Performance of individual items of the quantitative myasthenia gravis score

Barnett, Carolina; Bril, Vera; Davis, Aileen M.

doi:10.1016/j.nmd.2013.02.008

Cited by 19 publications

(31 citation statements)

References 19 publications

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“…We also confirmed the concordance between the QMGS and the MGFA classification of disease severity, Class I to III. It is important to acknowledge that our study group did not include patients in the MGFA Class IV and V. Barnett et al 25 similarly confirmed the sensitivity of the QMGS to differentiate disease severity among patients with MG, as well as to quantify treatment effectiveness. In particular, Barnett et al provided evidence of the discriminative value of the timed items of the QMGS, evaluating upper and lower limb function and neck endurance.…”

Section: Discussionmentioning

confidence: 62%

Quantitative Myasthenia Gravis Score: a Brazilian multicenter study for translation, cultural adaptation and validation

Oliveira

Valério

Cavalcante

et al. 2017

Arq. Neuro-Psiquiatr.

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Objective: To perform the translation, cross-cultural adaptation and validation of the Quantitative Myasthenia Gravis Score (QMGS) to Brazilian Portuguese in accordance with international ethical standards. Methods: The following steps were taken: (1) implementation of the translation protocol and transcultural adaptation, (2) validation of the adapted content, and (3) assessment of reliability. To check intraand inter-observer reproducibility, each patient underwent two interviews with interviewer-A and one with B. The QMGS was compared to the Myasthenia Gravis Composite Scale and Myasthenia-specific Quality of Life Questionnaire. Results: Our study group consisted of 30 patients, with a mean age of 47.6±11.4 years and a mean duration of illness of 11.33±8.49 years. Correlation between the QMGS and MGC was very strong (r = 0.928; p < 0.001) and substantial between the QMGS and MG-QOL 15 (r = 0.737; p < 0.001). Conclusion: The Brazilian Portuguese translation, and validation of the QMGS was successfully performed.Keywords: myasthenia gravis; cross-cultural comparison; translations; validation studies. RESUMOObjetivo: O objetivo foi realizar a tradução e validação do teste quantitativo para Miastenia Gravis (QMGS) para Português do Brasil, de acordo com as diretrizes internacionais. Métodos: Foram realizadas as etapas de implementação do protocolo de tradução e adaptação transcultural, validação do conteúdo adaptado e avaliação da confiabilidade. Para verificar a reprodutibilidade intra e inter-observador cada paciente foi submetido a duas entrevistas por um entrevistador-A e um B. O QMGS foi comparado ao MG Composite Scale e Myasthenia-specific Quality of Life Questionnaire. Resultados: O estudo inclui 30 pacientes, com a média de idade de 47,6±11,4 anos e tempo médio de doença de 11,33±8,49 anos. A correlação entre QMGS e MGC apresentou-se muito forte (r = 0,928; p < 0,001) e substancial entre QMGS e MG-QOL 15 (r = 0,737; p < 0,001). Conclusão: A tradução, e validação do QMGS para o português do Brasil foi realizada com sucesso.Palavras-chave: miastenia gravis; comparação transcultural; traduções; estudos de validação.Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disorder characterized by weakness of the skeletal muscles 1 . In approximately 85% to 90% of patients with MG, antibodies against nicotinic acetylcholine receptors are identified in the neuromuscular junctions, with a smaller group of patients having autoantibodies against muscle-specific kinase 2 , low-density lipoprotein-related protein 4, and even against agrin 3,4 .

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Section: Discussionmentioning

confidence: 62%

Quantitative Myasthenia Gravis Score: a Brazilian multicenter study for translation, cultural adaptation and validation

Oliveira

Valério

Cavalcante

et al. 2017

Arq. Neuro-Psiquiatr.

