities of the Swedish MGQ are equivalent to those of the original Italian version of the MGQ. The questionnaire was successfully validated as an outcome measure also for Swedish MG patients, which is important for international multicentre clinical trials.     

Translation and validation of the 15‐item Myasthenia Gravis Quality of life scale in Dutch

Introduction: The 15‐item Myasthenia Gravis Quality of Life (MG‐QOL15) scale has been developed to assess the health‐related quality of life of patients with myasthenia gravis (MG). The aim of this study was to translate the original English version into Dutch and to test the test–retest reliability and construct validity. Methods: Fifty patients with MG were included. Test–retest reliability and internal consistency were assessed using the intraclass correlation coefficient (ICC) and the Cronbach α. Construct validity was assessed by testing 5 predefined hypotheses. Results: A good test–retest reliability was confirmed with an ICC of 0.866. The Cronbach α was 0.93. The predefined hypotheses were confirmed in 80% of cases, which points to good construct validity. Discussion: The Dutch MG‐QOL15 has good test–retest reliability and good construct validity. It can be used for research in a Dutch‐speaking population. It is also suitable for monitoring individual patients in clinical practice. Muscle Nerve 57 : 206–211, 2018