Skip to main content

Table 2 Psychometric properties

From: The use of digital outcome measures in clinical trials in rare neurological diseases: a systematic literature review

Disease

Validity

Reliability

Sensitivity to negative change

Sensitivity to positive change

Actigraph, Motionlogger Watch; Ambulatory Monitoring, Ardsley, NY, USA

DMD

PIM score correlated with six min walk distance and knee extension strength [138]

   

Actimyo, Sysnav, Vernon

DMD

For the MoviPlate and BBT, all variables correlated with the functional scores. Norm of the angular velocity, power, elevation rat correlated with the Minnesota scores and the writing task. The mean of the rotation rate and mean of the elevation rate had the best correlations with task scores [43]

All ActiMyo variables showed high to very high reliability as assessed using ICC values. The mean of the rotation rate and mean of the elevation rate had the best reliability scores [43]

  

SMA

MFM 32 and grip strength were found to have high correlation with wrist acceleration as measure by ActiMyo [42]

 

Wrist angular velocity, the wrist acceleration, the wrist vertical acceleration and power decreased significantly over six and 12 months as measures in patients with Type 2 SMA and non-ambulant with Type 3 [42]

 

FSHD

Strong correlations between representative (stride lenght and velocity) gait variables and MMT variables [44]

All the measurements showed high reliability according to ICC values (all > 0.9). Representative variables showed lower SEMs (all < 0.03) compared to cumulative variables (40.2 and 28.5) [44]

Significant decline for median speed, SV95C and SL95C at 3 months; The SRM of median speed, SV95C and 95th centile length (was significantly elevated at 3 months [44]

 

ActivPal, PAL Technologies Ltd, Glasgow, UK

Sarcoidosis

Patients with sarcoidosis had s lower daily step counts than controls and a trend towards fewer sit-to-stand transitions each day. Correlation between 6MWD and the daily step count [102]

   

Dermatomyositis

Patients with inactive disease had lower physical activity levels compared with controls [139]

   

Rett syndrome

   

The sedentary time was decreased after intervention. Positive effects with small to medium effect sizes were seen in sedentary time [122]

Actiwatch 2, AW2; Philips Respironics, Bend, Oregon

DMD

Habitual daytime activity level was associated with 6MWT performance [140]

   

AD_BRC sensor

HD

Sensor derived velocity was significantly higher in healthy controls and premanifest HD when compared to HD. Step and stride length was significantly longer in controls and premanifest HD when compared to HD. Significant diffences between subject groups across all four balance tasks [58]

   

AIM- system

FRDA

Smoothness, trajectory length, duration, and range of motion were the most effective to distinguish individuals with FRDA from controls. Strong correlation between the AIM-S score and the mFARS score and the NeuroUL score [130]

The AIM-S score showed good to excellent test re-test reliability [130]

The sensitivity of the AIM-S to detect deterioration in upper limb function was greater than other measures [130]

The AIM-S score showed minimal variability [130]

ASUR-Autonomous Sensing Unit Recorder + Physilog

DMD

Except cadence, all gait parameters showed significant differences between patients and controls. All gait parameters were more affected in the moderate group compared to the mild group. Moderate correlations between the MFM and: stride velocity, cadence and spectral entropy [21]

   
   

Duration of the walking episodes or the succession of two or three walking episodes lasting more than 30 s were the most improved after prednisolone treatment [24]

Biopack

FD

The incidence of tremor was significantly higher in dystonic patients as compared to controls [80]

   

BioStampRC® wearable sensors developed by MC10 Inc

HD

The average truncal Chorea Index was higher in individuals with HD than in controls. Individuals with HD walked less and took longer duration steps than the other groups. Correlation between the UHDRS maximal truncal chorea score and the average truncal Chorea Index [49]

 

Walk speed of individuals with HD showed a significant decrease over 12 months but not time spent lying, sitting, standing, and walking, truncal Chorea Index, step count and duration.[49]

 

Individuals with HD spent over 50% of the total time lying down, more than individuals with prodromal HD, PD, and controls [47]

   

Cambridge Neurotechnology AW4 or Respironics Actiwatch 2 actigraph

MPS

Children with MPS III had significantly higher activity levels during the early morning hours compared to controls [117]

