CoMoN Research Project

Design and development of digital tools for the management of Huntington patients

Group :  Information, Signal, Image and Life Sciences

Labelling: none

Duration: 2 years (2020 - 2022)

Funding: SAM network project

Staff involved from LARIS: Paul Richard, Anne Humeau-Heurtier, Fabrice Guérin,

Project partners: Sylvain Durand (EA 4334 MIP -Le Mans University), Philippe Allain (EA 4638 LPP - Department of Neurology, CHU Angers)

Context :

In the absence of curative medical treatments, physical exercise and/or cognitive stimulation are now relevant approaches for the management of symptoms associated with progressive neurological diseases [1]. Huntington's disease (HD) is one of these diseases and is probably one of the most rapidly disabling in terms of function. It is a rare hereditary neurodegenerative disease of the central nervous system that usually manifests itself around the age of 40-50 years, resulting in motor disorders (choreic movements, balance disorders, walking disorders, etc.), cognitive disorders (executive functions, attention, working memory, etc.) and emotional and behavioural regulation. These disorders progressively worsen until a severe grabatisation and cognitive deterioration. To date, a few rare studies have shown the feasibility and interest of cognitive stimulation in these patients to slow intellectual decline, encouraging further trials [2]. A few studies have shown that short physical activity programmes (8-16 weeks) are feasible, but without significant effects on disease-related motor symptoms, inviting more intensive and longer clinical trials [3]. To date, no work has examined the effects of a sustained management programme, combining physical exercise and cognitive stimulation, on symptomatology and functional autonomy in patients with early to moderate HD.

Main objective:

To study the feasibility and effects of a management programme combining physical activities and cognitive stimulation on the deficits of patients with symptomatic MH stage 1 or 2 by comparing (inter- and intra-group comparison) 2 groups of patients with MH treated with this programme on the scores obtained on the motor scales (UHDRS motor, timed up and go test, 30 second chair stand test), functional (functional capacity scale, functional appreciation scale, dependency scale), cognitive (global efficiency, executive functions), affective (anxiety and depression), behavioural (Problem behaviours assessment) and quality of life (SF12) scales proposed as part of the annual follow-up of the patients (Enroll-HD protocol)

Project references:

[1] McEwen SC, Siddarth P, Rahi B, Kim Y, Mui W, Wu P, Emerson ND,  Lee J, Greenberg S, Shelton T, Kaiser S, Small GW, Merrill DA. Simultaneous Aerobic Exercise and Memory Training Program in  Older Adults with Subjective Memory Impairments. J Alzheimers  Dis. 2018;62(2):795-806. doi: 10.3233/JAD-170846.
[2] Sadeghi M, Barlow-Krelina E, Gibbons C, Shaikh KT, Fung WLA, Meschino WS, et al. Feasibility of computerized working memory training in individuals with Huntington disease. PLoS ONE 2017;12(4): e0176429. doi.org/10.1371/journal.pone.0176429
[3] Playle R, Dimitropoulou P, Kelson M, Quinn L1, Busse M. Exercise Interventions in Huntington's Disease: An Individual Patient Data Meta-Analysis. Mov Disord Clin Pract. 2019;18;6(7):567-575. doi: 10.1002/mdc3.12809