Hanan Khalil

What effect does a structured home-based exercise programme have on people with Huntington’s disease? A randomized, controlled pilot study

Objective: The aim of this study was to explore feasibility, safety and outcome of an exercise intervention in people with Huntington’s disease. Design: A randomized controlled pilot trial. Setting: A home-based exercise programme. Subjects: A total of 25 subjects with early to mid-stage Huntington’s disease. Intervention: Subjects were randomly allocated to either an exercise intervention (n = 13) or a control group (n = 12). Subjects in the exercise intervention group were asked to perform exercises at home three times a week for eight weeks using an exercise DVD, specifically developed for this purpose. The control group received their usual care. Measures: Adherence in the intervention group was calculated from exercise diaries. Measures of gait, balance, function, level of physical activity and quality of life were evaluated. Analysis of covariance was used to compare follow-up scores across groups after adjustment for chance baseline differences. Effect sizes were calculated. Results: Eleven participants from the intervention and ten from the control group completed the study. Mean adherence was 29.4 SD 1.8 for the 32 prescribed sessions. There were no related adverse events. Differences between groups were observed in gait speed, balance, function and level of physical activity, but not quality of life as measured by the SF36. Effect sizes were large (>0.8) for the majority of the outcomes. Conclusions: Short-term structured home exercise programmes are feasible, beneficial and safe for people with early to mid-stage of Huntington’s disease. Our findings support the implementation of a larger trial of longer-term home exercise.

Analysis of gait and balance through a single triaxial accelerometer in presymptomatic and symptomatic Huntington's disease

PURPOSE To investigate the capacity of a single triaxial accelerometer sensor in detecting gait and balance impairments in pre-manifest and manifest Huntingtons disease (HD) subjects. METHODS Fourteen manifest HD (MHD) (age: 51.83±14.8), ten pre-manifest HD (PHD) (age: 44.8±11.7) and ten healthy subjects (HLY) (age: 56.4±10.9) were recruited. The sensor was attached to the upper sternum as subjects completed gait and Romberg balance tests. An inverted pendulum model of the bodys centre of mass and an unbiased autocorrelation procedure were employed to derive gait parameters from the triaxial accelerometer signal. The accuracy of the gait measurements was compared to those recorded by a computerized walkway. RESULTS Strong agreement was seen between the sensor and the walkway; cadence (ICC=0.95, CI=[0.75, 0.97]), velocity (ICC=0.94, CI=[0.75, 0.97]) and step length (ICC=0.89, CI=[0.77, 0.95]). Sensor derived velocity was significantly higher in HLY (p<0.001) and PHD (p<0.005) when compared to MHD. Step and stride length was significantly longer in HLY (p<0.05) and PHD (p<0.001) when compared to MHD. Significant differences between subject groups across all four balance tasks (p<0.001) were found. CONCLUSION An accelerometer based sensor may be an effective means of differentiating between pre-manifest and manifest Huntingtons disease subjects.

Physical Therapy Intervention for People With Huntington Disease

Background and Purpose: The clinical symptoms of Huntington disease (HD) include progressive movement disorders, cognitive deficits, and behavioral changes, all of which affect an individuals ability to participate in activities of daily living. To date, very few quantitative or qualitative studies have been conducted to guide physical therapists working with people with HD. The objective of this study was to characterize current physical therapist practice for people with HD, thus informing the development of standardized clinical care and future research studies. Subjects and Methods: Consultation with physical therapists working with people with HD was undertaken in the form of mailed questionnaires (n=49) and semistructured interviews (n=8). The development of the interview schedule was aided by consideration of the data obtained from the questionnaires. Themes identified from the interviews were considered in light of published literature and questionnaire responses. Results: The main issues that emerged from the interviews were classified into 3 subthemes: (1) there is insufficient use of routine physical therapy–related outcome measures at different stages of HD, (2) there is underutilization of physical therapy services in managing HD (particularly in the early stages), and (3) the management of falls and mobility deficit progression is a key treatment aim for people with HD. Discussion and Conclusion: A conceptual framework for physical therapy intervention in HD was developed on the basis of the themes that emerged from the data in this study. Such a framework has utility for complex, progressive conditions such as HD and may facilitate clinical decision making and standardization of practice and affect the development of future physical therapy trials.

Adherence to Use of a Home-Based Exercise DVD in People With Huntington Disease: Participants' Perspectives

Background Individualized exercise instruction on a regular basis may be desirable for people with Huntington disease (HD). Sustained interventions, however, may not be the most feasible for lifelong disease management. It is critical, therefore, for physical therapists to find ways to facilitate engagement in independent exercise programs. The capabilities of DVD technology can make the DVD a useful format to facilitate engagement for people with HD who have motivational and cognitive problems. Objective The purpose of this study was to explore how people with HD used a home-based exercise DVD and how it was perceived by the participants and their caregivers. Design An exploratory, mixed-method design was used in this study. Methods Fifteen participants with HD were provided with an exercise DVD, developed by the authors, to support their engagement in a home-based exercise program. Exercise diaries were used to record adherence rates. Semistructured interviews were conducted to explore what factors affected the usability of the DVD. Conversations were audio recorded and fully transcribed. Content analysis approach was used to analyze the interviews. Results Most of the participants (11/15, 73.3%) adhered well to the use of the DVD. Participants felt that the exercises were suitable. Commitment of the caregiver as a key to the success of the program was the main theme that emerged from the data. Participants identified barriers and facilitators that affected adherence to using the exercise DVD and described management strategies that helped promote adherence to the exercise program. Conclusions The DVD was perceived to be suitable and supportive. The DVD could be appropriate for use in supporting people with HD to engage in exercise at home, either outside of therapy sessions or upon completion of a therapy program.