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“…Items were chosen based on correlations with quality of life scales and clinical change, such that some endurance items were less responsive and thus were excluded from the final measure. Endurance tests of the arms, legs and neck have been used in some measures as a marker of fatigability, demonstrating responsiveness in clinical trials [27], [28], however, they can be time consuming for routine clinical assessments. Patient reported outcomes asking specifically about impairments triggered or worsened with activity or through the day might be more feasible.…”

Section: Discussionmentioning

confidence: 99%

A Conceptual Framework for Evaluating Impairments in Myasthenia Gravis

et al. 2014

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BackgroundMyasthenia gravis is characterized by weakness and fatigability of different muscle groups, including ocular, bulbar and the limbs. Therefore, a measure of disease severity at the impairment level in myasthenia needs to reflect all the relevant impairments, as well as their variations with activity and fatigue. We conducted a qualitative study of patients with myasthenia, to explore their experiences and related impairments, aimed at developing a conceptual framework of disease severity at the impairment level in myasthenia gravis.MethodsTwenty patients representing the spectrum of disease participated in semi-structured interviews. Interviews were recorded and the transcripts were analyzed by content analysis using an inductive approach with line-by-line open coding. Themes were generated from these codes.ResultsTwo main themes were identified: the severity of the impairments and fatigability (i.e., triggering or worsening of an impairment with activity). The impairments were further classified within body regions (ocular, bulbar and axial/limbs). Fatigability was described as a phenomenon affecting the whole body but also affecting specific impairments, and was associated with fluctuation of the symptoms. Patients were concerned that clinical examination at a single point in time might not reflect their true clinical state due to fatigability and fluctuations in severity.ConclusionsThis conceptual framework reflects the relevance of both severity and fatigability in understanding impairment-based disease severity in myasthenia. This framework could inform the development of impairment measures in myasthenia gravis.

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“…A detailed evaluation of the ability of components of the QMGS to accurately measure MG disease activity shows that bulbar and grip strength items do not perform well, but timed items, such as limb activation, are useful [35]. Marked floor effects are observed for bulbar items, vital capacity and grip strength, a surprising result, but limiting the value of these items in the QMGS.…”

Section: Outcome Measuresmentioning

confidence: 99%

“…The items showing most responsiveness are timed activation of upper eyelid elevation (to elicit ptosis), and timed activation of arm, leg, and neck muscles. Furthermore, new normal ranges of activation times for these items have been proposed based on updated normative data [35]. The more reliable normative ranges are 90 s for leg lift, 180 s for arm abduction, and 40 s for neck lift [35].…”

Section: Outcome Measuresmentioning

confidence: 99%

“…Furthermore, new normal ranges of activation times for these items have been proposed based on updated normative data [35]. The more reliable normative ranges are 90 s for leg lift, 180 s for arm abduction, and 40 s for neck lift [35]. Since the limbs correlate strongly with each other, testing of a single side is likely sufficient and further reduces the time necessary for performing the QMGS in the clinic.…”

Section: Outcome Measuresmentioning

confidence: 99%

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Intravenous immunoglobulin as treatment for myasthenia gravis: current evidence and outcomes

Alabdali

Barnett

Katzberg

et al. 2014

Expert Review of Clinical Immunology

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We examined the current evidence for the efficacy of IV immunoglobulin (IVIG) in myasthenia gravis (MG) and the outcomes used to demonstrate this efficacy. There is class 1 evidence for the use of short-term IVIG in MG patients worsening MG and also good evidence for IVIG use in myasthenic crisis. For long-term maintenance therapy, controlled studies are lacking and the evidence is limited to class III retrospective studies. The clinical scales, serological, electrophysiological, and patient-reported quality of life outcomes with IVIG have been assessed. At this time, the quantitative myasthenia gravis score, a functional scale, remains the preferable outcome measure as it has demonstrated responsiveness in the clinical trial setting, but a scale incorporating patient-reported outcomes and the patients complaint of fatigue is likely to be preferable. The MG-composite is such a scale, but has measurement limitations that may reduce its sensitivity. Across trials, IVIG has generally been well tolerated.

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Performance of individual items of the quantitative myasthenia gravis score

Cited by 19 publications

References 19 publications

Quantitative Myasthenia Gravis Score: a Brazilian multicenter study for translation, cultural adaptation and validation

Quantitative Myasthenia Gravis Score: a Brazilian multicenter study for translation, cultural adaptation and validation

A Conceptual Framework for Evaluating Impairments in Myasthenia Gravis

Intravenous immunoglobulin as treatment for myasthenia gravis: current evidence and outcomes

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