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Journal of Clinical Neurology (Seoul, Korea) logoLink to Journal of Clinical Neurology (Seoul, Korea)
. 2022 May 20;18(5):514–521. doi: 10.3988/jcn.2022.18.5.514

Validation of the Individualized Neuromuscular Quality of Life Questionnaire in Korean Patients With Genetic Neuromuscular Diseases

Hee Jo Han a, Seung-Ah Lee a, Young-Chul Choi a, Michael R Rose b, Hyung Jun Park a,
PMCID: PMC9444564  PMID: 36062768

Abstract

Background and Purpose

The Individualized Neuromuscular Quality of Life questionnaire (INQoL) is a widely used measure of the quality of life in patients with neuromuscular diseases. The purpose of this study was to translate and validate the Korean version of INQoL in Korean patients with neuromuscular diseases.

Methods

We translated the original INQoL version into Korean while applying appropriate language adaptations. The internal consistency, known-group validity, and test-retest reliability were also assessed. Construct validity was measured using the modified Rankin Scale (mRS) score and the manual muscle testing (MMT)-sum score based on the Medical Research Council scale, and concurrent validity was measured using the 36-item Short Form Survey (SF-36) questionnaire.

Results

This study enrolled 193 patients. The coefficients for internal consistency (Cronbach’s α=0.805 to 0.987) and test-retest reliability (Spearman’s ρ=0.453 to 0.886) were adequately high for all subscales except in the ‘treatment effects’ dimension. INQoL subscales other than those for locking, droopy eyelids, double vision, and swallowing difficulties were significantly associated with their relevant SF-36 domains (Spearman’s ρ=-0.274 to -0.833). Functional status and muscle strength were most strongly associated with independence (Spearman’s ρ=0.753 and p<0.001 for mRS score, Spearman’s ρ=-0.741 and p<0.001 for MMT-sum score).

Conclusions

The Korean INQoL is a reliable and validated measurement tool for Korean patients with neuromuscular diseases.

Keywords: neuromuscular diseases, quality of life, validation study

INTRODUCTION

Genetic neuromuscular diseases are heterogeneous groups of genetic diseases that primarily affect the muscles, peripheral nerves, or neuromuscular junctions. Approximately 600 causative genes of genetic neuromuscular diseases have been identified.1 These neuromuscular diseases share the common feature of motor weakness, but the age at symptom onset, rate of disease progression, and pattern of involvement vary widely according to the genetic causes. Many studies have investigated the pathogenic mechanisms and therapeutic candidates of genetic neuromuscular diseases, but a cure remains elusive. There is also an increasing need for reliable biomarkers that assess the severity of disability.

The health-related quality of life (HRQoL) is defined as the perceived physical and mental health of an individual or group.2 The concept of HRQoL is particularly emphasized in patients with chronic diseases such as genetic neuromuscular diseases, because most of these patients have disabilities and require lifelong management. Therefore, improving HRQoL is considered one of the most important therapeutic goals in patients with chronic diseases. HRQoL is often used as an outcome of clinical trials for determining therapeutic benefits.3 HRQoL is usually measured by patient-reported questionnaires, which are classified into two categories: generic and disease-specific. Disease-specific questionnaires are developed for patients with a specific disease, which allows the HRQoL to be measured more precisely since these questionnaires are better able to reflect disease characteristics such as muscle weakness, pain, or spasticity.

The Individualized Neuromuscular Quality of Life questionnaire (INQoL) is a neuromuscular disease-specific questionnaire that was originally developed in the United Kingdom.4 It is currently used to assess the disease burden in patients with various neuromuscular diseases.5,6 INQoL has been translated and validated in several countries, including the United States, Italy, the Netherlands, Serbia, Spain, and Japan.7,8,9,10,11,12

This study aimed to translate INQoL into Korean and validate the new version for Korean patients with neuromuscular diseases.

