Abstract
Quantitative assessment of movement impairment in Huntington’s disease (HD) is essential to monitoring of disease progression. This paper aimed to develop and validate a novel low cost, objective automated system for the evaluation of upper limb movement impairment in HD in order to eliminate the inconsistency of the assessor and offer a more sensitive, continuous assessment scale. Patients with genetically confirmed HD and healthy controls were recruited to this observational study. Demographic data, including age (years), gender, and unified HD rating scale total motor score (UHDRS-TMS), were recorded. For the purposes of this paper, a modified upper limb motor impairment score (mULMS) was generated from the UHDRS-TMS. All participants completed a brief, standardized clinical assessment of upper limb dexterity while wearing a tri-axial accelerometer on each wrist and on the sternum. The captured acceleration data were used to develop an automatic classification system for discriminating between healthy and HD participants and to automatically generate a continuous movement impairment score (MIS) that reflected the degree of the movement impairment. Data from 48 healthy and 44 HD participants was used to validate the developed system, which achieved 98.78% accuracy in discriminating between healthy and HD participants. The Pearson correlation coefficient between the automatic MIS and the clinician rated mULMS was 0.77 with a p-value < 0.01. The approach presented in this paper demonstrates the possibility of an automated objective, consistent, and sensitive assessment of the HD movement impairment.
Highlights
HUNTINGTON’S disease (HD) is an autosomal dominant, progressive neurodegenerative genetic disorder, which affects 11.2 to 13.5 people per 100,000 of the general population
The Unified Huntington’s Disease Rating Scale (UHDRS) score does not relate motor impairment to function in daily life, which is desirable in HD assessment [6]
The results showed that the recurrence rate feature of the non-dominant hand X-axis was significant for discriminating between HD patients and healthy controls in all three Money Box Test (MBT) tasks
Summary
HUNTINGTON’S disease (HD) is an autosomal dominant, progressive neurodegenerative genetic disorder, which affects 11.2 to 13.5 people per 100,000 of the general population. HD is characterised by the development of progressive motor impairment, cognitive decline and behavioural problems [1], [2], caused by an expanded trinucleotide CAG sequence in the Huntingtin (HTT) gene [2][4]. A critical problem for the evaluation of novel therapeutics is the acknowledged lack of objective clinical measures suitable for evaluating the components of the movement disorder. The Unified Huntington’s Disease Rating Scale (UHDRS) [5] is currently the gold standard to assess disease symptoms in HD. The UHDRS score does not relate motor impairment to function in daily life, which is desirable in HD assessment [6]
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
More From: IEEE Transactions on Neural Systems and Rehabilitation Engineering
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.