Validity of the 6 minute walk test in facioscapulohumeral muscular dystrophy

Eichinger, Katy; Heatwole, Chad; Heininger, Susanne; Stinson, Nikia; Stock, Carly Matichak; Grosmann, Carla; Wagner, Kathryn R.; Tawil, Rabi; Statland, Jeffrey

doi:10.1002/mus.25251

Cited by 38 publications

(38 citation statements)

References 34 publications

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“…The 6MWT is a well-established measurement of endurance, widely adopted to monitor patients with neuromuscular or cardiorespiratory diseases. It has been validated in many neuromuscular diseases (e.g., Pompe disease, FSHD, Duchenne and Becker dystrophy, spinal muscular atrophy, DM1) but not yet in myotonic dystrophy type 2 (7,(31)(32)(33)(34). In our study the mean distance walked on 6MWT was higher than what reported in previous studies on DM1, this might be surprising considering the more proximal involvement of DM2 but is well-explained by the usually milder phenotype (8).…”

Section: Discussioncontrasting

confidence: 79%

Validation of Motor Outcome Measures in Myotonic Dystrophy Type 2

Montagnese¹,

Rastelli²,

Khizanishvili³

et al. 2020

Front. Neurol.

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Introduction: Myotonic dystrophy type 2 (DM2) lacks disease-specific, validated, motor outcome measures (OMs), and patients' reported outcomes (PROs). This represents a limit for the monitoring of disease progression and treatment response. Our aim was to identify the most appropriate OMs to be translated in clinical practice and clinical trials on DM2. This study has been registered on clinicaltrials.gov NCT03603171 (https:// clinicaltrials.gov/ct2/show/NCT03603171).Methods: Sixty-six patients with genetically confirmed DM2 underwent a baseline and a follow-up visit after 1 year. The tested OMs included: hand opening time, pressure pain threshold (PPT), manual muscle testing (MMT), hand held dynamometry (HHD), scale for the assessment and rating of ataxia (SARA), quantitative motor function test (QMFT), gait stairs Gowers chair (GSGC), 30-s sit to stand test, functional index 2 (FI-2) and 6MWT. The PROs included DM1-Active-C, Rasch-built Pompe-specific activity scale (R-Pact), fatigue and daytime sleepiness (FDSS), brief pain inventory short form (BPI-sf), myotonia behavior scale (MBS), and the McGill pain questionnaire.Results: All patients completed the MBS and the results correlated well with the handopening time. The PPT showed a low reliability, no correlation with pain questionnaires, and did not differentiate patients with or without myalgia. Both muscle strength assessments, MMT and HHD, showed good construct validity. The QMFT showed an acceptable ceiling effect (14.5%), good convergent and differential validity and performed overall better than GSGC. The SARA score showed high flooring effect and is not useful in DM2. 6MWT proved a valid outcome measure in DM2. The 30-s sit to stand is a feasible test with good convergent validity, showing a flooring effect of 20% as it cannot be used in more severely affected patients. The FI-2 is time-consuming and has a high ceiling effect. At the 1-year visit the only assessments able to detect a worsening of DM2 were HHD, QMFT, and 6MWT, which are the most sensitive to change, and therefore clinically meaningful OMs in DM2. Conclusion:The clinical meaningful motor outcome measures that best depict the multifaceted phenotype of DM2 and its slow progression are MBS, MMT, or HHD (depending on the clinical setting), QMFT, and the 6MWT.

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Section: Discussioncontrasting

confidence: 79%

Validation of Motor Outcome Measures in Myotonic Dystrophy Type 2

Montagnese¹,

Rastelli²,

Khizanishvili³

et al. 2020

Front. Neurol.

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“…Manual muscle testing was performed by using a modified Medical Research Council scale (bilateral hip flexors, knee flexors/extensors, and ankle dorsiflexors) . The 6MWT was performed by using a standardized protocol on a linear course . Electrical impedance myography recordings of the bilateral vastus lateralis (VL), tibialis anterior (TA), and medial gastrocnemius (MG) muscles were obtained by using a device from Skulpt (Brookline, Massachusetts) with handheld sensors (20–00045) in longitudinal configuration.…”

Section: Methodsmentioning

confidence: 99%

Magnetic resonance imaging correlates with electrical impedance myography in facioscapulohumeral muscular dystrophy

et al. 2020

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Introduction Electrical impedance myography (EIM) has been proposed as a noninvasive biomarker of muscle composition in facioscapulohumeral muscular dystrophy (FSHD). Here we determine the associations of EIM variables with muscle structure measured by MRI. Methods We evaluated 20 patients with FSHD at two centers, comparing EIM measurements (resistance, reactance, and phase at 50, 100, and 211 kHZ) recorded from bilateral vastus lateralis, tibialis anterior, and medial gastrocnemius muscles to MRI skin and subcutaneous fat thickness, MRI T1‐based muscle severity score (T1 muscle score), and MRI quantitative intramuscular Dixon fat fraction (FF). Results While reactance and phase both correlated with FF and T1 muscle score, 50 kHz reactance was most sensitive to muscle structure alterations measured by both T1 score (ρ = −0.71, P < .001) and FF (ρ = −0.74, P < .001). Discussion This study establishes the correlation of EIM with structural MRI features in FSHD and supports further evaluation of EIM as a potential biomarker in FSHD clinical trials.

