Open AccessObjective Assessment of Progression and Disease Characterization of Friedreich Ataxia via an Instrumented Drinking Cup: Preliminary Results
Author(s) -
Ragil Krishna,
Pubudu N. Pathirana,
Malcolm K. Horne,
David J. Szmulewicz,
Louise A. Corben
Publication year - 2021
Publication title -
ieee transactions on neural systems and rehabilitation engineering
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.093
H-Index - 140
eISSN - 1558-0210
pISSN - 1534-4320
DOI - 10.1109/tnsre.2021.3124869
Subject(s) - bioengineering , computing and processing , robotics and control systems , signal processing and analysis , communication, networking and broadcast technologies
The monitoring of disease progression in certain neurodegenerative conditions can significantly be quantified with the help of objective assessments. The severity assessment of diseases like Friedreich ataxia (FRDA) are usually based on different subjective measures. The ability of a participant with FRDA to perform standard neurological tests is the most common way of assessing disease progression. In this feasibility study, an Ataxia Instrumented Measurement-Cup (AIM-C) is proposed to quantify the disease progression of 10 participants (mean age 39 years, onset of disease 16.3 years) in longitudinal timepoints. The device consists of a sensing system with the provision of extracting both kinetic and kinematic information while engaging in an activity closely associated with activities of daily living (ADL). A common functional task of simulated drinking was used to capture features that possesses disease progression information as well as certain other features which intrinsically correlate with commonly used clinical scales such as the modified Friedreich Ataxia Rating Scale (mFARS), the Functional Staging of Ataxia score and the ADL scale. Frequency and time-frequency domain features allowed the longitudinal assessment of participants with FRDA. Furthermore, both kinetic and kinematic measures captured clinically relevant features and correlated 85% with clinical assessments.