   

CHDR Monitoring Remotely (CHDR MORE) platform

FSHD

The classification between patients with FSHD and controls with 93% accuracy, 100% sensitivity, and 80% specificity. Features relating to smartphone acceleration, app use, location, physical activity, sleep, and call behavior were the most salient features for the classification [39]

   

Computational Motor Objective Rater (CMOR)

FD

Head posture severity correlated with severity ratings from movement disorders neurologists using both the TWSTRS-2 and an adapted version of the Global Dystonia Rating Scale [85]

   

DynaPort Move Monitor, McRoberts, The Hague, The Netherlands

MG

Patients perform less vigorous PA, spend more time sedentary and engage in less and shorter durations of MVPA than controls. Habitual PA correlated positively with 6 min walking distance [41]

   

PAL was lower in patients than in controls. No correlation between disease severity and number of steps/day nor between disease severity and PAL [40]

   

EBIMU 9DOF

FD

All parameters were validated by comparing with the TSWTRS-total score and the TWSTRS-severity score. The MAV parameters showed a higher correlation with clinical severity than RA parameters [84]

The ICCs were all more than 0.9 for the four parameters, which showed good agreement for all parameters [84]

  

eMotion Faros 180

ALS

Average daytime active; percentage of daytime active; total daytime activity score; total 24-h activity score showed correlations with ALSFRS-R total and gross motor domain scores [141]

 

All the activity endpoints investigated changed from baseline over the course of the study (48 weeks) to indicate a decline in physical activity over time [141]

 

Fitbit One TM

Pompe disease

Mean step count differed by age (p < 0.01), diagnostic delay (p < 0.05), disease duration (p < 0.05), and ambulatory status (p < 0.05)

Patient-reported “fatigue and pain” score was inversely correlated with step count (p < 0.05) and peak 1-min activity (p < 0.01) [115]

   

GaitUp Physilog 5, Lausanne, Switzerland

FRDA

Controls were significantly more active than the FRDA group. Peak swing and stance period were the most discriminatory parameters. Correlation between the 25-foot walk test and the cadence. Digitally derived stride width strongly reflected the risk of falling. Mean stride width from the real-world gait analysis dropped with GAA repeat length of the short allele [99]

   

GENEActiv, Kimbolton, Cambs, United Kingdom

HD

The developed system achieved 98.78% accuracy in discriminating between healthy and HD participants. Correlation between MIS and mULMS [67]

   

HD participants had a greater percentage of walking bouts with irregular movements, lower walking consistency and higher across-bout variability compared to controls. Negative correlation between UHDRS-TMS scores and walking time and steps per day. Moderate correlation within-bout and across-bout gait consistency and the clinical measures of upper extremity chorea and total chorea [56]

   

DM1

A significant difference between the myotonic dystrophy group and the controls was detectable at each test. Stronger correlation values between the 6MWT distance and the ankle-worn accelerometry units. No correlation identified in the DM1 group for the wrist-worn devices [142]

High intra-accelerometer reliability (p < 0.001). There was no inter-accelerometer reliability between wrist-worn devices and ankle-worn [142]

  

G-SensorVR, BTS Bioengineering, Italy

FD

All the spatio-temporal parameters of the sub-phases of the Timed up and go test (turning, standing-up and sitting-down from a chair) had a significantly higher duration in cervical dystonia patients compared to the controls [83]

   

PWS

PWS exhibited significantly reduced values of HR in the antero-posterior and vertical directions comparing to controls [109]

   

GT3X Actigraph, Manufacturing Technology, Inc./GT9X

HD

PA was lower in patients compared to controls [48]

   

Dermatomyositis

Patients with inactive disease had lower physical activity levels compared with controls [139]

Sedentary time was positively correlated with disease duration and negatively with VO2 at RCP and VO2peak. Moreover, MVPA time was negatively associated with disease duration and timed-up-and-go score and positively associated with time-to-RCP, VO2 at RCP, time-to-exhaustion, VO2peak and current use of glucocorticoid [143]

   

