Real-life Wrist Movement Patterns Capture Motor Impairment in Individuals with Ataxia-Telangiectasia

Abstract

Sensitive motor outcome measures are needed to efficiently evaluate novel therapies for neurodegenerative diseases. Devices that can passively collect movement data in the home setting can provide continuous and ecologically valid measures of motor function. We tested the hypothesis that movement patterns extracted from continuous wrist accelerometer data capture motor impairment and disease progression in ataxia-telangiectasia. One week of continuous wrist accelerometer data were collected from 31 individuals with ataxia-telangiectasia and 27 controls aged 2–20 years old. Longitudinal wrist sensor data were collected in 14 ataxia-telangiectasia participants and 13 controls. A novel algorithm was developed to extract wrist submovements from the velocity time series. Wrist sensor features were compared with caregiver-reported motor function on the Caregiver Priorities and Child Health Index of Life with Disabilities survey and ataxia severity on the neurologist-performed Brief Ataxia Rating Scale. Submovements became smaller, slower, and less variable in ataxia-telangiectasia compared to controls. High-frequency oscillations in submovements were increased, and more variable and low-frequency oscillations were decreased and less variable in ataxia-telangiectasia. Wrist movement features correlated strongly with ataxia severity and caregiver-reported function, demonstrated high reliability, and showed significant progression over a 1-year interval. These results show that passive wrist sensor data produces interpretable and reliable measures that are sensitive to disease change, supporting their potential as ecologically valid motor biomarkers. The ability to obtain these measures from a low-cost sensor that is ubiquitous in smartwatches could help facilitate neurological care and participation in research regardless of geography and socioeconomic status. Supplementary Information The online version contains supplementary material available at 10.1007/s12311-022-01385-5.

Assessment of upper limb movement disorders using wearable sensors during functional tasks: a systematic review

“…Current overview can support clinicians and researchers to select the most sensitive pathology-dependent sensor features and measurement methodologies for detection and quantification of upper limb movement disorders and for the objective evaluations of treatment effects. Accelerometer PD [63,89,96], dystonia [140] , ataxia [134], MS [127]…”

“…Twelve studies included patients with essential tremor [102][103][104][105][106][107][108][109][110][111][112][113] and 11 included adults post-stroke [15,28,[114][115][116][117][118][119][120][121][122], whereas six included adults with MS [123][124][125][126][127][128]. One study included adults with HD and eight studies included children or adults with ataxia [29,[129][130][131][132][133][134][135][136]. Five studies included children with CP while two studies included children with dystonia and spasticity, respectively [12,13,[137][138][139][140][141] (Figure 2).…”

“…Teeth brushing was included in four studies [52,68,77,84] as well as putting clothes on/off [47,48,77,87]. In stroke, the Wolf Motor Function test or parts of this clinical scale were included four times [115,[118][119][120] and four studies measured activities in an unrestricted home environment [63,89,134,139]. Combing hair was included in three studies [52,77,87] as well as typing and folding laundry [76,77,86] and forwards and sideways reaching [12,114,116].…”

“…For the studies where both acceleration and angular velocity signals were collected, both mean and standard deviation (STD) were often calculated [62,78,86,87,90,118,129,139], as well as rootmean-square (RMS) values [11,86,89,129,139]. Additionally, mean and RMS or STD of acceleration and angular velocity separately were used in studies were one of the signals was available [29,50,52,54,57,63,64,66,71,74,82,83,85,89,96,104,105,108,110,111,115,119,120,[132][133][134]137], as well as median angular velocity in one study [82]. Maximal linear velocity was additionally often used as key feature, mostly by integration of the acceleration signal [51,54,115].…”

“…The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. Accelerometer PD [63,89,96], dystonia [140] , ataxia [134], MS [127]…”