Clinical recommendations to guide physical therapy practice for Huntington disease.

OBJECTIVE: In the past decade, an increasing number of studies have examined the efficacy of physical therapy interventions in people with Huntington disease (HD). METHODS: We performed a mixed-methods systematic review using Joanna Briggs Institute (JBI) methodology and included experimental and observational study designs. The search resulted in 23 quantitative studies and 3 qualitative studies from which we extracted data using JBI standardized extraction tools. Results of this review suggested that physical therapy interventions may improve motor impairments and activity limitations in people with HD. Here, we expand on the review findings to provide specific recommendations to guide clinical practice. RESULTS: We recommend the following specific physical therapy interventions for people with HD: aerobic exercise (grade A evidence), alone or in combination with resistance training to improve fitness and motor function, and supervised gait training (grade A evidence) to improve spatiotemporal features of gait. In addition, there is weak (grade B) evidence that exercise training improves balance but does not show a reduction in the frequency of falls; inspiratory and expiratory training improves breathing function and capacity; and training of transfers, getting up from the floor, and providing strategies to caregivers for involvement in physical activity in the midstages of HD may improve performance. There is expert consensus for the use of positioning devices, seating adaptations, and caregiver training in late stages of HD. CONCLUSIONS: There is strong evidence to support physical therapy interventions to improve fitness, motor function, and gait in persons with HD.

Multidisciplinary rehabilitation reduces hypothalamic grey matter volume loss in individuals with preclinical Huntington's disease: A nine-month pilot study.

BACKGROUND: Hypothalamic pathology is a well-documented feature of Huntington's disease (HD) and is believed to contribute to circadian rhythm and habitual sleep disturbances. Currently, no therapies exist to combat hypothalamic changes, nor circadian rhythm and habitual sleep disturbances in HD. OBJECTIVE: To evaluate the effects of multidisciplinary rehabilitation on hypothalamic volume, brain-derived neurotrophic factor (BDNF), circadian rhythm and habitual sleep in individuals with preclinical HD. METHODS: Eighteen individuals with HD (ten premanifest and eight prodromal) undertook a nine-month multidisciplinary rehabilitation intervention (intervention group), which included exercise, cognitive and dual task training and social events, and were compared to a community sample of eleven individuals with premanifest HD receiving no intervention (control group). Hypothalamic volume, serum BDNF, salivary cortisol and melatonin concentrations, subjective sleep quality, daytime somnolence, habitual sleep-wake patterns, stress and anxiety and depression symptomatology were evaluated. RESULTS: Hypothalamus grey matter volume loss was significantly attenuated in the intervention group compared to the control group after controlling for age, gender, Unified Huntington's Disease Rating Scale-Total Motor Score and number of cytosine-adenine-guanine repeats. Serum BDNF levels were maintained in the intervention group, but decreased in the control group following the study period. Both groups exhibited decreases in cortisol and melatonin concentrations. No changes were observed in sleep or mood outcomes. CONCLUSIONS: This exploratory study provides evidence that multidisciplinary rehabilitation can reduce hypothalamic volume loss and maintain peripheral BDNF levels in individuals with preclinical HD but may not impact on circadian rhythm. Larger, randomised controlled trials are required to confirm these findings.

A qualitative examination of physiotherapist led community-based yoga for individuals with Huntington's disease.

PURPOSE: The purpose of this study is to examine community-based yoga, led by a physiotherapist, for individuals affected by Huntington's disease (HD). METHODS: Qualitative case study methodology was used to examine the Hatha yoga led by a certified yoga instructor who was a neurologic physiotherapist. Data collection included participant observations, semi-structured interviews with the instructor, and structured participant surveys. Data were coded and thematically analyzed. Strategies for rigor included field engagement, triangulation, member checks, and reflexivity. OUTCOMES: Five major themes emerged regarding the value and role of yoga for individuals affected by HD: [1] Emphasis on mindfulness, [2] Yoga is modifiable and accessible, [3] Precise communication, [4] Yoga fosters a sense of community, and [5] Poses tailored to HD-specific deficits. CONCLUSIONS: Yoga led by a physiotherapist can be tailored to enable participation by those affected by HD, addresses HD-specific deficits, and promotes a sense of community to supplement traditional physiotherapy.

Respiratory muscle training on pulmonary and swallowing function in patients with Huntington's disease: a pilot randomised controlled trial.

