Clinical Decision Trees to Guide Physical Therapy Management of Persons with Huntington's Disease.

BACKGROUND: In 2020, our group published physical therapy clinical practice guidelines (CPG) for people with Huntington's disease (HD). The guideline recommendations were categorized according to six primary movement impairment classifications. OBJECTIVE: To facilitate implementation of this CPG, we have developed guideline-based algorithms for physical therapy assessments and interventions and recommendations for therapists to overcome barriers to CPG implementation for people with HD. METHODS: We conducted a literature review of papers that evaluated physical therapy interventions in individuals with HD (n = 26) to identify assessments for each of the primary movement impairment classifications, and then searched for papers (n = 28) that reported their clinometric/psychometric properties in HD. Assessments were evaluated using modified Movement Disorder Society Committee on Rating Scales criteria and other relevant criteria. RESULTS: We identified a "core set" of physical therapy assessments for persons with HD, including the Six Minute Walk Test, Timed Up and Go Test, Berg Balance Scale, and the Medical Outcomes Study Short Form 36 (SF-36). We then developed guideline-based decision trees to assist in decision making and implementation of the CPG into practice for persons with HD across the continuum of care. Finally, we developed strategies for overcoming barriers to implementation, such as seeking specialized training in HD, engaging caretakers or family members to help the person with HD to exercise, and establishing clinical pathways that support early physical therapy referrals. CONCLUSION: Knowledge translation documents such as this are essential to promoting implementation of the physical therapy CPGs into clinical practice.

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

OBJECTIVE: To determine whether the psychological benefits of intense, inpatient, multimodal rehabilitation for persons with Huntingtons disease (HD), as found in earlier studies, also apply in a shorter, day-care setting. DESIGN: Prospective, non-randomized cohort study. SUBJECTS: Twenty patients attending a group-based 8-week (3 days/week) rehabilitation programme aimed at persons in early stages of HD. METHODS: An explorative cohort study on register data from a specialized rehabilitation centre, includ-ing descriptive data, number of cancellations, a self-reported evaluation, and measures of psychiatric symptoms, health-related quality of life, sense of coherence and physical function at baseline and at the end of rehabilitation. RESULTS: Patients' attendance rate was almost 90%. Patients were satisfied, and displayed significantly reduced anxiety and depression and improved health-related quality of life after rehabilitation. Baseline measures of sense of coherence showed significant negative correlation with the number of cancelled days of rehabilitation. Physical function improved, but did not correlate significantly with psychological outcome measures. CONCLUSION: These results indicate that an 8-week multimodal day-care rehabilitation programme can be tolerable, reduce psychiatric symptoms, and improve health-related quality of life for people with HD. A higher sense of coherence seems to promote attendance rates. Further larger studies, including the impact of cognition and disease progression on the treatment effect, are warranted.

Mejoría de la capacidad funcional en la enfermedad de Huntington tras un programa de marcha nórdica. A propósito de un caso / Improved functional capacity in Huntington disease after Nordic walking training: A case report

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Drumming Motor Sequence Training Induces Apparent Myelin Remodelling in Huntington's Disease: A Longitudinal Diffusion MRI and Quantitative Magnetization Transfer Study.

BACKGROUND: Impaired myelination may contribute to Huntington's disease (HD) pathogenesis. OBJECTIVE: This study assessed differences in white matter (WM) microstructure between HD patients and controls, and tested whether drumming training stimulates WM remodelling in HD. Furthermore, it examined whether training-induced microstructural changes are related to improvements in motor and cognitive function. METHODS: Participants undertook two months of drumming exercises. Working memory and executive function were assessed before and post-training. Changes in WM microstructure were investigated with diffusion tensor magnetic resonance imaging (DT-MRI)-based metrics, the restricted diffusion signal fraction (Fr) from the composite hindered and restricted model of diffusion (CHARMED) and the macromolecular proton fraction (MPF) from quantitative magnetization transfer (qMT) imaging. WM pathways linking putamen and supplementary motor areas (SMA-Putamen), and three segments of the corpus callosum (CCI, CCII, CCIII) were studied using deterministic tractography. Baseline MPF differences between patients and controls were assessed with tract-based spatial statistics. RESULTS: MPF was reduced in the mid-section of the CC in HD subjects at baseline, while a significantly greater change in MPF was detected in HD patients relative to controls in the CCII, CCIII, and the right SMA-putamen post-training. Further, although patients improved their drumming and executive function performance, such improvements did not correlate with microstructural changes. Increased MPF suggests training-induced myelin changes in HD. CONCLUSION: Though only preliminary and based on a small sample size, these results suggest that tailored behavioural stimulation may lead to neural benefits in early HD, that could be exploited for delaying disease progression.