Reliability and Minimal Detectable Change of Physical Performance Measures in Individuals With Pre-manifest and Manifest Huntington Disease

Background Clinical intervention trials in people with Huntington disease (HD) have been limited by a lack of reliable and appropriate outcome measures. Objective The purpose of this study was to determine the reliability and minimal detectable change (MDC) of various outcome measures that are potentially suitable for evaluating physical functioning in individuals with HD. Design This was a multicenter, prospective, observational study. Methods Participants with pre-manifest and manifest HD (early, middle, and late stages) were recruited from 8 international sites to complete a battery of physical performance and functional measures at 2 assessments, separated by 1 week. Test-retest reliability (using intraclass correlation coefficients) and MDC values were calculated for all measures. Results Seventy-five individuals with HD (mean age=52.12 years, SD=11.82) participated in the study. Test-retest reliability was very high (>.90) for participants with manifest HD for the Six-Minute Walk Test (6MWT), 10-Meter Walk Test, Timed “Up & Go” Test (TUG), Berg Balance Scale (BBS), Physical Performance Test (PPT), Barthel Index, Rivermead Mobility Index, and Tinetti Mobility Test (TMT). Many MDC values suggested a relatively high degree of inherent variability, particularly in the middle stage of HD. Minimum detectable change values for participants with manifest HD that were relatively low across disease stages were found for the BBS (5), PPT (5), and TUG (2.98). For individuals with pre-manifest HD (n=11), the 6MWT and Four Square Step Test had high reliability and low MDC values. Limitations The sample size for the pre-manifest HD group was small. Conclusions The BBS, PPT, and TUG appear most appropriate for clinical trials aimed at improving physical functioning in people with manifest HD. Further research in people with pre-manifest HD is necessary.

Insights into gait disorders: Walking variability using phase plot analysis, Huntington's disease

Huntingtons disease (HD) is a progressive inherited neurodegenerative disorder. Identifying sensitive methodologies to quantitatively measure early motor changes have been difficult to develop. This exploratory observational study investigated gait variability and symmetry in HD using phase plot analysis. We measured the walking of 22 controls and 35 HD gene carriers (7 premanifest (PreHD)), 16 early/mid (HD1) and 12 late stage (HD2) in Oxford and Cardiff, UK. The unified Huntingtons disease rating scale-total motor scores (UHDRS-TMS) and disease burden scores (DBS) were used to quantify disease severity. Data was collected during a clinical walk test (8.8 or 10 m) using an inertial measurement unit attached to the trunk. The 6 middle strides were used to calculate gait variability determined by spatiotemporal parameters (co-efficient of variation (CoV)) and phase plot analysis. Phase plots considered the variability in consecutive wave forms from vertical movement and were quantified by SDA (spatiotemporal variability), SDB (temporal variability), ratio ∀ (ratio SDA:SDB) and Δangleβ (symmetry). Step time CoV was greater in manifest HD (p<0.01, both manifest groups) than controls, as was stride length CoV for HD2 (p<0.01). No differences were found in spatiotemporal variability between PreHD and controls (p>0.05). Phase plot analysis identified differences between manifest HD and controls for SDB, Ratio ∀ and Δangle (all p<0.01, both manifest groups). Furthermore Ratio ∀ was smaller in PreHD compared with controls (p<0.01). Ratio ∀ also produced the strongest correlation with UHDRS-TMS (r=-0.61, p<0.01) and was correlated with DBS (r=-0.42, p=0.02). Phase plot analysis may be a sensitive method of detecting gait changes in HD and can be performed quickly during clinical walking tests.

Relationship of circulatory BDNF with cognitive deficits in people with Parkinson's disease

BACKGROUND Brain-derived neurotrophic factor (BDNF) and cognitive function are diminished in people with Parkinsons disease (PD). The relationship of cognitive function and serum level of BDNF, however is yet to be examined. The aim of this study was to examine serum BDNF levels in PD. Subsequently, the relationship of cognitive function to the serum levels of BDNF was evaluated. METHODS Serum BDNF levels were measured in 29 idiopathic PD subjects and 30 healthy-matched controls using ELISA technique. Cognitive function was assessed using the Montreal Cognitive Assessment (MOCA) scale. RESULTS Serum BDNF levels and MOCA total score were significantly lower (P<0.001) in PD patients versus healthy controls. MOCA total score correlated with serum BDNF (r=0.44; P=0.012) but not with age, years of education, duration of disease and severity of symptoms. The regression analysis showed that serum BDNF accounted (P=0.019) for 19% of MOCA total score variance. CONCLUSIONS The data confirm lowered serum BDNF in PD. Additionally; it suggests that BDNF may play a role in the cognitive deficit of PD. Further studies are required to identify association of BDNF in cognitive decline with PD.