PWS

Youth with PWS spent 19.4% less time in weekly LPA and 29.8% less time in weekly VPA compared to controls [110]. Physical activity across intensity categories differed between study groups. Amount of activity was lower in all patients than controls and in non-ambulatory than ambulatory patients and controls, but similar between ambulatory patients and control [144]. The PWS group displayed lower PA and higher sedentary time compared to the control group [113]

  

MVPA and walking capacity increased after the programme without significant effect on body composition [113]

DMD

Strongest relationship between step activity and timed functional tests (particularly the 10-m walk/run) and moderate correlations with the 6 min walking distance, knee extensor peak torque, and plantar flexor peak torque. Moderate correlations between step activity and 10 m walk/run test, supine up, four stairs. Patients who were still ambulatory after 2 years demonstrated baseline step activity nearly double that of those who were no longer walking 2 years later [19]

   

QMT and accelerometry measures had a moderate or strong correlation, particularly indexed arm QMT with total wrist vector magnitude, total indexed QMT with total wrist vector magnitude and indexed leg QMT with total ankle vector magnitude [145]

 

DMD participants also demonstrated a progressive decrease in physical activity at 1- and 2-years for wrist accelerometry and at 2-years for ankle accelerometry (Killian et al. 2020)

 

ALS

Results were associated with ALSFRS-R. The variation in vertical axis showed the strongest correlation [28]

Less variability comparing to the ALSFRS-R (co-efficient of variation 0.64–0.81 for inertial outcomes) [28]

Activity declined by 0.64% per month [28]

 

The accelerometer models achieved a median multiclass AUC of 0.73 on six limb-related functions. The correlations across functions observed in self-reported ALSFRS-R scores were preserved in ML-derived scores [146]

  

In the cohort of 54 test participants who received edaravone as part of their usual care, the ML-derived scores were consistent with the self-reported ALSFRS-R scores. At the individual level, the continuous ML-derived score can capture gradual changes that are absent in the integer ALSFRS-R scores [146]

Sarcoidosis

Daily PA and VO2max were lower in sarcoidosis patients than the known predicted values in healthy age-matched individuals. Sedentary time was positively correlated with disease duration and negatively with VO2peak. MVPA was negatively associated with disease duration, and positively associated with VO2peak, and current use of corticoids [101]

   

IDEEA, Intelligent Device for Energy Expenditure and Activity; MinisunLLC, Fresno, CA

CMT

Count of step climbing and sit to stand were lower in patients than in controls as well as mean daily step-climbing and walking velocities. Positive correlation between strength of the knee extensor muscles and both count of steps climbed and sit to stand [147]

   

Ipod

HD

Amplitude of thoracic and pelvic trunk movements was significantly greater in participants with HD. Individuals with HD demonstrated rapid movements with varying amplitudes that continuously increased without stabilizing [66]

   

Jamar Plus Digital; JLW Instruments

DM1

Grip strength demonstrated strong correlations with self-reported inventories of upper [38]

   

JiBuEn gait analysis system

SCA

There were significant differences in stride length, velocity, supporting-phase percentage, and swinging- phase percentage between the SCA group and the gait control group. Negative correlation between Velocity and ICARS and SARA scores. Correlation between Midsagittal relative vermis diameter and ICARS and SARA scores, as well as stride velocity variability [90]

   

Kinesia motion sensor

FD

Measures of head tremor are logarithmically related to Tremor Rating Assessment Scale [82]

  

Minimum detectable change (percent reduction) was approximately 66% of the baseline geometric mean. That is comparable to those previously reported for hand tremor [82]

LabVIEW2011, National Instruments, Ireland

HD

Step time CoV was greater in manifest HD than controls, as was stride length CoV for late HD. Phase plot analysis identified differences between manifest HD and controls for SDB, Ratio and Δangleβ

DBS score was significantly associated with Ratio, SDB. UHDRS-TMS was significantly associated with Ratio , SDB, Δangleβ and step time CoV, cadence CoV, stride length, and stride length CoV. Ratio produced the strongest correlation with UHDRS-TMS [60]

There was no significant difference between tests for any measure (ICCs: speed 0.94, step time 0.89, step time CoV 0.67, cadence 0.72, cadence CoV 0.59, stride length 0.83 and stride length CoV 0.57) [60]

