Cognitive Reserve in Early Manifest Huntington Disease Patients: Leisure Time Is Associated with Lower Cognitive and Functional Impairment

Journal of Personalized Medicine

Silvia Romano¹  Giovanni Ristori¹  Giulia D’Aurizio²  Giuseppe Curcio²  Mario Fichera³  Anna Castaldo³  Sabrina Maffi⁴  Ferdinando Squitieri⁴  Eugenia Scaricamazza⁴  Simone Migliore⁴  Consuelo Ceccarelli⁵ 

[1] Centre for Experimental Neurological Therapies, Department of Neuroscience, Mental Health and Sensory Organs, Faculty of Medicine and Psychology, S. Andrea Hospital, Sapienza University, 00189 Rome, Italy;Department of Biotechnological and Applied Clinical Sciences, University of L’Aquila, 67100 L’Aquila, Italy;Department of Medical Genetics and Neurogenetics, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy;Huntington and Rare Diseases Unit, Fondazione IRCCS Casa Sollievo Della Sofferenza Hospital, 71013 San Giovanni Rotondo, Italy;Italian League for Research on Huntington and Related Diseases Foundation, 00185 Rome, Italy;

关键词: cognitive engagement;    neurodegenerative;    executive functions;    cognition;   

DOI  :  10.3390/jpm12010036

来源: DOAJ

【 摘 要 】

We focused on Cognitive Reserve (CR) in patients with early Huntington Disease (HD) and investigated whether clinical outcomes might be influenced by lifetime intellectual enrichment over time. CR was evaluated by means of the Cognitive Reserve Index questionnaire (CRIq), an internationally validated scale which includes three sections: education, working activity, and leisure time. The clinical HD variables were quantified at three different time points (baseline-t0, 1 year follow up-t1 and 2 years follow up-t2) as per the Unified Huntington’s Disease Rating Scale (UHDRS), an internationally standardized and validated scale including motor, cognitive, functional and behavioral assays. Our sample consisted of 75 early manifest patients, withclinical stage scored according to the Total Functional Capacity (TFC) scale. Our correlational analysis highlighted a significant inverse association between CRIq leisure time (CRIq_LA) and longitudinal functional impairment (namely, the differential TFC score between t2 and t0 or ΔTFC) (p < 0.05), and the multidimensional progression of HD as measured by the composite UHDRS (cUHDRS, p < 0.01). CRIq_LA was significantly and positively associated with better cognitive performances at all time points (p < 0.05). Our results suggest that higher is the CRIq_LA, milder is the progression of HD in terms of functional, multidimensional and cognitive outcome.

【 授权许可】

Unknown