METHODS

INQoL

INQoL (version 2.0) consists of 45 items within 11 subscales, which are classified into 3 dimensions.12 The ‘symptoms’ dimension comprises seven subscales focusing on specific symptoms of neuromuscular diseases: weakness, locking, pain, fatigue, droopy eyelids, double vision, and swallowing difficulties. The ‘life domains’ dimension comprises five subscales that relate to how much their muscle condition affects the personal lives of patients: activities, independence, social relationships, emotions, and body image. The ‘treatment effects’ dimension comprises two subscales asking about the positive and negative effects of treatment: perceived treatment effects and expected treatment effects. The quality of life (QoL) score is calculated from the items in the ‘life domains’ dimension, and represents the level of the patient’s overall HRQoL.

All INQoL items are scored on a 7-point Likert scale from 0 to 6 or from 1 to 7. A higher subscale score indicates a greater impact of the disease on the patient’s symptoms or life domains, and a higher QoL score indicates a worse overall HRQoL of the patient.

Translation and cultural adaptation

INQoL was translated in accordance with previously reported guidelines.13,14 Prior to initiating this study, we received approval from the MAPI Research Trust (Lyon), a nonprofit research institute hosting INQoL. We developed the Korean version of INQoL (version 2.0) according to the MAPI guidelines for linguistic validation of a clinical outcome assessment. The forward translation (from English to Korean) of INQoL was executed independently by two neurologists. One neurologist was a researcher who knew the concepts of INQoL, and the other was naïve about them. Differences between the two translations were reconciled through discussion by these two translators plus a senior neurologist.

The draft version of the Korean INQoL was then translated into English by a native English speaker who was bilingual in Korean. The backward-translated version of the questionnaire was reviewed by the forward translators and the original author (M.R. Rose). After several discussions about discordance between the original version and the back-translated version, the revised version of the Korean INQoL was produced. The revised version of the questionnaire was tested on ten patients before making appropriate modifications to produce the final version of the questionnaire.

Patient recruitment and data collection

Patients were recruited at Gangnam Severance Hospital. The inclusion criteria were 1) having a genetic neuromuscular disease, 2) aged >19 years, and 3) able to communicate in Korean. Our study included patients who were able to write or speak despite receiving mechanical ventilation. However, patients who had cognitive impairment or drowsiness were excluded due to the patient-reported nature of the questionnaire. We obtained information regarding sex, age at symptom onset, age at the examination, muscle strength, and genetic causes by reviewing the medical records of the patients and also interviewing them. Muscle strength was assessed using manual muscle testing (MMT) scores based on the Medical Research Council scale (0, 1, 2, 3-, 3, 3+, 4-, 4, 4+, 5-, and 5), which were then converted into an 11-point scale from 0 to 10.15 The MMT-sum score was the sum of the following 30 strength values: neck flexion, neck extension, and bilateral shoulder abduction, elbow extension, elbow flexion, wrist extension, wrist flexion, finger extension, finger flexion, hip flexion, hip extension, hip abduction, knee extension, knee flexion, ankle dorsiflexion, and ankle plantarflexion. The functional status of the patients was assessed using the modified Rankin Scale (mRS).

The patients were also assessed using the 36-item Short Form Survey (SF-36) for concurrent validation of INQoL. The SF-36 is the most commonly used generic measurement of HRQoL. The Korean version of the SF-36 questionnaire was previously validated in a general population.16,17 All assessments were performed over a 1-day period in the hospital. Patients who visited the hospital twice within 6 months repeated the questionnaire to evaluate test-retest reliability.

Statistical analyses

Nonparametric tests were used to assess the association and group differences for INQoL, because most of the INQoL domain scores in the current sample were not consistent with the assumption of normality. Reliability was assessed by the internal consistency, item-total correlation, and test-retest reliability. The internal consistency of items within each subscale was measured using Cronbach’s α. Spearman’s correlation coefficients were used to assess the test-retest reliability of the questionnaire, and the item-total correlation was assessed between the INQoL subscales of the life domains and the QoL score. Validity was assessed using construct validation (with the mRS and MMT-sum scores) and concurrent validation (with the SF-36 questionnaire). Correlations between the two groups were assessed using Spearman’s correlation analysis. The Mann-Whitney U test was used to identify the differences in the INQoL subscales between males and females, with differences considered statistically significant at p≤0.05. SPSS Statistics software (version 25, IBM Corporation, Armonk, NY, USA) was used for the statistical analyses.

Ethical considerations

This study was approved by the Institutional Review Board (IRB) of Gangnam Severance Hospital, Korea (approval number: 3-2020-0467). Written informed consent was obtained from all patients according to the protocol of the IRB.

