Amyotrophic lateral sclerosis (ALS) is the most common subtype of motor neuron disease (MND). The current gold-standard measure of progression is the ALS Functional Rating Scale—Revised (ALS-FRS(R)), a clinician-administered questionnaire providing a composite score on physical functioning. Technology offers a potential alternative for assessing motor progression in both a clinical and research capacity that is more sensitive to detecting smaller changes in function. We reviewed studies evaluating the utility and suitability of these devices to evaluate motor function and disease progression in people with MND (pwMND). We systematically searched Google Scholar, PubMed and EMBASE applying no language or date restrictions. We extracted information on devices used and additional assessments undertaken. Twenty studies, involving 1275 (median 28 and ranging 6–584) pwMND, were included. Sensor type included accelerometers (n = 9), activity monitors (n = 4), smartphone apps (n = 4), gait (n = 3), kinetic sensors (n = 3), electrical impedance myography (n = 1) and dynamometers (n = 2). Seventeen (85%) of studies used the ALS-FRS(R) to evaluate concurrent validity. Participant feedback on device utility was generally positive, where evaluated in 25% of studies. All studies showed initial feasibility, warranting larger longitudinal studies to compare device sensitivity and validity beyond ALS-FRS(R). Risk of bias in the included studies was high, with a large amount of information to determine study quality unclear. Measurement of motor pathology and progression using technology is an emerging, and promising, area of MND research. Further well-powered longitudinal validation studies are needed.

Background. The aims of this study were to assess the health-related quality of life (HRQoL) of patients awaiting anterior cruciate ligament (ACL) reconstruction compared to the population at risk: whether knee-specific function was predictive of HRQoL and to identify factors associated with a worse HRQoL. Methods. Sixty-seven patients (male, n = 50; female, n = 17; mean age, 29) identified from the surgical waiting list completed a questionnaire that included demographics, BMI, time of injury, EuroQol 5-dimension (EQ-5D), Short-Form (SF-36), and International Knee Documentation Committee (IKDC) scores. Age- and sex-matched HRQoL data were obtained from population level data. Results. The mean EQ-5D score for the study cohort was significantly worse than the matched score (difference, 0.367; p < 0.001 ), and the same trend was observed for all eight dimensions of the SF-36 score. Thirty-three (49%) patients felt their health, in general, was somewhat or much worse compared to one year ago. There was a correlation between IKDC and EQ-5D scores (r = 0.540, p < 0.001 ). Linear regression was used to formulate the EQ-5D score: EQ-5D = (IKDCx0.013)–0.015 (constant). The SF-36 physical component and length of time on the waiting list were independently associated with the HRQoL, with each 14-point drop or for every 200 days, a clinically significant deterioration in patients’ HRQoL occurred, respectively. Conclusions. Patients had a significantly worse HRQoL when compared to the age- and sex-matched population, which deteriorates with worsening physical function and increasing length of time on the waiting list. The knee-specific IKDC correlated with HRQoL and could be used to estimate the EQ-5D score.