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 Systematic Review of Rehabilitation for Corticobulbar Symptoms in Adults with Huntington's Disease.

BACKGROUND: Corticobulbar symptoms have been reported in all stages of Huntington's disease (HD); aspiration pneumonia associated with swallowing impairment has been identified as the most common cause of death. Whilst recent research has described positive effects of corticobulbar rehabilitation in other neurodegenerative conditions, it is unclear if this is similarly effective in HD. Preliminary evidence in corticospinal rehabilitation has revealed physical therapy and exercise could be beneficial for individuals with HD. OBJECTIVE: This systematic review will explore the literature relative to rehabilitation of corticobulbar symptoms in adults with HD. METHODS: Two investigators independently searched relevant electronic databases for literature related to corticobulbar rehabilitation in HD, published in English until October 2019. Included studies were critically appraised using the Oxford Centre for Evidence-based Medicine Levels of Evidence, Cochrane Risk of Bias Tool and Scottish Intercollegiate Guidelines Network checklists. Study outcomes included measurements of function, quality of life or neuromuscular physiology. RESULTS: Seventy-seven publications were screened with eight studies meeting the inclusion criteria - two randomised control trials and six intervention studies. Validated and objective outcome measures of corticobulbar symptoms were infrequently used. There was a high risk of bias identified in 7/8 studies. The data suggested positive clinical outcomes, no adverse effects and no deterioration observed across longitudinal studies. CONCLUSIONS: This systematic review documented a lack of high-quality evidence to support the use of rehabilitation to treat corticobulbar symptoms in HD. However, the suggestion of potential positive effects based on available, albeit limited, studies provides justification for further research in this area.

Physical Activity and Exercise Outcomes in Huntington Disease (PACE-HD): Protocol for a 12-Month Trial Within Cohort Evaluation of a Physical Activity Intervention in People With Huntington Disease.

BACKGROUND: Exercise is emerging as an important aspect in the management of disease-related symptoms and functional decline in people with Huntington disease (HD). Long-term evaluation of physical activity and exercise participation in HD has yet to be undertaken. OBJECTIVE: The objective is to investigate the feasibility of a nested randomized controlled trial (RCT) alongside a longitudinal observational study of physical activity and exercise outcomes in people with HD. DESIGN: This will be a 12-month longitudinal observational study (n = 120) with a nested evaluation of a physical activity intervention (n = 30) compared with usual activity (n = 30) using a "trial within a cohort" design. SETTING: The study will take place in HD specialist clinics in Germany, Spain, and the United States, with intervention delivery in community settings. PARTICIPANTS: The participants will have early-mid-stage HD and be participating in the Enroll-HD study. INTERVENTION: This will be a 12-month physical activity behavioral change intervention, delivered by physical therapists in 18 sessions, targeting uptake of aerobic exercise and increased physical activity. MEASUREMENTS: All participants (n = 120) will complete Enroll-HD assessments (motor, cognitive, behavioral, and quality of life) at baseline and at 12 months. Additional Physical ACtivity and Exercise Outcomes in Huntington Disease (PACE-HD) assessments include fitness (predicted maximal oxygen uptake [V o2max]), self-reported and quantitative measures of physical activity, disease-specific symptoms, and walking endurance. RCT participants (n = 60) will complete an additional battery of quantitative motor assessments and a 6-month interim assessment. Enroll-HD data will be linked to PACE-HD physical activity and fitness data. LIMITATIONS: The limitations include that the embedded RCT is open, and assessors at RCT sites are not blinded to participant allocation. CONCLUSION: PACE-HD will enable determination of the feasibility of long-term physical activity interventions in people with HD. The novel "trial within a cohort" design and incorporation of data linkage have potential to reduce participant burden. This design could be applied to other neurological diseases and movement disorders where recruitment and retention are challenging.

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

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.

Effects of multidisciplinary therapy on physical function in Huntington's disease.