RESULTS

Translation and cultural adaptation

Linguistic and cultural differences between English and Korean produced several difficulties in the translation process. Translating ‘myotonia’ was difficult because there is no such terminology in Korean that is familiar to the general public. Moreover, there were some cultural differences in the selection of the terms, such as ‘at the moment’ and ‘in the face of my condition’ in Korean, but these did not cause practical problems or misunderstandings about the meaning of each item.

Patient characteristics

This study enrolled 193 patients (141 males and 52 females) with genetic neuromuscular diseases, of which 175 (91.0%) and 18 (9.0%) had genetic myopathy and neuropathy, respectively (Table 1). The most common disease was facioscapulohumeral muscular dystrophy type 1 (n=27), followed by myotonic dystrophy (n=24), dysferlinopathy (n=20), Becker muscular dystrophy (n=17), and spinal muscular atrophy (n=9). The disease was not confirmed genetically in 26 patients (14.0%). The median ages at symptom onset and the examination were 18.0 years (interquartile range [IQR]=8.0–30.0 years) and 38.0 years (IQR=30.5–46.0 years), respectively. An invasive or noninvasive mechanical ventilator was used in 28 patients (15.0%). The median mRS and MMT-sum scores were 2 (IQR=1.5–3.0) and 230.0 (IQR=176.0–270.0), respectively.

Table 1. Genetic profiles of 193 Korean patients with genetic neuromuscular diseases.

Disease name Gene symbol Total patients (n=193) Retested patients (n=20)
Genetically confirmed myopathy 149 (77) 18 (90)
Dystrophinopathy DMD 17 (9) 3 (15)
Myotonic dystrophy DMPK 24 (12) 4 (20)
Facioscapulohumeral muscular dystrophy type 1 DUX4 * 27 (14) 3 (15)
Limb-girdle muscular dystrophy R2 DYSF 20 (10) 3 (15)
GNE myopathy GNE 8 (4) 2 (10)
ADSS1 myopathy ADSS1 7 (4) 0
Limb-girdle muscular dystrophy R1 CAPN3 6 (3) 1 (5)
Collagen-VI-related myopathy COL6A1 5 (3) 0
Glycogen storage disease type 2 GAA 5 (3) 1 (5)
Myotonia congenita CLCN1 4 (2) 0
Distal myopathy with nebulin defect NEB 3 (2) 0
TTN-mutation-related myopathy TTN 3 (2) 0
Progressive external ophthalmoplegia, autosomal dominant 1 POLG 2 (1) 0
Myopathy, congenital, with fiber-type disproportion RYR1 2 (1) 0
Limb-girdle muscular dystrophy R4 SGCB 2 (1) 0
Myopathy, congenital, with fiber-type disproportion TPM3 2 (1) 0
Very-long-chain acyl-coenzyme A dehydrogenase deficiency ACADVL 1 (1) 1 (5)
Myopathy, distal, Tateyama type CAV3 1 (1) 0
Myasthenic syndrome, congenital, 1A, slow-channel CHRNA1 1 (1) 0
Limb-girdle muscular dystrophy D1 DNAJB6 1 (1) 0
Limb-girdle muscular dystrophy R9 FKRP 1 (1) 0
Limb-girdle muscular dystrophy R16 DAG1 1 (1) 0
Limb-girdle muscular dystrophy R17 PLEC1 1 (1) 0
Myasthenia, congenital, 12, with tubular aggregates GFPT1 1 (1) 0
Emery-Dreifuss muscular dystrophy 2, autosomal dominant LMNA 1 (1) 0
Rigid spine muscular dystrophy 1 SELENON 1 (1) 0
Facioscapulohumeral muscular dystrophy type 2 SMCHD1 1 (1) 0
Inclusion-body myopathy with early-onset Paget disease with or without frontotemporal dementia 1 VCP 1 (1) 0
Genetic confirmed neuropathy 18 (9) 1 (5)
Spinal muscular dystrophy SMN1 9 (5) 1 (5)
Spinal and bulbar muscular atrophy AR 7 (4) 0
Charcot-Marie-Tooth disease, type 1A PMP22 1 (1) 0
Charcot-Marie-Tooth disease, type 2O DYNC1H1 1 (1) 0
Undiagnosed genetic myopathy 26 (14) 1 (5)

Data are presented as n (%).

