Marie Kierkegaard

Reliability and feasibility of the six minute walk test in subjects with myotonic dystrophy

The objective was to describe test-retest reliability and feasibility of the six minute walk test in adult subjects with myotonic dystrophy type 1. Twelve subjects (28-68 years, mean 44) performed three six minute walk tests on two occasions, one week apart. Relative reliability was high (ICC(2.1)=0.99) and absolute reliability values were low (standard error of measurement 12 m, repeatability 33 m). Feasibility was investigated in a sample of 64 subjects (19-70 years, mean 43). Fifty-two subjects were able to perform two tests on the same day. Subjects with severe proximal weakness had difficulties performing repeated tests. A practice trial followed by a second test on the same day can be recommended for most subjects, and the best test should be used for evaluations. In conclusion, even though the study sample was small, the present study indicates that the six minute walk test is reliable and feasible in subjects with myotonic dystrophy type 1.

The relationship between walking, manual dexterity, cognition and activity/participation in persons with multiple sclerosis

Background: Multiple sclerosis has a vast impact on health, but the relationship between walking, manual dexterity, cognition and activity/participation is unclear. Objective: The specific aims were to explore the discriminative ability of measures of walking, manual dexterity and cognition, and to identify cut-off values in these measures, for prediction of independence in personal and instrumental activities of daily living (ADL) and activity/participation in social and lifestyle activities. Methods: Data from 164 persons with multiple sclerosis were collected during home visits with the following measures: the 2 × 5 m walk test, the Nine-hole Peg Test, the Symbol Digit Modalities Test, the Katz Personal and Instrumental ADL Indexes, and the Frenchay Activities Index (measuring frequency in social and lifestyle activities). Results: The 2 × 5 m walk test and the Nine-hole Peg Test had high and better discriminative and predictive ability than the Symbol Digit Modalities Test. Cut-off values were identified. The accuracy of predictions was increased above all by combining the 2 × 5 m walk test and the Nine-hole Peg Test. Conclusion: The proposed cut-off values in the 2 × 5 m walk test and the Nine-hole Peg Test may be used as indicators of functioning and to identify persons risking activity limitations and participation restrictions. However, further studies are needed to confirm the usefulness in clinical practice.

Functioning and disability in adults with myotonic dystrophy type 1.

Purpose. To provide a comprehensive description of functioning and disability with regard to stages of disease progression in adults with myotonic dystrophy type 1 (DM1). Further to explore associations of measures of manual dexterity and of walking capacity with measures of activities of daily living (ADL) and participation in social and lifestyle activities. Methods. Seventy persons with DM1 underwent examinations, tests and answered questionnaires. Stages of disease progression were based on the muscular impairment rating scale. Results. Overweight, cardiac dysfunctions, respiratory restrictions, fatigue and/or low physical activity levels were found in approximately 40%% of those with DM1. Over 75%% had muscle impairments, and activity limitations in manual dexterity and walking. Dependence in personal and instrumental ADL was found in 16%% and 39%%, respectively, and participation restrictions in social and lifestyle activities in 52%%. The presence of concurrent body-function impairments, activity limitations and participation restrictions was high. Significant differences were found in muscle impairment, manual dexterity, mobility, ADL and social and lifestyle activities with regard to disease progression. Cut-off values in measures of manual dexterity and walking capacity associated to functioning are proposed. Conclusion. This information can be used for developing clinical practise and for health promotion for persons with DM1.

Effects of hand-training in persons with myotonic dystrophy type 1 – a randomised controlled cross-over pilot study

Abstract Purpose: To investigate the effects of a hand-training programme on grip, pinch and wrist force, manual dexterity and activities of daily living, in adults with myotonic dystrophy type 1 (DM1). Method: In this randomised controlled trial with a crossover design, 35 adults with DM1 were, after stratification for grip force, assigned by lot to two groups. Group A started with 12 weeks of hand training, while group B had no intervention. After a wash-out period of 12 weeks, where none received training, the order was reversed. The Grippit® was used as primary outcome measure and the hand-held Microfet2™ myometer, the Purdue Pegboard, the Canadian Occupational Performance Measure (COPM) and the Assessment of Motor and Process Skills (AMPS) were secondary outcome measures. Assessments were performed before and after training and control periods, i.e. four times altogether. Results: Ten persons dropped out and 13 had acceptable adherence. Intention-to-treat analyses revealed significant intervention effects for isometric wrist flexor force (p = 0.048), and for COPM performance (p = 0.047) and satisfaction (p = 0.027). On an individual level, improvements were in general showed after a training period. Conclusion: The hand-training programme had positive effects on wrist flexor force and self-perception of occupational performance, and of satisfaction with performance. No evident detrimental effects were shown. Implications for Rehabilitation Myotonic dystrophy type 1 (DM1) is a slowly progressive neuromuscular disease characterised by myotonia and muscle weakness and wasting. People with DM1 are often concerned about their ability to carry out ADL and to participate in, e.g. work, sports and hobbies when they gradually become weaker. This pilot study showed that a hand-training programme improved wrist flexor force and self-perception and satisfaction of occupational performance. Resistance training of hand muscles with a silicon-based putty can be a therapy option for people with DM1 in clinical practise.