  

LEGSysTM, BioSensics, Newton, MA

SCA

Stride length variability, stride duration, cadence, stance phase, pelvis sway, and turn duration were different between SCA and controls. Sway and sway velocity of the ankle, hip, and center of mass differentiated SCA and controls. Stride length variability, stride duration, cadence, stance phase, pelvis sway, turn duration, sway and sway velocity of the ankle, hip, and center of mass showed moderate-to-strong correlation with SARA assessments of gait and stance and the BARS2 gait assessment [92]

   

The cycle detection technique showed an accuracy of 97.6% in a Bland–Altman analysis and a 94% accuracy in predicting the severity of the finger-to-nose test. Among the exctrated features, 22 showed an excellent correlation with finger-to-nose test and discriminated between SCA and control participants [148]

The results showed excellent intra-rater reliability as the ICC was in the range of 0.94–0.99 [148]

  

Locometrix®, Centaure Metrix, Evry, France

DM1

Patients displayed lower walking speed, stride frequency, stride length, gait regularity, and gait symmetry than controls. Strength of ankle plantar flexors, ankle dorsal flexors and neck flexors correlated with interstride regularity in the vertical direction. Knee extension strength correlated with gait symmetry in the anteroposterior direction. Center of pressure velocity was greater in patients and correlated with neck flexion and ankle plantar flexion weakness and with interstride regularity in the vertical direction [149]

   

MetaMotionR, mbientlabs, San Francisco, CA

ALS

Decreased stride length, increased stride duration and decreased walking speed were associated with lower functional walking scores, and the presence of a cane or walker [150]

   

Mimamori- GaitTM System; LSI Medience, Tokyo, Japan

PSP

Both PSP and PD patients shared the following similar hypokinetic gait characteristics: decreased velocity, step length, cadence and mean acceleration. Step time and variability in step time were mutually related. PSP patients showed characteristically low vertical displacement and a higher acceleration than PD patients at the same cadence [71]

   

Move 3 actigraphs (movisens GmbH; Karlsruhe, Germany)

TSC

Actigraph-measured movement was positively associated with ADHD and ASD symptoms. Higher ADHD symptoms and actigraph-measured movement levels were positively associated with ASD symptoms and negatively associated with IQ [126]

   

MOX Accelerometry; Maastricht Instruments BV, Maastricht, the Netherlands

DMD

Correlations with the PUL scale score were high for intensity and the total frequency of arm elevations per hour. Loderate correlation between number of transfers per hour and PUL scale score from low-middle and from middle-high. High correlation between the total number of transfers per hour and the PUL scale score [151]

   

Opal sensors, Mobility Lab, APDM, Portland, Oregon

PSP

The RF classifier allowed discrimination of PSP from PD with 86% sensitivity and 90% specificity, and PSP from HC with 90% sensitivity and 97% specificity [76]

   

Control subjects were able to change their postural strategy, whilst PSP and PD subjects persisted in use of an ankle strategy in all conditions. PD subjects had root mean square values similar to control subjects even without changing postural strategy appropriately, whereas PSP subjects showed much larger root mean square values than controls, resulting in several falls during the most challenging sensory organization test conditions [73]

   
  

A simple linear regression model incorporating the three features with the clearest progression pattern was able to detect statistically significant progression 3 months in advance of the clinical scores [77]

 

DMD

T-test results show that, for all age groups, children of the same age with DMD and controls show significant differences in RCC [20]

ICC of all groups exceeded 0.8 [20]

  

PWS

Children with Down syndrom and PWS exhibit reduced gait symmetry and higher accelerations at pelvis level than controls. While these accelerations are attenuated by about 40% at sternum level in controls and down syndrom, PWS children display significant smaller attenuations meaning reduced gait stability. Significant correlations between the estimated parameters and the GMFM-88 scale when considering the PWS group [152]

   

ALS

The ability to extend the head backward and flex it laterally were the most compromised, with significantly lower angular velocity, reduced smoothness and greater presence of coupled movements with respect to the controls [32]