*Inappropriate reactivation

All patients had genetic myopathy (Table 1). The median ages at symptom onset and the examination were 16.0 years (IQR=10.0–23.0 years) and 30.0 years (IQR=22.0–37.0 years), respectively. An invasive or noninvasive mechanical ventilator was used in one patient (7.0%). The median mRS and MMT-sum scores were 2.0 (IQR=1.0–3.0) and 244.0 (IQR=180.0–286.0), respectively.

Descriptive statistics of the INQoL subscales are presented in Table 2. In the ‘symptoms’ dimension, the most frequently experienced symptom was weakness (94.3%), and the least common symptom was double vision (5.2%). The median score was highest on the weakness subscale (78.9, IQR=63.2–86.8) and lowest on the subscales for droopy eyelids, double vision, and swallowing difficulties (all 0.0, IQR=0.0–0.0). In the ‘life domains’ dimension, the median score was highest on the activities subscale (75.0, IQR=52.3–90.7) and lowest on the social relationships subscale (50.9, IQR=23.6–71.8). The median QoL score was 66.1 (IQR=48.3–78.6).

Table 2. Descriptive statistics of the INQoL.

INQoL subscale Symptom present, n (%) Median [interquartile range] Range
Muscle weakness 182 (94.3) 78.9 [63.2–86.8] 0–100
Pain 113 (58.5) 36.8 [0.0–68.4] 0–100
Fatigue 169 (87.6) 68.4 [47.4–84.2] 0–100
Locking 77 (39.9) 0.0 [0.0–68.4] 0–100
Droopy eyelids 25 (13.0) 0.0 [0.0–0.0] 0–100
Double vision 10 (5.2) 0.0 [0.0–0.0] 0–100
Swallowing difficulties 29 (15.0) 0.0 [0.0–0.0] 0–100
Activities 75.0 [52.3–90.7] 0–100
Independence 55.6 [22.2–86.1] 0–100
Social relationships 50.9 [23.6–71.8] 0–100
Emotions 63.9 [41.7–80.6] 0–100
Body image 66.7 [47.2–83.3] 0–100
Perceived treatment effect 0.0 [0.0–16.7] -33.3 to 91.7
Expected treatment effect 0.0 [0.0–16.7] -50.0 to 100.0
QoL score 66.1 [48.3–78.6] 0–94.4

INQoL, Individualized Neuromuscular Quality of Life questionnaire; QoL, quality of life.

Reliability and validity of the Korean INQoL

Table 3 presents the findings for the internal consistency of each subscale. Cronbach’s α values other than for expected treatment effects exceeded 0.8, ranging from 0.805 for perceived treatment effects to 0.987 for locking. The average item-scale correlation within each subscale was also strong, with the correlation coefficient ranging from 0.715 for perceived treatment to 0.990 for locking. Twenty patients completed INQoL twice for assessing the test-retest reliability. All subscales except the ‘treatment effects’ dimension were significantly correlated (Table 3). Spearman’s correlation coefficients ranged from 0.453 for pain to 0.886 for droopy eyelids. All subscales in the ‘life domains’ dimension were significantly associated with each other, and their Spearman’s correlation coefficients ranged from 0.506 for emotions and independence to 0.816 for QoL score and social relationships (Supplementary Table 1 in the online-only Data Supplement). In particular, the Spearman’s coefficients for the correlations between the QoL score and all other subscales exceeded 0.7 (Supplementary Table 1 in the online-only Data Supplement).

Table 3. Reliability as measured using the internal consistency of items.