Practice, progress and future directions for physical therapies in Huntington's Disease

Physical therapies and exercise may have potential as a disease modifying agent in Huntingtons disease (HD) and in recent years, there have been several small scale feasibility studies that have shown benefit as a result of physical interventions. When evaluating complex physical interventions, a phased approach using mixed methodology designs that report specific intervention components, adherence, acceptability, adverse events and defined intervention protocols is important for replication and planning of future trials and to ensure potential for implementation in clinical practice. A narrative review of the available literature related to physical activity, physical therapy and exercise in people with HD was performed using a population, intervention, comparison and outcome (PICO) approach. Eight studies met specific inclusion criteria and were reviewed in terms of their systematic conduct and reporting standards. All of the studies (n = 8) provided details of intervention including location and duration. The majority of interventions included balance training activities in combination with other complex activities of daily living that required therapist supervision. Two of the interventions were home based, the remainder were facility or hospital based. None of the studies reported adverse events whilst only 3/8 reported adherence rates which were ranging from 60-80%. In general, limited detail was provided on the specific individual components of the interventions. This review of primary publications and conference proceedings, suggests that researchers working in the field need to focus on clearer reporting of intervention protocols so as to generate a clearer understanding of the impact of exercise and physical therapies on the symptoms of HD, as well as any potential synergistic role alongside the impending disease-modifying interventions.

F18 Clinical measurement of sit to stand performance in people with huntington's disease: reliability and validity for 30 seconds chair sit to stand test

Background People with HD often demonstrate difficulties with transitional movements that may be related to impairments in balance, strength or neuromuscular coordination. The 30s Chair sit to stand test (30s CST) is a test designed to measure the number of chair stands a person can complete in 30s. Aim This study aimed to examine test-retest reliability and concurrent validity of the 30s CST as a measure of transitional movement ability in people with HD. Methods Concurrent validity was assessed in the entire cohort (n=12) by comparison with measures of gait (velocity and cadence), balance (Timed up and Go (TUG) and Berg Balance Scale (BBS)), function (Physical Performance Test (PPT)) and disease severity (Total Functional Capacity (TFC) scale). Associations between the tests were examined using Pearson correlations. Reliability was assessed with intra-class correlations (ICC) (3,3) and 95% limits of agreement, in a sub-group of participants (n=7) who performed the 30s CST test twice with one week between testing. Results 12 subjects participated in the study (mean±SD, 54.6±15.27 years; 6.2±2 units in TFC scale). The 30s CST was not significantly correlated with TFC but correlations with measures of gait, balance and function were all significant. Velocity Cadence TFC TUG BBS PPT 30s CST Pearson correlation 0.86* 0.73* 0.45 −0.87* 0.85* 0.86* * p<0.01. Test-retest reliability was excellent (ICC=0.99). Variability of scores between the test sessions was small (95% limits of agreement (−0.498 to 1.64)). Conclusion 30s CST test is a simple measure that evaluates a task commonly impaired in HD. The test was not correlated with TFC; the fact that most subjects scored close to 6 may have influenced this result. The test however, has excellent reliability and concurrent validity with other measures of gait and balance; and therefore, it incorporates multiple domains of physical impairments in HD which makes it an important test to be included in future therapeutic trials.

Parkinson’s disease in Jordan: Barriers and motivators to exercise

ABSTRACT Background: Perceived barriers to engaging in exercise in people with Parkinson’s disease (PD) are becoming more defined in countries such as the UK and the US. This, however, may vary by culture and environment. This study aimed to explore the perceptions of exercise and barriers that may affect participation in people with PD from Jordan. Methods: Two focus groups and seven individual interviews were conducted with people with PD. Additionally, individual interviews were conducted with two neurologists. Conversations were digitally recorded and transcribed. Transcripts were analyzed using thematic analysis and validated via researcher triangulation and peer checking. Results: Most of the PD participants lacked previous participation in any disease-specific exercises. Several barriers were perceived by PD participants for such lack of participation. Barriers included difficulty of diagnosis, lack of informational support provided by neurologists, lack of referral to physiotherapy services, disease-specific issues, and setting-related issues. Neurologists indicated a number of barriers in counseling their PD patients on exercise including lack of time and lack of health system resources. Motivators to participate in future exercise included outcome expectations and family support. Conclusions: Findings of the study shed light into large areas of unmet needs of supporting exercise and physiotherapy for people with PD in developing countries as per Jordan. For better patient outcomes, findings of the study suggest that it is crucial to raise awareness among all PD-related stakeholders on the benefits of early referrals to physiotherapy and early engagement in exercise programs.