ICC was moderate to good in all movements and for most parameters [32]

  

FSHD

  

For an average of 20.6 months, the iTUG duration stayed constant, whereas stride length, stride velocity, and trunk sagittal range of motion changed, indicating poorer performance. Arm swing changed in a compensatory direction toward the normative mean [153]

 

Gait parameters in FSHD participants were significantly altered compared with normative values. Stride velocity and trunk sagittal range of motion had moderate to strong correlations to other FSHD disease measures [154]

Reliability was excellent (ICC 0.84–0.99) [154]

  

HD

Gait speed, stride length, lateral step variability, and stride length variability were consistently observed to be significantly different between the HD and control. HD participants demonstrated significantly greater dual-task cost for turning. Poorer performance on the SDMT and animal naming was significantly associated with increased gait variability. UHDRS-TMS were correlated with percent of time spent in swing phase, stride length CoV and percent of time spent in double support for all three conditions [57]

   

Postural sway and control differed between patients with HD and patients with premanifest HD. Selected postural measures had positive correlations with CAP scores and TMS [65]

All measures of interesthad good reliability (ICC 0.764–0.887), except Total Power AP for patients with premanifest HD that had moderate reliability (ICC: 0.644) [65]

  

Individuals with HD had greater APA acceleration amplitudes, smaller first step ROM and longer first step durations compared to controls. APA ML amplitude and duration under the no-load condition were significantly correlated. APA ML amplitudes under cognitive-load condition were significantly correlated with TFC and TMC. APA ML amplitudes under no-load condition were significantly correlated with TMS and SDMT. Anticipatory postural adjustment evaluation could predict gait speed [61]

   

The 90.5% of subjects was assigned to the right group after leave-one-subject–out cross validation and majority voting [68]

   

Individuals with HD had a greater increase in standing postural sway compared to controls from single to dual-tasks and with changes to support surface. Patients with HD showed a greater dual-task motor cost compared to controls [66]. Total sway, root mean square and mean velocity during sitting, as well as gait speed had the greatest importance in classifying disease status. Stepwise regression showed that root mean square during standing with feet apart significantly predicted clinical measure of chorea, and ordinal regression model showed that root mean square and total sway standing feet together significantly predicted clinical measure of tandem walking [61]

   

FXS

FXS participants had reduced stride length and velocity, swing time, and peak turn velocity and greater double limb support time and number of steps to turn compared to controls under all conditions. Stride length variability was increased and cadence was reduced in FXS participants in the fast pace condition. There was greater dual task cost on peak turn velocity in men with FXTAS compared to controls [97]

   

SCA

Larger variability of the swing period, toe-off angle and toe-out angle in pre-SCA2, and larger mean coronal and transverse ranges of motion of the trunk at the lumbar location and of the sagittal range of motion of the trunk at the sternum location compared to controls. During tandem gait, pre-SCA2 subjects showed larger lumbar, trunk, and arm ranges of motion than controls. The toe-off angle and swing time variability were significantly correlated with the time to ataxia onset, whereas the toe-off angle and transverse range of motion at trunk position during tandem gait were significantly associated with the SARA score [93]

   

Increased gait variability was the most discriminative gait feature of SCA; toe-out angle variability (sensitivity = 0.871; specificity = 0.896) and double-support time variability (sensitivity = 0.834; specificity = 0.865) were the most sensitive and specific measures. These variability measures were also significantly correlated with the scale for the assessment and rating of ataxia and disease duration. The same gait measures discriminated gait of people with prodromal SCA from the gait of controls [91]

   

Lateral velocity change (LVC) and outward acceleration but not general turning measures such as speed, allowed differentiating ataxic against healthy subjects in real life, with LVC also differentiating preataxic against healthy subjects. LVC was highly correlated with SARA score, and activity-specific balance confidence scale [89]

  

Moreover, LVC in real life allowed detecting significant longitudinal change in 1-year follow-up with high effect size [89]

CMT

Five mean gait outcomes measured, four showed statistically significant changes over the 6-min fast-as-possible walk: velocity, cadence, step time and trunk ROM. Stride length variability changed during the walking task, decreasing from bins 1–2, and remaining stable for bins 2–6. Changes in velocity, cadence, step time were related to general life satisfaction, but not perceived fatigue [36]