Subscale Internal consistency (n=193) Test-retest reliability (n=20)
Number of items Range of item-scale correlations Cronbach’s α Correlation coefficient p
Muscle weakness 3 0.883–0.911 0.924 0.772 <0.001
Pain 3 0.952–0.971 0.973 0.453 0.045
Fatigue 3 0.937–0.962 0.963 0.791 <0.001
Locking 3 0.978–0.984 0.987 0.517 0.020
Droopy eyelids 3 0.974–0.986 0.984 0.886 <0.001
Double vision 3 0.968–0.988 0.985 0.838 <0.001
Swallowing difficulties 3 0.942–0.968 0.971 0.805 <0.001
Activities of daily living 5 0.848–0.886 0.886 0.774 <0.001
Independence 3 0.750–0.923 0.882 0.859 <0.001
Social relationships 10 0.946–0.955 0.954 0.595 0.006
Emotions 6 0.901–0.930 0.927 0.638 0.002
Body image 3 0.788–0.851 0.871 0.678 0.001
Perceived treatment effects 3 0.617–0.801 0.805 0.348 0.133
Expected treatment effects 3 0.348–0.812 0.577 0.224 0.342
QoL score 14 0.925–0.933 0.933 0.840 <0.001

QoL, quality of life.

Table 4 presents the concurrent validity as assessed by evaluating correlations between the INQoL subscales and their relevant domains in the SF-36. The INQoL subscales other than those for locking, droopy eyelids, double vision, and swallowing difficulties were significantly associated with their relevant SF-36 domains (Spearman’s ρ=-0.274 to -0.833).

Table 4. Concurrent validity, assessed using the correlations between INQoL subscales and items on the SF-36.

INQoL subscale SF-36 item Correlation coefficient p
Weakness Physical functioning -0.635 <0.001
Role physical -0.619 <0.001
Pain Pain -0.561 <0.001
Fatigue Energy/fatigue -0.571 <0.001
Locking Physical functioning -0.112 0.120
Role physical -0.215 0.003
Droopy eyelids Physical functioning -0.124 0.086
Role physical -0.106 0.144
Double vision Physical functioning -0.148 0.039
Role physical -0.122 0.092
Swallowing difficulties Physical functioning -0.143 0.048
Role physical -0.138 0.056
Activities Physical functioning -0.698 <0.001
Role physical -0.677 <0.001
Social functioning -0.622 <0.001
Independence Physical functioning -0.833 <0.001
Role physical -0.603 <0.001
Social relationships Social functioning -0.714 <0.001
Emotions Role emotional -0.449 <0.001
Emotional well-being -0.611 <0.001
Body image Role physical -0.387 <0.001
Role emotional -0.274 <0.001
QoL score General health -0.531 <0.001
Health change -0.514 <0.001

INQoL, Individualized Neuromuscular Quality of Life questionnaire; SF-36, 36-item Short Form Survey; QoL, quality of life.

Table 5 indicates that the INQoL subscales other than those for locking, droopy eyelids, double vision, and swallowing difficulties were significantly correlated with the mRS and MMT-sum scores. Among the significantly associated subscales, clinical severity was associated most strongly with independence (Spearman’s ρ=0.753 and p<0.001 for mRS score, Spearman’s ρ=-0.741 and p<0.001 for MMT-sum score) and most weakly with pain (Spearman’s ρ=0.215 and p=0.003 for mRS score, Spearman’s ρ=-0.277 and p<0.001 for MMT-sum score). Patients who were older at the examination had lower scores on INQoL subscales such as muscle weakness (Spearman’s ρ=0.311, p<0.001), fatigue (Spearman’s ρ=0.202, p=0.005), swallowing difficulties (Spearman’s ρ=0.213, p=0.003), activities (Spearman’s ρ=0.298, p<0.001), independence (Spearman’s ρ=0.332, p<0.001), social relationships (Spearman’s ρ=0.341, p<0.001), emotions (Spearman’s ρ=0.145, p=0.045), body image (Spearman’s ρ=0.213, p=0.003), and QoL score (Spearman’s ρ=0.300, p<0.001) (Supplementary Table 2 in the online-only Data Supplement). Females had higher scores on the fatigue subscale (Mann-Whitney U=2852.5, p=0.018) (Supplementary Table 2 in the online-only Data Supplement). The scores on the INQoL subscales were not associated with the age at symptom onset (Supplementary Table 2 in the online-only Data Supplement).

Table 5. Known-group validity, assessed using the correlations between INQoL subscales and clinical severity as measured using the mRS score and the MMT-sum score based on the Medical Research Council scale.