OBJECTIVE: The primary objective of this trial was to evaluate the effects of outpatient multidisciplinary therapy, compared to usual care, on measures of physical function and muscle strength in patients with manifest Huntington's disease (HD). METHODS: Twenty-two patients with clinically verified HD were randomized to receive 36 weeks of outpatient multidisciplinary therapy or usual care. Outpatient multidisciplinary therapy comprised 9 months of supervised exercise, cognitive therapy and self-directed home-based exercise. Usual care consisted of standard medical care. Patients were assessed at 0 and 36 weeks by blinded assessors. The primary outcome was changed in mobility as measured by the 10-m Timed Walk Test. Secondary outcome measures included changes in manual dexterity (Timed Nut and Bolt Test), balance (Berg Balance Scale), cardiorespiratory endurance (6-Minute Walk Test) and upper and lower extremity muscle strength (isokinetic and isometric muscle strength and 10 Repetition Sit-to-Stand Tests). RESULTS: Patients receiving outpatient multidisciplinary therapy demonstrated significantly enhanced manual dexterity (P < 0.05) and lower extremity muscle strength (P < 0.05) than patients receiving usual care. No significant differences in mobility, balance, cardiorespiratory endurance and upper extremity strength outcomes were observed between groups after the intervention period. There were no adverse events associated with multidisciplinary therapy. CONCLUSION: Our findings suggest that outpatient multidisciplinary therapy has positive effects on manual dexterity and muscle strength, but no meaningful effects on mobility, balance, cardiorespiratory endurance and upper extremity muscle strength in patients with HD. Larger randomized controlled trials are needed to confirm these preliminary findings.

Restorative Strategies in Movement Disorders: the Contribution of Imaging.

PURPOSE OF REVIEW: The purpose of this review was to review the imaging, particularly positron emission tomography (PET), findings in neurorestoration studies in movement disorders, with specific focus on neural transplantation in Parkinson's disease (PD) and Huntington's disease (HD). RECENT FINDINGS: PET findings in PD transplantation studies have shown that graft survival as reflected by increases in dopaminergic PET markers does not necessarily correlate with clinical improvement. PD patients with more denervated ventral striatum and more imbalanced serotonin-to-dopamine ratio in the grafted neurons tended to have worse outcome. In HD transplantation studies, variable graft survival and clinical responses may be related to host inflammatory/immune responses to the grafts. Information gleaned from imaging findings in previous neural transplantation studies has been used to refine study protocol and patient selection in future trials. This includes identifying suitable candidates for transplantation using imaging markers, employing multiple and/or novel PET tracers to better assess graft functions and inflammatory responses to grafts.

Physical Therapy and Exercise Interventions in Huntington's Disease: A Mixed Methods Systematic Review.

BACKGROUND: A number of studies evaluating physical therapy and exercise interventions in Huntington's disease have been conducted over the past 15 years. However, an assessment of the quality and strength of the evidence in support of these interventions is lacking. OBJECTIVE: The purpose of this systematic review was to investigate the effectiveness of physical therapy and exercise interventions in people with Huntington's disease, and to examine the perceptions of patients, families and caregivers of these interventions. METHODS: This mixed-methods systematic review utilized the Joanna Briggs Institute (JBI) approach and extraction tools to evaluate the literature from January 2003 until May 2016. The review considered interventions that included exercise and physical therapy interventions, and included both quantitative and qualitative outcome measures. RESULTS: Twenty (20) studies met the inclusion criteria, including eighteen (18) that had quantitative outcome measures and two (2) that utilized qualitative methods. JBI Levels of evidence for the 18 quantitative studies were as follows: Eight studies were at evidence Level 1, seven were at Level 2, two were at Level 3, and one was at Level 4. CONCLUSIONS: Our review suggests that there is preliminary support for the benefits of exercise and physical activity in Huntington's disease in terms of motor function, gait speed, and balance, as well as a range of physical and social benefits identified through patient-reported outcomes. Variability in mode of intervention as well as outcome measures limits the interpretability of these studies, and high-quality studies that incorporate adaptive trial designs for this rare disease are needed.

The role of rehabilitation therapy in Huntington disease.

The role of rehabilitation interventions is increasingly considered a key component to effective management of people with Huntington disease (HD). Lifestyle factors, such as activity level and exercise, as well as specific motor training may be helpful in managing the functional sequelae of HD and possibly slowing disease progression. In this chapter, we focus on the role of rehabilitation therapy in secondary and tertiary prevention of the potentially devastating consequences of HD. We provide a brief overview of the range of motor and cognitive impairments in HD and their effect on functional abilities. We further discuss emerging evidence in terms of the role of exercise, physical activity, and physical therapies in helping to minimize functional loss and maximize quality of life throughout the disease process. Future directions with respect to intensive and goal-directed exercise, including aerobic and strengthening programs, are also discussed. This is an area of particular importance alongside exploring the potential that motor-training paradigms have in mediating the effects of disease-modifying drugs, cell replacement therapy, or genetic manipulations, when available.

