A powered neck exoskeleton for ALS neck weakness

About This Grant

PROJECT SUMMARY Amyotrophic lateral sclerosis (ALS) is a rare but extremely debilitating neurodegenerative disease. Weakness starts focally and progresses to involve all regions, and finally respiration. Neck weakness is common and problematic. Although static neck collars are prescribed, most patients do not use them due to discomfort and ineffectiveness. While neck weakness is an important marker for intervention planning and design of clinical trials, measuring neck strength can only be completed by physicians in clinics; and current methods suffer from rater subjectivity. By contrast, a powered neck exoskeleton was developed to restore head-neck motions and adapt to patient’s needs as ALS progresses. It also has the potential to objectively measure neck strength remotely. However, the extent to which this powered neck exoskeleton improves daily functions, how it adapts to patients’ needs as ALS progresses, and how reliable it is to measure neck strength are yet to be determined, which are major roadblocks for clinical translation. The long-term goal is to improve the clinical care and quality of life of patients with ALS. The overall objective is to determine the efficacy of the powered neck exoskeleton to restore complex neck function to ALS patients and to quantify neck muscle weakness using the powered neck exoskeleton. The central hypothesis is that ALS patients with neck muscle weakness will gain greater function for a wide range daily activities, that their neck muscle strength can be quantified reliably, and that their complex needs will be met as ALS progresses. The rationale is that demonstrating the benefits of a powered neck exoskeleton for daily use in ALS and providing a measure outside the clinic for neck muscle strength will provide the necessary scientific justification for the clinical use of the neck exoskeleton for ALS, ultimately providing a better standard of care. The overall objective will be attained by pursuing three specific aims: 1) determine ability to perform daily tasks in ALS patients with neck weakness, 2) validate the ability of the neck exoskeleton to estimate neck muscle strength, and 3) quantify adaptability of robotic assistance as ALS progresses. In aim 1, change of head range of motion from baseline without a device will be compared between using the neck exoskeleton and a gold-standard static neck collar. In aim 2, the neck strength of ALS patients will be estimated by the neck exoskeleton and compared to a clinical standard method (hand-held dynamometry). In aim 3, the trajectory of change of head range of motion will be compared using the neck exoskeleton and the static neck collar longitudinally. This research is innovative, as it presents a clear departure from the status quo for treating and monitoring ALS neck weakness and the previous technology-focused investigations of the principal investigator. This research is significant, because it is expected to provide the scientific justification for using the neck exoskeleton technology to optimize ALS care, as well as support future pivotal at-home clinical trials. Ultimately, such knowledge has the potential to offer new opportunities to provide better care for ALS.