INQoL subscale mRS score MMT-sum score
Correlation coefficient p Correlation coefficient p
Weakness 0.523 <0.001 -0.566 <0.001
Pain 0.215 0.003 -0.277 <0.001
Fatigue 0.325 <0.001 -0.316 <0.001
Locking 0.061 0.397 -0.142 0.049
Droopy eyelids -0.038 0.605 -0.060 0.409
Double vision 0.093 0.201 -0.139 0.055
Swallowing difficulties 0.031 0.673 -0.115 0.110
Activities 0.560 <0.001 -0.546 <0.001
Independence 0.753 <0.001 -0.741 <0.001
Social relationships 0.413 <0.001 -0.418 <0.001
Emotions 0.258 <0.001 -0.296 <0.001
Body image 0.369 <0.001 -0.391 <0.001
QoL score 0.473 <0.001 -0.466 <0.001

INQoL, Individualized Neuromuscular Quality of Life questionnaire; MMT, manual muscle testing; mRS, modified Rankin Scale; QoL, quality of life.

DISCUSSION

The present results indicate that the Korean INQoL has adequate reliability and validity for Korean patients with neuromuscular diseases. The present Korean patients had difficulty understanding the term myotonia, which was also found in a Dutch study,7 but all of them fully understood the other contents of the Korean INQoL despite the presence of cultural differences. Our study also included patients with various neuromuscular diseases. INQoL was initially developed for a wide range of neuromuscular diseases,18 but many previous studies primarily involved patients with myopathies.7,8,11,12,19 Therefore, one strength of this study is that it has expanded the scope of the diseases covered by INQoL, including genetic neuropathies.

The internal consistency and test-retest reliability were high except for the ‘treatment effects’ dimension, which might be because that dimension is intended to evaluate how patients perceive their ongoing treatment. Moreover, this study investigated patients with genetic neuromuscular diseases. Although gene therapy is being investigated widely at present, most interventions are symptom-relieving treatments rather than disease-modifying treatments. Therefore, the concept of treatment effects might be ambiguous for patients, and they might feel differently about treatment effects depending on their condition or mood at the time when they complete a questionnaire. These findings were similar to those of previous validation studies.8,12 Thus, the ‘treatment effects’ dimension may be inadequate for clinical usage.10,11,12 Our results for the item-total correlations between INQoL subscales of the life domains and QoL score in the Korean population were similar to some previous results8,12 and moreover were strongly correlated with the Dutch results.7

The INQoL subscales other than those for locking, droopy eyelids, double vision, and swallowing difficulties had moderately strong correlations with the SF-36 items in Korean patients with genetic neuromuscular diseases. These findings were similar to those of a Japanese study.12 The weak associations of SF-36 items with four disease-specific symptoms indicate that they have been ignored in other generic questionnaires. Therefore, our study emphasized the importance of disease-specific questionnaires such as INQoL in patients with neuromuscular diseases. The INQoL subscales other than those for locking, droopy eyelids, double vision, and swallowing difficulties were negatively affected by older age and severe disabilities (based on mRS and MMT-sum scores). Functional status and muscle strength generally affect the QoL, and previous studies have demonstrated that the mRS score and measured muscle strength are correlated with the INQoL subscales.7,8,12 However, locking, droopy eyelids, double vision, and swallowing difficulties are affected more by the presence of a specific disease than by the functional status due to the disease severity. We also found that the INQoL subscales were negatively affected by older age at the time of the examination. This is similar to the findings of a Japanese study, and may be associated with disease progression.12

Our study had some limitations. First, the study population was relatively small. However, the subject-to-item ratio was 4.29, which could be considered fair,20 and the total sample size and subgroup sizes (for test-retest reliability) of the study were not inferior to those in other studies validating INQoL in Japan and Serbia.11,12 The number of hospital visits during the study period was also relatively small, which is probably due to most genetic neuromuscular diseases being chronic and disabling. Completing the questionnaire over the phone or on the internet might be useful for overcoming these accessibility limitations. Second, the time interval between the two tests when assessing the test-retest reliability was both inconsistent and slightly longer than those in previous studies. Even though we only included patients with chronic and genetic neuromuscular diseases, this inconsistency might have underestimated the test-retest reliability. Third, this study did not assess the responsiveness of the questionnaire, and so this should be evaluated in future studies with long-term observations.