Predictors of health-related quality of life in people with amyotrophic lateral sclerosis

BACKGROUND Knowledge of factors influencing health-related quality of life (HRQL) in people with amyotrophic lateral sclerosis (ALS) is important because some factors might be amenable to intervention. OBJECTIVES The aim was to describe and explore the effects of disease severity, fatigue, anxiety, depression, frequency of social and lifestyle activities, coping capacity and mechanical ventilator use on HRQL in people with ALS. METHODS Sixty people with ALS were enrolled in this cross-sectional study. Data were collected with questionnaires during home visits. The Sickness Impact Profile and the EuroQol Visual Analogue Scale were used to assess HRQL. Multivariate regression analyses explored associations between HRQL and independent factors. RESULTS Low frequency of social and lifestyle activities, and severe disease, were associated with worse HRQL, explaining 57% of total variance in the Sickness Impact Profile physical score. Severe disease, weak coping capacity and anxiety and/or depression were associated with worse HRQL, explaining 33% of total variance in Sickness Impact Profile psychosocial score. Fatigue and mechanical ventilator use were associated with worse HRQL, explaining 17% of variance in the EuroQol Visual Analogue Scale. CONCLUSION Knowledge and understanding of how frequency of social and lifestyle activities, disease severity, coping capacity, anxiety and/or depression, fatigue and ventilator use contribute to and predict self-rated HRQL can optimize person-centred care and support.

Dimensions Underlying Measures of Disability, Personal Factors, and Health Status in Cervical Radiculopathy: A Cross-Sectional Study

AbstractThis cross-sectional study sought to identify dimensions underlying measures of impairment, disability, personal factors, and health status in patients with cervical radiculopathy.One hundred twenty-four patients with magnetic resonance imaging-verified cervical radiculopathy, attending a neurosurgery clinic in Sweden, participated. Data from clinical tests and questionnaires on disability, personal factors, and health status were used in a principal-component analysis (PCA) with oblique rotation.The PCA supported a 3-component model including 14 variables from clinical tests and questionnaires, accounting for 73% of the cumulative percentage. The first component, pain and disability, explained 56%. The second component, health, fear-avoidance beliefs, kinesiophobia, and self-efficacy, explained 9.2%. The third component including anxiety, depression, and catastrophizing explained 7.6%. The strongest-loading variables of each dimension were “present neck pain intensity,” “fear avoidance,” and “anxiety.”The three underlying dimensions identified and labeled Pain and functioning, Health, beliefs, and kinesiophobia, and Mood state and catastrophizing captured aspects of importance for cervical radiculopathy. Since the variables “present neck pain intensity,” “fear avoidance,” and “anxiety” had the strongest loading in each of the three dimensions; it may be important to include them in a reduced multidimensional measurement set in cervical radiculopathy.

Reliability and concurrent validity of postural asymmetry measurement in adolescent idiopathic scoliosis