   

PAMSys-XTM sensors, BioSensics, Cambridge, MA

HD

In the clinic, the standard deviation of step time was increased in HD compared to controls. At home, significant differences were observed in seven additional gait measures (cadence, maximum step peak acceleration, maximum medial–lateral velocity, maximum medial–lateral displacement, average step peak acceleration, average medial–lateral velocity and displacement). The gait of individuals with higher TMS differed significantly from those with lower TMS on multiple measures at home [50]

   

Philips Respironics, Bend, OR

NP-C

Significant correlations were demonstrated between BK25, BK50 and BK75. FDS correlated with PDQ, UPDRS IV, UPDRS and AIMS. DK25 in comparison with NUCOG-A and DK75 in comparison with NUCOG and NUCOG-A demonstrated significant correlations. Additionally, duration of illness in comparison with PTI demonstrated significance [120]

   

Philips Respironics, Bend, OR

SBMA

   

Patients with higher AMAT subscore increased total activity count comparing to controls after intervention [45]

RehaGait (HAS-OMED, Magdeburg, Germany)

HD

   

Device-extracted parameters revealed significant improvement in area, velocity, acceleration and jerkiness of sway in cerebellar repetitive Transcranial Magnetic Stimulation versus sham stimulation [74]

SAM (Modus Health LLC, Washington, DC, USA)

Rett syndrome

 

Repeatability of step-count pairs was excellent (ICC 0.91, 95%). The standard error of measurement was 6 steps/min and we would be 95% confident that a change more than 17 steps/min would be greater than within-subject measurement error [125]

  
   

After intervention, the sedentary time was decreased. Positive effects with small to medium effect sizes were seen in sedentary time and that was maintained during follow-up period [122]

SenseWear Armband, BodyMedia, Inc., Pitts burgh, PA, USA

Sarcoidosis

There was a significant correlation with SGRQ score, SF-12 physical health, Physical fatigue and reduced activity MFIS subscores, 6MWD [103]

   

Scleroderma

Exercise capacity during daily activity was reduced compared with controls, and was associated with early evidence of functional decay [107]

   

CMT

Results showed a decrease in daily steps taken in the CMT group, but shorter bouts of sedentary activity and more frequent transitions from sedentary to active behaviors compared to controls [155]

   

SHIMMER sensors, Shimmer Research Ltd., Dublin, Ireland

HD

Stride length and gait velocity were reduced, while stride and stance time were increased in patients with HD. Parameters reflecting gait variability were substantially altered in HD patients (stride length CV and stride time CV). Parameters representing gait variability (stride time CV, stance time CV, swing time CV, stride length CV, gait velocity CV) showed moderate to strong correlations to UHDRS-TMS. Stride length and gait velocity showed moderate inverse correlations to UHDRS-TMS [156]

   

PSP

Gait speed was significantly reduced in patients with PD compared to controls and even more in atypical PD. Similar results were obtained for stride length. The maximum toe clearance and heel strike angle, toe off angles were significantly impaired in PD and atypical PD patients compared to controls but did not reveal a significant difference between both patient cohorts. Clinical ratings significantly correlated with gait speed and stride length [72]

   

HSP

There were significant associations of absolute stride parameters with single SPRS items reflecting impaired mobility, with patients’ quality of life, and notably with disease duration. Sensor-derived coefficients of variation, on the other hand, were associated with patient-reported fear of falling and cognitive impairment [96]

 

In a small 1-year follow-up analysis of patients with complicated HSP and fast progression, the absolute values of mobile gait parameters had significantly worsened compared with baseline. [96]

 

SilmeeTM Bar-type Light, manufactured by Toshiba Corporation, Tokyo, Japan)

DMD

The Cj values had significant and very strong or strong correlations with the Brooke Upper Extremity Scale and the arm function scores for the DMD Functional Ability Self-Assessment Tool. The values also had a very strong or strong correlation with the elbow flexion strength [157]

   

Smartwatch

HD

There was a significant correlation between the model chorea score and the patient-reported chorea score for the same assessment [46]