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“…These assessments are detailed below and in Table 1. (8)(9)(10)(11)(12)(13)(14)(15)(16)(32)(33)(34)(35)(36)(37)(38)(39)…”

Section: Assessmentsmentioning

confidence: 99%

“…PHILLIPS ET AL. (8) • Originally developed for assessment of aerobic activity in patients with respiratory disease (9) ; also validated in numerous other patient populations, (10)(11)(12) including those with musculoskeletal diseases (eg, Duchenne/Becker muscular dystrophy, (13,14) facioscapulohumeral muscular dystrophy, (15) spinal muscle atrophy (16) ). • Patients were instructed to walk a 60-m lap along the length of a hallway for 6 min, and the total number of meters walked was recorded.…”

Section: Statistical Analysesmentioning

confidence: 99%

“…The 6-Minute Walk Test (6MWT) is a self-paced walking test that measures the distance an individual is able to walk on a hard, flat surface for 6 min. (8) Originally developed for assessment of aerobic activity in patients with respiratory disease, (9) the 6MWT is now validated in numerous other patient populations, (10)(11)(12) including those with musculoskeletal diseases (eg, Duchenne/Becker muscular dystrophy, (13,14) facioscapulohumeral muscular dystrophy, (15) spinal muscle atrophy (16) ), and has been used as an efficacy measure in clinical studies of patients with muscular (17)(18)(19) and metabolic (20)(21)(22)(23) disorders. The 6MWT has also been used as an outcome measure in clinical studies of patients with other rare diseases, such as Pompe disease, (24) Hunter syndrome, (25) and Morquio A syndrome.…”

Section: Introductionmentioning

confidence: 99%

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Reliability and Validity of the 6‐Minute Walk Test in Hypophosphatasia

et al. 2019

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This investigation evaluated the reliability and validity of the 6‐Minute Walk Test (6MWT) in patients with pediatric hypophosphatasia (HPP). Children (aged 6 to 12 years; n = 11), adolescents (13 to 17 years; n = 4), and adults (18 to 65 years; n = 9) completed the 6MWT at screening and baseline in two clinical studies of asfotase alfa. Test‐retest reliability of the 6MWT, evaluated with Pearson's correlation coefficients ( r ) for screening versus baseline, was high for children ( r = 0.95; p < 0.0001), adolescents ( r = 0.81; p = 0.125), and adults ( r = 0.94; p = 0.0001). The most conservative minimal clinically important differences, estimated using distribution‐based methods, were 31 m (children and adults) and 43 m (adolescents). In children, the 6MWT correlated significantly with scores on measures of skeletal disease, which included the Radiographic Global Impression of Change scale ( r = 0.50; p < 0.0001) and the Rickets Severity Scale ( r = −0.78; p < 0.0001), such that distance walked increased as the severity of skeletal disease decreased. Significant ( p < 0.0001) correlations with the 6MWT distance walked were also observed for children with scores on parent‐reported measures of disability ( r = −0.67), ability to function in activities of daily living ( r = 0.71 to 0.77), and parent‐reported measures of pain ( r = −0.39). In adolescents and adults, 6MWT distance walked correlated significantly ( p < 0.05) with measures of lower extremity function ( r = 0.83 and 0.60, respectively), total pain severity ( r = −0.41 and −0.36, respectively), and total pain interference ( r = −0.41 and −0.49, respectively). Collectively, these data indicate that the 6MWT is a reliable, valid measure of physical functioning in patients with pediatric HPP. © 2018 The Authors. JBMR Plus Published by Wiley Periodicals, Inc. on behalf of the American Society for Bone and Mineral Research.

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Validity of the 6 minute walk test in facioscapulohumeral muscular dystrophy

Cited by 38 publications

References 34 publications

Validation of Motor Outcome Measures in Myotonic Dystrophy Type 2

Validation of Motor Outcome Measures in Myotonic Dystrophy Type 2

Magnetic resonance imaging correlates with electrical impedance myography in facioscapulohumeral muscular dystrophy

Reliability and Validity of the 6‐Minute Walk Test in Hypophosphatasia

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