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.

Physical therapy and exercise interventions in Huntington's disease: a mixed methods systematic review protocol.

REVIEW QUESTION/OBJECTIVE: The review seeks to evaluate the effectiveness of physical therapy and exercise interventions in Huntington's disease (HD). The review question is: What is the effectiveness of physiotherapy and therapeutic exercise interventions in people with HD, and what are patients', families' and caregivers' perceptions of these interventions?The specific objectives are:This mixed methods review seeks to develop an aggregated synthesis of quantitative, qualitative and narrative systematic reviews on physiotherapy and exercise interventions in HD, in an attempt to derive conclusions and recommendations useful for clinical practice and policy decision-making.

A Classification System to Guide Physical Therapy Management in Huntington Disease: A Case Series.

BACKGROUND AND PURPOSE: Individuals with Huntington disease (HD), a rare neurological disease, experience impairments in mobility and cognition throughout their disease course. The Medical Research Council framework provides a schema that can be applied to the development and evaluation of complex interventions, such as those provided by physical therapists. Treatment-based classifications, based on expert consensus and available literature, are helpful in guiding physical therapy management across the stages of HD. Such classifications also contribute to the development and further evaluation of well-defined complex interventions in this highly variable and complex neurodegenerative disease. The purpose of this case series was to illustrate the use of these classifications in the management of 2 individuals with late-stage HD. CASE DESCRIPTION: Two females, 40 and 55 years of age, with late-stage HD participated in this case series. Both experienced progressive declines in ambulatory function and balance as well as falls or fear of falling. Both individuals received daily care in the home for activities of daily living. INTERVENTION: Physical therapy Treatment-Based Classifications for HD guided the interventions and outcomes. Eight weeks of in-home balance training, strength training, task-specific practice of functional activities including transfers and walking tasks, and family/carer education were provided. OUTCOMES: Both individuals demonstrated improvements that met or exceeded the established minimal detectible change values for gait speed and Timed Up and Go performance. Both also demonstrated improvements on Berg Balance Scale and Physical Performance Test performance, with 1 of the 2 individuals exceeding the established minimal detectible changes for both tests. Reductions in fall risk were evident in both cases. DISCUSSION: These cases provide proof-of-principle to support use of treatment-based classifications for physical therapy management in individuals with HD. Traditional classification of early-, mid-, and late-stage disease progression may not reflect patients' true capabilities; those with late-stage HD may be as responsive to interventions as those at an earlier disease stage.Video Abstract available for additional insights from the authors (see Supplemental Digital Content 1, available at: http://links.lww.com/JNPT/A172).

Feasibility and Efficacy of Brief Computerized Training to Improve Emotion Recognition in Premanifest and Early-Symptomatic Huntington's Disease.

OBJECTIVES: Deficits in the recognition of negative emotions emerge before clinical diagnosis in Huntington's disease (HD). To address emotion recognition deficits, which have been shown in schizophrenia to be improved by computerized training, we conducted a study of the feasibility and efficacy of computerized training of emotion recognition in HD. METHODS: We randomly assigned 22 individuals with premanifest or early symptomatic HD to the training or control group. The training group used a self-guided online training program, MicroExpression Training Tool (METT), twice weekly for 4 weeks. All participants completed measures of emotion recognition at baseline and post-training time-points. Participants in the training group also completed training adherence measures. RESULTS: Participants in the training group completed seven of the eight sessions on average. Results showed a significant group by time interaction, indicating that METT training was associated with improved accuracy in emotion recognition. CONCLUSIONS: Although sample size was small, our study demonstrates that emotion recognition remediation using the METT is feasible in terms of training adherence. The evidence also suggests METT may be effective in premanifest or early-symptomatic HD, opening up a potential new avenue for intervention. Further study with a larger sample size is needed to replicate these findings, and to characterize the durability and generalizability of these improvements, and their impact on functional outcomes in HD. (JINS, 2017, 23, 314-321).

Characterisation of aggression in Huntington's disease: rates, types and antecedents in an inpatient rehabilitation setting.