In conclusion, the Korean INQoL is a reliable and validated measurement tool for Korean patients with neuromuscular diseases. Further studies of its responsiveness and subgroup analyses are needed. This questionnaire could be widely used as a unified tool to assess the clinical course and treatment response of patients with neuromuscular diseases in Korea.

Acknowledgements

The authors would like to thank the patients for their help with this work.

Footnotes

Author Contributions:
  • Conceptualization: Hyung Jun Park, Young-Chul Choi.
  • Data curation: Hee Jo Han, Seung-Ah Lee.
  • Formal analysis: Hee Jo Han, Seung-Ah Lee, Hyung Jun Park.
  • Supervision: Michael R. Rose, Young-Chul Choi.
  • Writing—original draft: Hee Jo Han, Hyung Jun Park.
  • Writing—review & editing: Hee Jo Han, Hyung Jun Park.

Conflicts of Interest: The authors have no potential conflicts of interest to disclose.

Funding Statement: None

Availability of Data and Material

The datasets generated or analyzed during the study are available from the corresponding author on reasonable request.

Supplementary Materials

The online-only Data Supplement is available with this article at https://doi.org/10.3988/jcn.2022.18.5.514.

Supplementary Table 1

Coefficients of the item-total correlations between the INQoL subscales of the life domains and the QoL score

jcn-18-514-s001.pdf (20.5KB, pdf)
Supplementary Table 2

Associations of INQoL domains with sex, age at symptom onset, and age at examination

jcn-18-514-s002.pdf (24.6KB, pdf)