AIM To investigate the reliability and concurrent validity of the Baseline® Body Level/Scoliosis meter for adolescent idiopathic scoliosis postural assessment in three anatomical planes. METHODS This is an observational reliability and concurrent validity study of adolescent referrals to the Orthopaedic department for scoliosis screening at Karolinska University Hospital, Stockholm, Sweden between March-May 2012. A total of 31 adolescents with idiopathic scoliosis (13.6 ± 0.6 years old) of mild-moderate curvatures (25° ± 12°) were consecutively recruited. Measurement of cervical, thoracic and lumbar curvatures, pelvic and shoulder tilt, and axial thoracic rotation (ATR) were performed by two trained physiotherapists in one day. The intraclass correlation coefficient (ICC) was used to determine the inter-examiner reliability (ICC2,1) and the intra-rater reliability (ICC3,3) of the Baseline® Body Level/Scoliosis meter. Spearman’s correlation analyses were used to estimate concurrent validity between the Baseline® Body Level/Scoliosis meter and Gold Standard Cobb angles from radiographs and the Orthopaedic Systems Inc. Scoliometer. RESULTS There was excellent reliability between examiners for thoracic kyphosis (ICC2,1 = 0.94), ATR (ICC2,1 = 0.92) and lumbar lordosis (ICC2,1 = 0.79). There was adequate reliability between examiners for cervical lordosis (ICC2,1 = 0.51), however poor reliability for pelvic and shoulder tilt. Both devices were reproducible in the measurement of ATR when repeated by one examiner (ICC3,3 0.98-1.00). The device had a good correlation with the Scoliometer (rho = 0.78). When compared with Cobb angle from radiographs, there was a moderate correlation for ATR (rho = 0.627). CONCLUSION The Baseline® Body Level/Scoliosis meter provides reliable transverse and sagittal cervical, thoracic and lumbar measurements and valid transverse plan measurements of mild-moderate scoliosis deformity.

Is one trial enough for repeated testing? Same-day assessments of walking, mobility and fine hand use in people with myotonic dystrophy type 1

Performance-based assessments of physical function are essential in people with myotonic dystrophy type 1 (DM1) to monitor disease progression and evaluate interventions. Commonly used are the six-minute walk test, the 10 m-walk test, the timed up-and-go test, the timed-stands test, grip strength tests and the nine-hole peg test. The number of trials needed on a same-day test occasion and whether the first, best or average of trials should be reported as result is unknown. Thus, the aim was to describe and explore differences between trials in these measures of walking, mobility and fine hand use in 70 adults with DM1. Three trials were performed for each test except for the six-minute walk test where two trials were allowed. There were statistical significant differences over trials in all tests except for the 10 m-walk test and grip strength tests. Pair-wise comparisons showed that the second and third trials were in general better than the first, although effect sizes were small. At which trial the individuals performed their best differed between individuals and tests. People with severe muscular impairment had difficulties to perform repeated trials. Intraclass correlation coefficients were all high in analyses exploring how to report results. The conclusion and clinical implication is that, for a same-day test occasion, one trial is sufficient for the 10 m-walk test and grip strength tests, and that repeated trials should be allowed in the timed up-and-go test, timed-stands test and nine-hole peg tests. We recommend that two trials are performed for these latter tests as such a protocol could accommodate people with various levels of impairments and physical limitations.

Participation restriction in childhood phenotype of myotonic dystrophy type 1: a systematic retrospective chart review

Myotonic dystrophy type 1 (DM1), a neuromuscular disorder, is divided into four clinical phenotypes: congenital; childhood; adult‐onset, and late‐onset. Publications about the childhood phenotype, especially the long‐term outcome, are scarce. The aims of this study were to assess and describe participation outcomes in adults with the childhood phenotype.

Responsiveness of performance-based outcome measures for mobility, balance, muscle strength and manual dexterity in adults with myotonic dystrophy type 1

OBJECTIVE To assess changes and responsiveness in outcome measures of mobility, balance, muscle strength and manual dexterity in adults with myotonic dystrophy type 1. DESIGN A 9-year longitudinal study conducted with 113 patients. METHODS The responsiveness of the Timed Up and Go test, Berg Balance Scale, quantitative muscle testing, grip and pinch-grip strength, and Purdue Pegboard Test was assessed using criterion and construct approaches. Patient-reported perceived changes (worse/stable) in balance, walking, lower-limb weakness, stair-climbing and hand weakness were used as criteria. Predefined hypotheses about expected area under the receiver operating characteristic curves (criterion approach) and correlations between relative changes (construct approach) were explored. RESULTS The direction and magnitude of median changes in outcome measures corresponded with patient-reported changes. Median changes in the Timed Up and Go test, grip strength, pinch-grip strength and Purdue Pegboard Test did not, in general, exceed known measurement errors. Most criterion (72%) and construct (70%) approach hypotheses were supported. Promising responsiveness was found for outcome measures of mobility, balance and muscle strength. Grip strength and manual dexterity measures showed poorer responsiveness. CONCLUSION The performance-based outcome measures captured changes over the 9-year period and responsiveness was promising. Knowledge of measurement errors is needed to interpret the meaning of these longitudinal changes.