AIMS AND OBJECTIVES: To systematically review aggression in an inpatient Huntington's cohort examining rates, types and antecedents. BACKGROUND: Although the prevalence of aggression in Huntington's disease is high, research into this problematic behaviour has been limited. Few studies have investigated the nature of aggressive behaviour in Huntington's disease or antecedents that contribute to its occurrence. DESIGN: A systematic, double-coded, electronic medical file audit. METHODS: The electronic hospital medical records of 10 people with Huntington's disease admitted to a brain disorders unit were audited for a 90-day period using the Overt Aggression Scale-Modified for Neurorehabilitation framework, yielding 900 days of clinical data. RESULTS: Nine of 10 clients exhibited aggression during the audit period. Both verbal (37·1%) aggression and physical aggression were common (33·8%), along with episodes of mixed verbal and physical aggression (15·2%), while aggression to objects/furniture was less prevalent (5·5%). The most common antecedent was physical guidance with personal care, far exceeding any other documented antecedents, and acting as the most common trigger for four of the nine clients who exhibited aggression. For the remaining five clients, there was intraindividual heterogeneity in susceptibility to specific antecedents. CONCLUSION: In Huntington's sufferers at mid- to late stages following disease onset, particular care should be made with personal care assistance due to the propensity for these procedures to elicit an episode of aggression. However, given the degree of intraindividual heterogeneity in susceptibility to specific antecedents observed in the present study, individualised behaviour support plans and sensory modulation interventions may be the most useful in identifying triggers and managing aggressive episodes. RELEVANCE TO CLINICAL PRACTICE: Rates of aggression in Huntington's disease inpatients can be high. Knowledge of potential triggers, such as personal care, is important for nursing and care staff, so that attempts can be made to minimise distress for patients and maximise the personal safety of care staff.

A randomized, controlled trial of a multi-modal exercise intervention in Huntington's disease.

INTRODUCTION: This study aimed to evaluate the feasibility and benefit of a structured exercise intervention in people with Huntington's Disease (HD). METHODS: This study was conducted at 6 sites, and participants were randomized into either exercise or control (usual care) groups, and were assessed at baseline, 13 and 26 weeks. The intervention was a 12 week, three times per week progressive exercise program, including aerobic (stationary cycling) and upper and lower body strengthening exercise with tapered 1:1 support for 20 of 36 sessions. RESULTS: 314 adults were assessed for eligibility: 248 did not meet inclusion criteria, 34 declined, and 32 were recruited and randomized. Three individuals in the intervention group were withdrawn within the first month due to concomitant medical conditions, resulting in 14 participants in intervention and 15 in control groups. There were two AEs in the intervention group, both related to previous medical conditions, and there were two SAEs, both in the control group. The intervention group had better fitness (predicted VO2 max difference: 492.3 ml min-1, 95% CI: [97.1, 887.6]), lower UHDRS mMS (difference 2.9 points, 95% [-5.42, -0.32]) and lower weight at Week 13 (difference 2.25 kg, 95% CI: [-4.47, -0.03]). CONCLUSION: This study demonstrates that a short-term exercise intervention is safe and feasible. Individuals with HD may benefit from structured exercise, and intensity, monitoring and support may be key factors in optimizing response. Larger scale trials are now required to fully elucidate the extended clinical potential of exercise in HD. TRIAL REGISTRATION: Current Controlled Trials ISRCTN11392629.

The effects of physiotherapy with PNF concept on gait and balance of patients with Huntington's disease - pilot study.

BACKGROUND AND PURPOSE: Huntington's disease (HD) is a neurodegenerative, progressive disorder of the central nervous system which causes significant gait and balance disturbances. This is a pilot study which aims to determine the effects of a physiotherapy programme with use of Proprioceptive Neuromuscular Facilitation (PNF) on gait and balance in HD patients. MATERIAL AND METHODS: 30 HD patients aged 21-60 with genetically confirmed diagnosis participated in the study. Participants followed a 3-week-long PNF-based physiotherapy programme. Gait and balance were evaluated twice in each participant: first at baseline and then after the course of physiotherapy. The following methods were used for gait disturbances: Tinetti Gait Assessment Tool, Up and Go Test, Timed Walking Tests for 10m and 20m (TWT10m, TWT20m). Balance was assessed with use of Berg Balance Scale, Pastor Test and Functional Reach Test. RESULTS: There was a significant improvement in all measures of balance and gait. CONCLUSION: PNF-based physiotherapy is effective and safe in HD patients.