A Road Map for Remote Digital Health Technology for Motor Neuron Disease

Eijk, Ruben P A van; Beelen, Anita; Kruitwagen, Esther T.; Murray, Deirdre; Radaković, Ratko; Hobson, Esther; Knox, Liam; Helleman, Jochem; Burke, Tom; Rubio, Miguel Ángel; Reviers, Evy; Genge, Angela; Steyn, Frederik J.; Ngo, Shyuan T.; Eaglesham, John; Roes, Kit C. B.; Berg, Leonard H. van den; Hardiman, Orla; McDermott, Christopher J.

doi:10.2196/28766

Cited by 27 publications

(28 citation statements)

References 43 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…A further potential challenge for researchers incorporating devices in trial design, and clinicians integrating devices into care, is how to use device data to evaluate progression. A clear, pre-defined plan for evaluating digitally derived data, correlating with existing validated outcomes and determining thresholds of progression are crucial [ 44 ].…”

Section: Discussionmentioning

confidence: 99%

A systematic review of digital technology to evaluate motor function and disease progression in motor neuron disease

et al. 2022

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionmentioning

confidence: 99%

A systematic review of digital technology to evaluate motor function and disease progression in motor neuron disease

et al. 2022

View full text Add to dashboard Cite

show abstract

“…In turn, this may result in earlier detection of a respiratory function decline, and more timely referral to a pulmonologist or initiation of NIV, compared to the usual 3 monthly in-clinic care. This study contributes to the recently published Road Map, which was created to facilitate the wide-scale adoption of digital technology and remote monitoring in MND, as it provides evidence on how to measure respiratory function in patients with MND [ 31 ].…”

Section: Discussionmentioning

confidence: 99%

Home-monitoring of vital capacity in people with a motor neuron disease

et al. 2022

Self Cite

View full text Add to dashboard Cite

Background Home-monitoring of spirometry has the potential to improve care for patients with a motor neuron disease (MND) by enabling early detection of respiratory dysfunction and reducing travel burden. Our aim was to evaluate the validity and feasibility of home-monitoring vital capacity (VC) in patients with MND. Methods We included 33 patients with amyotrophic lateral sclerosis, progressive muscular atrophy or primary lateral sclerosis who completed a 12-week home-monitoring protocol, consisting of 4-weekly unsupervised home assessments of VC and a functional rating scale. At baseline, during a home visit, patients/caregivers were trained in performing a VC test, and the investigator performed a supervised VC test, which was repeated at final follow-up during a second home visit. Validity of the unsupervised VC tests was evaluated by the differences between supervised and unsupervised VC tests, and through Bland–Altman 95% limits-of-agreement. Feasibility was assessed by means of a survey of user-experiences. Results The 95% limits-of-agreement were [− 14.3; 11.7] %predicted VC, and 88% of unsupervised VC tests fell within 10%predicted of supervised VC. 88% of patients experienced VC testing as easy and not burdensome, however, 15% patients did not think their VC test was performed as well as in the clinic. 94% of patients would like home-monitoring of VC in MND care. Discussion Unsupervised VC testing at home, with prior face-to-face training, is a valid and time-efficient method for the remote monitoring of respiratory function, and well-accepted by patients with MND and their caregivers.

show abstract

“…Data privacy and CE marking of novel devices or algorithms must also be taken into consideration [ 103 ]. Adoption of any new device is dependent on the strategies surrounding the CE mark and operational aspects, which reflect decisions that need to be taken early in the development of a device.…”

Section: Discussionmentioning

confidence: 99%

Measurement of upper limb function in ALS: a structured review of current methods and future directions

et al. 2022

View full text Add to dashboard Cite

Measurement of upper limb function is critical for tracking clinical severity in amyotrophic lateral sclerosis (ALS). The Amyotrophic Lateral Sclerosis Rating Scale-revised (ALSFRS-r) is the primary outcome measure utilised in clinical trials and research in ALS. This scale is limited by floor and ceiling effects within subscales, such that clinically meaningful changes for subjects are often missed, impacting upon the evaluation of new drugs and treatments. Technology has the potential to provide sensitive, objective outcome measurement. This paper is a structured review of current methods and future trends in the measurement of upper limb function with a particular focus on ALS. Technologies that have the potential to radically change the upper limb measurement field and explore the limitations of current technological sensors and solutions in terms of costs and user suitability are discussed. The field is expanding but there remains an unmet need for simple, sensitive and clinically meaningful tests of upper limb function in ALS along with identifying consensus on the direction technology must take to meet this need.

show abstract

A Road Map for Remote Digital Health Technology for Motor Neuron Disease

Cited by 27 publications

References 43 publications

A systematic review of digital technology to evaluate motor function and disease progression in motor neuron disease

A systematic review of digital technology to evaluate motor function and disease progression in motor neuron disease

Home-monitoring of vital capacity in people with a motor neuron disease

Measurement of upper limb function in ALS: a structured review of current methods and future directions

Contact Info

Product

Resources

About