Cognitive Performance After a One-Year Multidisciplinary Intensive Rehabilitation Program for Huntington’s Disease: An Observational Study

Abstract

Background:Studies of physical therapy and multidisciplinary rehabilitation programs for Huntington’s disease (HD) have shown improvements in gait function, balance, and physical quality of life. There is a gap in the literature on effects of cognitive interventions and the potential to improve cognitive performance.Objective:To assess changes in cognitive performance among patients with early to middle stage HD as secondary analyses from a one-year multidisciplinary rehabilitation program. The program included cognitive stimulation as a non-specific cognitive intervention in addition to physical interventions.Methods:A one-year rehabilitation program that included comprehensive neuropsychological assessments was completed by 31 out 37 participants with early to middle stages of HD. Socio-demographic and clinical information was recorded. A battery of neuropsychological tests was used to measure cognitive functions before and after the intervention. Descriptive statistics was used for sample characteristics. Paired sample t-tests and nonparametric Wilcoxon Signed ranked tests were used to compare cognitive measures at both time points.Results:Scores on the Symbol Digit Modalities Test (SDMT) were significantly lower post intervention. There were no significant differences in all other measures. Scores on the Stroop color naming and California Verbal Learning Test-II (CVLT-II) long-term delayed recall tasks showed tendencies towards lower scores post intervention.Conclusions:An intensive multidisciplinary rehabilitation program for patients with HD was generally well tolerated and feasible, with no indication of negative effects on cognition. Neuropsychological measures overall remained stable following an intensive multidisciplinary rehabilitation program, however continued progression of cognitive impairment was evident on the SDMT, suggesting that disease progression is not halted. Randomized controlled trials are needed to verify these findings.

Therapeutic Use of Music, Dance, and Rhythmic Auditory Cueing for Patients with Huntington’s Disease: A Systematic Review

“…Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder characterized by striatal and basal ganglion atrophy [1][2][3][4]. Cogni-tive, psychiatric, and motor dysfunctions progress through early, middle, and advanced phases that correspond to five HD stages on the Shoulson and Fahn Total Functional Capacity (TFC) scale [5][6][7]. Striatal neuropathology is associated with cognitive and psychiatric functions [8][9][10] that often precede motor deficits [8][9][10][11][12].…”

“…Striatal neuropathology is associated with cognitive and psychiatric functions [8][9][10] that often precede motor deficits [8][9][10][11][12]. Cognitive impairments are observed in executive function, memory, affective processing, and psychomotor speed [7,[13][14][15]. Psychiatric symptoms, including irritability, depression, anxiety, apathy, disinhibition, and emotion dysregulation, are common in HD [16][17][18][19].…”

“…Factors of disease presentation, such as age at onset, rate of decline, and severity of symptoms vary among the HD cohort [7]. However, since there is currently no cure or disease-modifying therapy [23], there is a consistent need for long-term, multidisciplinary care through pharmacological [24,25] and non-pharmacological [26,27] interventions.…”

“…However, since there is currently no cure or disease-modifying therapy [23], there is a consistent need for long-term, multidisciplinary care through pharmacological [24,25] and non-pharmacological [26,27] interventions. These methods may reduce or stabilize disease progression in other progressive neurodegenerative disorders, such as Parkinson's disease (PD) [7]. Recent studies have investigated the use of physical therapy for HD patients [28][29][30][31][32].…”

Tolerability and psychological effects of a multimodal day-care rehabilitation program for persons with Huntington's disease

“…The results are promising in terms of motor functions and physical quality of life ( 18 – 20 ). An early pilot-study shows some cognitive benefits ( 18 ), which have not been replicated in later studies ( 19 – 21 ). The rehabilitation programmes also seem to have a positive effect on psychiatric symptoms, with reduced depression and anxiety ( 19 , 20 ).…”