Remote Monitoring of Physical Activity and Upper Limb Function in Adults with Charcot-Marie-Tooth Disease

Topic:

Other

Poster Number: O382

Author(s):

Kayla Cornett, PhD, University of Sydney School of Health Sciences, Faculty of Medicine and Health, Sydney, Australia, Ram Kinker Mishra, PhD, BioSensics, Newton MA, Adonay Nunes Sastre, PhD, BioSensics, Newton MA, Kayla Clem, DPT, Neurology & Neuromuscular Care Center, Denton TX, Timothy Estilow, OTR/L, Children’s Hospital of Philadelphia, Philadelphia PA, Joy Aldrich, Hereditary Neuropathy Foundation, New York NY, Courtney Hollett, Hereditary Neuropathy Foundation, New York NY, Allison Moore, Hereditary Neuropathy Foundation, New York NY, Ashkan Vaziri, PhD, BioSensics, Newton MA

Background
Clinical Outcome Assessments are important for measuring disease severity and evaluating the effectiveness of therapeutic interventions. Wearable Sensors offer continuous monitoring providing real-world evidence of function in a home environment. Validating wearable sensors could enhance our ability to track disease progression and response to treatment as therapies should benefit individuals in their daily environment.
Methods
This cross-sectional cohort study was performed at the Hereditary Neuropathy Foundation CMT Clinical Trial Readiness Summit. All participants completed the CMT-FOM. Physical activity and upper limb activity were monitored continuously for 14 days at home using PAMSysTM pendant and wrist sensors (BioSensics, Newton MA). Sensor-derived metrics were compared with the CMT-FOM.
Results
26 Participants (19-70yrs, 81% female, 58% CMT1A) participated in this study. Participants ranged from mildly to severely affected as measured by the CMT-FOM (53.2 ± 8.0, range: 37 – 73). Compliance with PAMSys pendant and wrist sensors was excellent with mean non-compliance of 22 minutes per day. Disease severity, measured by the CMT-FOM was significantly correlated with daily step counts (ρ=-0.608,p=0.001), cadence (ρ=-0.579, p=0.002) and postures (p<0.05). Grip strength z-scores were correlated with acceleration zero crossings average duration (ρ=-0.571, p=0.003), acceleration zero crossings duration entropy (ρ=0.439, p=0.028), and velocity zero crossings count (ρ=-0.500, p=0.011) showing that individuals with weaker grip strength have less smooth goal directed movements with more sub-movements. However, the number of goal directed movements was not correlated with strength, fine motor function or overall disease severity (p>0.05)
Conclusion
Participants were compliant with the wearable pendant sensors. This is the first study to evaluate upper limb function using wearable devices in the home environment in individuals with CMT. These wearables provided real-world evidence by continuously capturing long-term data that reflects patients’ natural environments and daily activities. Sensor-derived metrics were correlated with established COAs.