OBJECTIVE: To examine the effects of 4-month of respiratory muscle training on pulmonary and swallowing function, exercise capacity and dyspnoea in manifest patients with Huntington's disease. DESIGN: A pilot randomised controlled trial. SETTING: Home based training program. PARTICIPANTS: Eighteen manifest Huntington's disease patients with a positive genetic test and clinically verified disease expression, were randomly assigned to control group (n=9) and training group (n=9). INTERVENTION: Both groups received home-based inspiratory (5 sets of 5 repetitions) and expiratory (5 sets of 5 repetitions) muscle training 6 times a week for 4 months. The control group used a fixed resistance of 9 centimeters of water, and the training group used a progressively increased resistance from 30% to 75% of each patient's maximum respiratory pressure. MAIN MEASURES: Spirometric indices, maximum inspiratory pressure, maximum expiratory pressure, six minutes walk test, dyspnoea, water-swallowing test and swallow quality of life questionnaire were assessed before, at 2 and 4 months after training. RESULTS: The magnitude of increases in maximum inspiratory (d=2.9) and expiratory pressures (d=1.5), forced vital capacity (d=0.8), forced expiratory volume in 1 second (d=0.9) and peak expiratory flow (d=0.8) was substantially greater for the training group in comparison to the control group. Changes in swallowing function, dyspnoea and exercise capacity were small (d ≤ 0.5) for both groups without substantial differences between groups. CONCLUSIONS: A home-based respiratory muscle training program appeared to be beneficial to improve pulmonary function in manifest Huntington's disease patients but provided small effects on swallowing function, dyspnoea and exercise capacity.

Aquatherapy for neurodegenerative disorders.

Aquatherapy is used for rehabilitation and exercise; water provides a challenging, yet safe exercise environment for many special populations. We have reviewed the use of aquatherapy programs in four neurodegenerative disorders: Parkinson's disease, multiple sclerosis, amyotrophic lateral sclerosis, and Huntington's disease. Results support the use of aquatherapy in Parkinson's disease and multiple sclerosis, however further evidence is required to make specific recommendations in all of the aforementioned disorders.

Terapias no farmacológicas en la enfermedad de Huntington y en el daño cerebral relacionado con el alcohol / No disponible

Las terapias no farmacológicas son protagonistas de los programas de rehabilitación psicosocial del Hospital Mare de Déu de la Mercè. En este artículo se revisa el papel que la terapia física y la equinoterapia desempeñan en la rehabilitación de los pacientes con enfermedad de Huntington y en el daño cerebral relacionado con alcohol, describiéndose los objetivos de trabajo y la metodología empleada (AU)

Non pharmacological therapies are a main component of the psychosocial rehabilitation program of Mare de Déu de la Mercè Hospital. In this paper the use of physical therapy and equine therapy in patients suffering from Huntington disease and brain injury related to alcohol abuse are reviewed, describing their aims and applied methodology (AU)

Surface electromyograph activity of submental muscles during swallowing and expiratory muscle training tasks in Huntington's disease patients.

INTRODUCTION: Huntington's disease (HD) patients have difficulty in swallowing, leading to aspiration pneumonia, which is a major cause of death. It seems possible that submental muscles that are crucial for preventing an escape of a bolus into the airway, are affected by HD, but no previous studies have investigated this. OBJECTIVE: To assess surface electromyograph (sEMG) activity of submental muscles during swallowing and expiratory muscle training (EMT) tasks in HD patients in comparison to healthy volunteers. METHODS: sEMG activities of submental muscles during saliva, water swallowing, EMT tasks performed at 25% and 75% of maximum expiratory pressure were recorded and normalised by the sEMG activity during an effortful swallow in 17 early to mid stage HD patients and 17 healthy volunteers. RESULTS: sEMG activity was greater (p<0.05) during EMT tasks than saliva and water swallowing, but was not significantly different between groups for saliva, water swallowing and EMT at 25%. HD patients had lower sEMG activity for EMT at 75% (p<0.05). CONCLUSION: Decreases in submental muscle activity were not evident in HD patients except during EMT at 75%. This suggests that relative submental muscle weakness is observed only during a high intensity task in early to mid stage HD patients.

Video game play (Dance Dance Revolution) as a potential exercise therapy in Huntington's disease: a controlled clinical trial.

