Objective assessment of walking impairments in myotonic dystrophy by means of a wearable technology and a novel severity index

Myotonic dystrophy type 1 (DM1) is a genetic inherited autosomal dominant disease characterized by multisystem involvement, including muscle, heart, brain, eye, and endocrine system. Although several methods are available to evaluate muscle strength, endurance, and dexterity, there are no validated outcome measures aimed at objectively evaluating qualitative and quantitative gait alterations. Advantageously, wearable sensing technology has been successfully adopted in objectifying the assessment of motor disabilities in different medical occurrences, so that here we consider the adoption of such technology specifically for DM1. In particular, we measured motor tasks through inertial measurement units on a cohort of 13 DM1 patients and 11 healthy control counterparts. The motor tasks consisted of 16 meters of walking both at a comfortable speed and fast pace. Measured data consisted of plantar-flexion and dorsi-flexion angles assumed by both ankles, so to objectively evidence the footdrop behavior of the DM1 disease, and to define a novel severity index, termed SI-Norm2, to rate the grade of walking impairments. According to the obtained results, our approach could be useful for a more precise stratification of DM1 patients, providing a new tool for a personalized rehabilitation approach.