   

Step WatchTM Activity monitor (SAM), Cyma Inc. Seattle, USA

DMD

Significant correlations for 10-m walk/run versus high and low stride rates were found at baseline. Changes in strides/day and percentages of high frequency and low frequency strides correlated significantly with changes in 10-m walk/run speed [158]

 

There were significant declines in average strides/day and percent strides at moderate, high and pediatric high rates as a function of age. Step activity outcomes were sensitive to change over 1 year, but the direction and parameter differed by age group [158]

 

DMD

StepWatch accelerometry identified a decreased capacity for ambulation in boys with Duchenne compared to healthy controls. There were strong, significant correlations between 6-min walk distance and all StepWatch parameters [23]

   

SCA

The objective monitor measurements were highly associated with disease duration and with the functional stage of disease. Monitor measurements were significantly correlated to SARA scores with the exception of the percent of steps expended in moderate and high speeds of activity. Fewer monitor measures had significant correlations with walk and peg board scores [159]

The objective SAM outputs also possessed high internal consistency, high ICC and could be fitted to a single factor by factor analysis (Subramony et al. 2012)

  

CMT

Minutes at low activity average, step at low and high activity average, peak activity index average was correlate with myometer measures. Minutes high activity average correlate with myometer measures and the Short Form 36 Physical Composite Score. Some Sustained Activity measures correlated with CMT Examination Score, myometer measures and Short Form 36 Physical Composite Score [128]

Test–retest for both the 6MWT and SAM demonstrated excellent reliability with a value > 0.90 obtained comparing the two evaluations [128]

Statistical analysis showed a worsening of the StepWatchTM Activity Monitor outputs [129]

 

Roche HD Digital Monitoring

HD

Good overall convergent validity of sensor-derived features to Unified HD Rating Scale outcomes and good overall known-groups validity among controls, premanifest, and manifest participants were observed. [55]

All sensor-based features showed good to excellent test–retest reliability (ICC 0.89–0.98) [55]

  

wGT3X-BT, Timik Medical, Herlev, Denmark

DM1

The individuals with DM1 were less physically active compared to healthy controls [160]

   
  1. DMD Duchenne muscular dystrophy, BBT block and box test, ICC intraclass correlation coefficient, SMA spinal muscular atrophy, MFM motor function measure, FSHD facioscapulohumeral dystrophy, MMT manual muscle testing, HD Huntington disease, FRDA Friedrich Ataxia, FD Focal Dystinia, PD Parkinson disease, UHDRS Unified Huntington's Disease Rating Scale, TMS total motor score, MPS mucopolysaccharidosis, TWSTRS Toronto Western Spasmodic Torticollis Rating Scale, MG myasthenia gravis, PA physical activity, MVPA moderate-to vigorous physical activity, PAL physical activity level, ALS amyotrophic lateral sclerosis, ALSFRS-R Revised Amyotrophic Lateral Sclerosis Functional Rating Scale, DM myotonic dystrophy, PWS Prader–Willi syndrome, HR harmonic ratio, LPA light physical activity, MPA moderate physical activity, VPA vigourous physical activity, QMT quantitative muscle testing, CMT Charcot-Marie-Tooth, SCA spinocerebellar ataxia, ICARS International Cooperative Ataxia Rating Scale, SARA Scale for the Assessment and Rating of Ataxia, BARS2 Brief Ataxia Rating Scale, PSP progressive supranuclear palsy, TSC tuberous sclerosis complex, ADHD Attention deficit hyperactivity disorder, ASD autism spectrum disorder, IQ intelligence quotient, CoV coefficient of variation, PUL performance of upper limb, GMFM gross motor function measure, TUG timed up and go test, SDMT symbol digit modalities test, CAP CAG-age-product, Total Functional Capacity Score, FXS Fragile X Syndrome, NP-C Niemann–Pick type C, UPDRS Unified Parkinson's Disease Rating Scale, AIMS Abnormal Involuntary Movement Scale, NUCOG neuropsychiatry unit cognitive assessment tool, CV coefficient of variation, HSP hereditary spastic paraplegia, SPRS Spastic Paraplegia Rating Scale