To develop a standardized, clinically-validated digital solution for rigorous, longitudinal assessment of ocular weakness and dysarthria in myasthenia gravis (MG), which can be applied remotely.
MG is a chronic autoimmune neuromuscular disease characterized by fluctuating weakness of skeletal muscle, which frequent affects ocular muscles and speech. Current assessments of myasthenic weakness require neurologic expertise, are time-consuming and can still be subjective. Additionally, measured at one point in time, assessments provide a snapshot of a patient’s MG, which may not adequately reflect the spectrum of fluctuating weakness. Ratings of ocular weakness and dysarthria are particularly subjective. These shortcomings of current outcome measures limit performance of clinical trials and provide a limited view of the patient’s clinical status.
A pilot study with a target sample size of 20 individuals with MG is ongoing to determine the feasibility of using the proposed solution as a health monitoring platform for MG. Two MG experts will rate speech and video data for dysarthria and ptosis based on the Quantitative Myasthenia Gravis (QMG) scale. Association between the extracted speech and facial characteristics to clinical scores of dysarthria and ptosis will be investigated.
Despite considerable recent advances in treatments for MG, rigorous, longitudinal tools for monitoring of its characteristic fluctuating weakness remain limited and represent an unmet need. The feasibility, validity and acceptability of the proposed digital solution will be determined for 20 patients with MG. If successful, this technology will be further developed and studied in a larger observational study.