OBJECTIVE: To investigate the feasibility, acceptability, and safety of a supervised video game exercise program administered via Dance Dance Revolution in individuals with Huntington's disease. DESIGN: A cross-over, controlled, single-blinded, six-week trial. SETTING: Home-based. PARTICIPANTS: Eighteen ambulatory individuals with Huntington's disease (seven male, mean age 50.7 SD 14.7). INTERVENTIONS: Participants played the Dance Dance Revolution game with supervision and the handheld game without supervision for 45 minutes, two days per week for six weeks. OUTCOME MEASURES: Game play performance and adherence, participant perceptions of the game, safety (vital signs, adverse health changes), spatiotemporal gait measures, Four-Square Step Test, Tinetti Mobility Test, Activities-Specific Balance Confidence Scale, and World Health Organization Quality of Life - Bref, before and after each intervention. RESULTS: Most participants improved on game play, enjoyed playing the game, and wanted to continue playing after study completion. After playing Dance Dance Revolution, participants showed significant reductions in double support percentage (adjusted mean difference (95% confidence intervals): -2.54% (-4.75, -0.34) for forward walking and -4.18 (-6.89, -0.48) for backward walking) and those with less severe motor symptoms had reductions in heel-to-heel base of support during forward walking. The remaining measures were not significantly impacted by the intervention. CONCLUSION: Dance Dance Revolution appears to be a feasible, motivating, and safe exercise intervention for individuals with Huntington's disease.

Effects of an intensive rehabilitation programme on patients with Huntington's disease: a pilot study.

OBJECTIVE: To investigate the effects of an intensive, inpatient rehabilitation programme on individuals affected by Huntington's disease. DESIGN: A pilot study. Within-subjects design. SETTING: Inpatient rehabilitation home of the Italian welfare system. SUBJECTS: Forty patients, early and middle stage of the disease, were recruited to an intensive, inpatient rehabilitation protocol. INTERVENTIONS: The treatment programme included respiratory exercises and speech therapy, physical and occupational therapy and cognitive rehabilitation exercises. The programme involved three-week admission periods of intensive treatment that could be repeated three times a year. MAIN MEASURES: A standard clinical assessment was performed at the beginning of each admission using the Zung Depression Scale, Mini-Mental State Examination (MMSE), Barthel Index, Tinetti Scale and Physical Performance Test (PPT). Tinetti and PPT were also used at the end of each admission to assess the outcomes in terms of motor and functional performance. RESULTS: Each three-week period of treatment resulted in highly significant (P < 0.001) improvements of motor performance and daily life activities. The average increase was 4.7 for Tinetti and 5.21 for PPT scores. No carry-over effect from one admission to the next was apparent but at the same time, no motor decline was detected over two years, indicating that patients maintained a constant level of functional, cognitive as well as motor performance. CONCLUSIONS: Intensive rehabilitation treatments may positively influence the maintenance of functional and motor performance in patients with Huntington's disease.

Effects of multisensory stimulation in people with Huntington's disease: a randomized controlled pilot study.

OBJECTIVE: To investigate whether behavioural, motor and physiological responses of individuals with Huntington's disease (HD) to a controlled multisensory environment (MSE) are effective as a therapeutic (sustained effects) or leisure (immediate effects) activity. DESIGN: Pilot study--a randomized, controlled, two-group design. SETTING: Specialist residential unit for people with mid-late stage HD. SUBJECTS: Twelve patients with HD (one subject from each group dropped out during the study after week 8 due to medical complications). INTERVENTIONS: Patients attended eight, 30-minute sessions over a four-week period, of multisensory stimulation (MSE, treatment group) or relaxation activities (control group). MAIN OUTCOME MEASURES: Between-group comparisons for changes between assessment sessions for two behavioural assessments: Rehabilitation Evaluation--Hall and Baker (REHAB), Behaviour and Mood Disturbance Scale (BMD); a motor assessment: the dyskinesia section of the St Hans Rating Scale (SHRS); physiological measures: blood pressure, heart rate and respiratory rate. Secondary measures during intervention sessions included behavioural assessment using the Interact. RESULTS: There were no significant differences found between the groups for any main outcome measures made between sessions. The MSE group showed some positive effects within-sessions, with the Interact showing significant between-group differences in immediate effects on mood (p = 0.028). There was also a significantly different change over time for within-session changes in stimulation levels (p = 0.0002) and mood (p = 0.0001) between the groups. No physiological effects were observed in relation to sessions in either group. Two MSE subjects underwent changes in medication during the study period. CONCLUSIONS: There was no therapeutic effect of MSEs over the four-week study period. MSEs appear to be more effective thanconventional relaxation techniques as a leisure activity.