References

  • 1.Benarroch L, Bonne G, Rivier F, Hamroun D. The 2021 version of the gene table of neuromuscular disorders (nuclear genome) Neuromuscul Disord. 2020;30:1008–1048. doi: 10.1016/j.nmd.2020.11.009. [DOI] [PubMed] [Google Scholar]
  • 2.Taylor VR. Measuring healthy days; population assessment of health-related quality of life. Atlanta: US Centers for Disease Control and Prevention; 2000. [Google Scholar]
  • 3.Gelber RD, Gelber S. In: Recent Advances in Clinical Trial Design and Analysis. Thall PF, editor. Boston: Springer; 1995. Quality-of-life assessment in clinical trials; pp. 225–246. [DOI] [PubMed] [Google Scholar]
  • 4.Vincent KA, Carr AJ, Walburn J, Scott DL, Rose MR. Construction and validation of a quality of life questionnaire for neuromuscular disease (INQoL) Neurology. 2007;68:1051–1057. doi: 10.1212/01.wnl.0000257819.47628.41. [DOI] [PubMed] [Google Scholar]
  • 5.Landfeldt E, Nikolenko N, Jimenez-Moreno C, Cumming S, Monckton DG, Gorman G, et al. Disease burden of myotonic dystrophy type 1. J Neurol. 2019;266:998–1006. doi: 10.1007/s00415-019-09228-w. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 6.Kacar A, Bjelica B, Bozovic I, Peric S, Nikolic A, Cobeljic M, et al. Neuromuscular disease-specific questionnaire to assess quality of life in patients with chronic inflammatory demyelinating polyradiculoneuropathy. J Peripher Nerv Syst. 2018;23:11–16. doi: 10.1111/jns.12251. [DOI] [PubMed] [Google Scholar]
  • 7.Seesing FM, van Vught LE, Rose MR, Drost G, van Engelen BG, van der Wilt GJ. The individualized neuromuscular quality of life questionnaire: cultural translation and psychometric validation for the Dutch population. Muscle Nerve. 2015;51:496–500. doi: 10.1002/mus.24337. [DOI] [PubMed] [Google Scholar]
  • 8.Sansone VA, Panzeri M, Montanari M, Apolone G, Gandossini S, Rose MR, et al. Italian validation of INQoL, a quality of life questionnaire for adults with muscle diseases. Eur J Neurol. 2010;17:1178–1187. doi: 10.1111/j.1468-1331.2010.02992.x. [DOI] [PubMed] [Google Scholar]
  • 9.Fagoaga J, Girabent-Farres M, Bagur-Calafat C. Translation and validation of the Individualised Neuromuscular Quality of Life scale for the Spanish population: quality of life assessment for persons with neuromuscular diseases. Rev Neurol. 2017;64:194–200. [PubMed] [Google Scholar]
  • 10.Sadjadi R, Vincent KA, Carr AJ, Walburn J, Brooks VL, Pandya S, et al. Validation of the individualised neuromuscular quality of life for the USA with comparison of the impact of muscle disease on those living in USA versus UK. Health Qual Life Outcomes. 2011;9:114. doi: 10.1186/1477-7525-9-114. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 11.Peric S, Sansone V, Lavrnic D, Meola G, Basta I, Miljkovic M, et al. Serbian validation of the Individualized Neuromuscular Quality of Life Questionnaire (INQoL) in adults with myotonic dystrophy type 1. J Neurol Res. 2011;1:153–160. [Google Scholar]
  • 12.Fujino H, Saito T, Takahashi MP, Takada H, Nakayama T, Ogata K, et al. Validation of the Individualized Neuromuscular Quality of Life in Japanese patients with myotonic dystrophy. Muscle Nerve. 2018;58:56–63. doi: 10.1002/mus.26071. [DOI] [PubMed] [Google Scholar]
  • 13.Guillemin F, Bombardier C, Beaton D. Cross-cultural adaptation of health-related quality of life measures: literature review and proposed guidelines. J Clin Epidemiol. 1993;46:1417–1432. doi: 10.1016/0895-4356(93)90142-n. [DOI] [PubMed] [Google Scholar]
  • 14.Beaton DE, Bombardier C, Guillemin F, Ferraz MB. Guidelines for the process of cross-cultural adaptation of self-report measures. Spine (Phila Pa 1976) 2000;25:3186–3191. doi: 10.1097/00007632-200012150-00014. [DOI] [PubMed] [Google Scholar]
  • 15.Brooke MH, Griggs RC, Mendell JR, Fenichel GM, Shumate JB, Pellegrino RJ. Clinical trial in Duchenne dystrophy. I. The design of the protocol. Muscle Nerve. 1981;4:186–197. doi: 10.1002/mus.880040304. [DOI] [PubMed] [Google Scholar]
  • 16.Nam BH, Lee SU. Testing the validity of the Korean SF-36 health survey. J Health Info Stat. 2003;28:3–25. [Google Scholar]
  • 17.Han CW, Lee EJ, Iwaya T, Kataoka H, Kohzuki M. Development of the Korean version of short-form 36-item health survey: health related QOL of healthy elderly people and elderly patients in Korea. Tohoku J Exp Med. 2004;203:189–194. doi: 10.1620/tjem.203.189. [DOI] [PubMed] [Google Scholar]
  • 18.Seesing FM, Drost G, Groenewoud J, van der Wilt GJ, van Engelen BG. Shared medical appointments improve QOL in neuromuscular patients: a randomized controlled trial. Neurology. 2014;83:240–246. doi: 10.1212/WNL.0000000000000588. [DOI] [PubMed] [Google Scholar]
  • 19.Moradi K, Jamal-Omidi S, Masoudi M, Bagheri S, Nafissi S, Fatehi F. Validation of Persian Individualized Neuromuscular Quality of Life in patients with muscular dystrophies. Curr J Neurol. 2020;19:13–19. [Google Scholar]
  • 20.Anthoine E, Moret L, Regnault A, Sébille V, Hardouin JB. Sample size used to validate a scale: a review of publications on newly-developed patient reported outcomes measures. Health Qual Life Outcomes. 2014;12:176. doi: 10.1186/s12955-014-0176-2. [DOI] [PMC free article] [PubMed] [Google Scholar]

Associated Data

This section collects any data citations, data availability statements, or supplementary materials included in this article.

Supplementary Materials

Supplementary Table 1

Coefficients of the item-total correlations between the INQoL subscales of the life domains and the QoL score

jcn-18-514-s001.pdf (20.5KB, pdf)
Supplementary Table 2

Associations of INQoL domains with sex, age at symptom onset, and age at examination

jcn-18-514-s002.pdf (24.6KB, pdf)

Data Availability Statement

The datasets generated or analyzed during the study are available from the corresponding author on reasonable request.


Articles from Journal of Clinical Neurology (Seoul, Korea) are provided here courtesy of Korean Neurological Association

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