The effects of multidisciplinary rehabilitation in patients with early‐to‐middle‐stage Huntington's disease: a pilot study

Thompson, Jennifer A.; Cruickshank, Travis; Peñailillo, Luis; Lee, J.; Newton, Robert U.; Barker, Roger A.; Ziman, Mel

doi:10.1111/ene.12053

Cited by 75 publications

(107 citation statements)

References 9 publications

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“…Eighteen patients with manifest HD (11 men, 7 women) were recruited using the Huntington’s Enrichment Research Optimisation scheme database [12], with assistance from the Huntington’s Western Australia Association. Inclusion criteria were a positive genetic test, clinically verified disease expression (Unified Huntington’s Disease Rating Scale–Total Motor Score [UHDRS-TMS] ≥5), and the ability to understand and respond to instructions.…”

Section: Methodsmentioning

confidence: 99%

Pulmonary function in patients with Huntington’s Disease

et al. 2014

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BackgroundHuntington’s disease (HD) is a neurodegenerative disorder characterized by progressive motor, cognitive and psychiatric disturbances. Chest muscle rigidity, respiratory muscle weakness, difficulty in clearing airway secretions and swallowing abnormalities have been described in patients with neurodegenerative disorders including HD. However limited information is available regarding respiratory function in HD patients. The purpose of this study was to investigate pulmonary function of patients with HD in comparison to healthy volunteers, and its association with motor severity.MethodsPulmonary function measures were taken from 18 (11 male, 7 female) manifest HD patients (53 ± 10 years), and 18 (10 male, 8 female) healthy volunteers (52 ± 11 years) with similar anthropometric and life-style characteristics to the recruited HD patients. Motor severity was quantified by the Unified Huntington’s Disease Rating Scale-Total Motor Score (UHDRS-TMS). Maximum respiratory pressure was measured on 3 separate days with a week interval to assess test-retest reliability.ResultsThe test-retest reliability of maximum inspiratory and expiratory pressure measurements was acceptable for both HD patient and control groups (ICC ≥0.92), but the values over 3 days were more variable in the HD group (CV < 11.1%) than in the control group (CV < 7.6%). The HD group showed lower respiratory pressure, forced vital capacity, peak expiratory flow and maximum voluntary ventilation than the control group (p < 0.05). Forced vital capacity, maximum voluntary ventilation and maximum respiratory pressures were negatively (r = -0.57; -0.71) correlated with the UHDRS-TMS (p < 0.05).ConclusionPulmonary function is decreased in manifest HD patients, and the magnitude of the decrease is associated with motor severity.

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Section: Methodsmentioning

confidence: 99%

Pulmonary function in patients with Huntington’s Disease

et al. 2014

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“…Studies evaluating the utility of multidisciplinary therapy have documented significant changes in grey matter volume, as well as improvements in memory, processing speed, balance and gait, mood and quality of life in patients with manifest HD (144)(145)(146)(147). Recent data from our research programme has shown, in particular, that multidisciplinary therapy increases grey matter volume in the caudate tail and dorsolateral PFC in patients with manifest HD (147).…”

Section: Effects Of Multidisciplinary Therapy On Brain Volume and Potmentioning

confidence: 99%

Neuroendocrine and neurotrophic signaling in Huntington’s disease: Implications for pathogenic mechanisms and treatment strategies

Bartlett

Cruickshank

Hannan

et al. 2016

Neuroscience & Biobehavioral Reviews

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Huntington's disease (HD) is a fatal neurodegenerative disease caused by an extended polyglutamine tract in the huntingtin protein. Circadian, sleep and hypothalamic-pituitary-adrenal (HPA) axis disturbances are observed in HD as early as 15 years before clinical disease onset. Disturbances in these key processes result in increased cortisol and altered melatonin release which may negatively impact on brain-derived neurotrophic factor (BDNF) expression and contribute to documented neuropathological and clinical disease features. This review describes the normal interactions between neurotrophic factors, the HPA-axis and circadian rhythm, as indicated by levels of BDNF, cortisol and melatonin, and the alterations in these intricately balanced networks in HD. We also discuss the implications of these alterations on the neurobiology of HD and the potential to result in hypothalamic, circadian, and sleep pathologies. Measurable alterations in these pathways provide targets that, if treated early, may reduce degeneration of brain structures. We therefore focus here on the means by which multidisciplinary therapy could be utilised as a non-pharmaceutical approach to restore the balance of these pathways.

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“…To date, rehabilitation studies of early-to-moderate stage HD have focused on improving motor functions such as gait and balance [10–13]. An exploratory study on the effects of multi-disciplinary rehabilitation on brain structure and cognition showed increased gray matter volume that corresponded with significant improvements in verbal learning and memory [14].…”

Section: Introductionmentioning

confidence: 99%

Feasibility of computerized working memory training in individuals with Huntington disease

et al. 2017

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ObjectivesHuntington disease (HD) is associated with a variety of cognitive deficits, with prominent difficulties in working memory (WM). WM deficits are notably compromised in early-onset and prodromal HD patients. This study aimed to determine the feasibility of a computerized WM training program (Cogmed QM), novel to the HD population.MethodsNine patients, aged 26–62, with early stage HD underwent a 25-session (5 days/week for 5 weeks) WM training program (Cogmed QM). Training exercises involved the manipulation and storage of verbal and visuospatial information, with difficulty adapted as a function of individual performance. Neuropsychological testing was conducted before and after training, and performance on criterion WM measures (Digit Span and Spatial Span), near-transfer WM measures (Symbol Span and Auditory WM), and control measures were evaluated. Post-training interviews about patient experience were thematically analyzed using NVivo software.ResultsSeven of nine patients demonstrated adherence to the training and completed all sessions within the recommended timeframe of 5 weeks. All adherent patients showed improvement on the Cogmed tasks as defined by the Improvement Index (M = 22.17, SD = 8.84, range = 13–36). All adherent patients reported that they found training helpful (n = 7), and almost all felt that their memory improved (n = 6). Participants also expressed that the training was difficult, sometimes frustrating, and time consuming.ConclusionsThis pilot study provides support for feasibility of computerized WM training in early-stage patients with HD. Results suggest that HD patients perceive benefits of intensive WM training, though a full-scale and controlled intervention project is needed to understand the size of the effect and reliability of changes over time.Trial registrationClinicalTrials.gov, Registry number NCT02926820

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The effects of multidisciplinary rehabilitation in patients with early‐to‐middle‐stage Huntington's disease: a pilot study

Abstract: This pilot study suggests that a prolonged multidisciplinary rehabilitation programme in early-to-middle-stage HD is feasible, well-tolerated and associated with therapeutic benefit. Further explorative, larger studies are warranted.

Cited by 75 publications

References 9 publications

Pulmonary function in patients with Huntington’s Disease

Pulmonary function in patients with Huntington’s Disease

Neuroendocrine and neurotrophic signaling in Huntington’s disease: Implications for pathogenic mechanisms and treatment strategies

Feasibility of computerized working memory training in individuals with Huntington disease

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