Rating Scale Total Motor Score Research Articles

Utilisation of Healthcare and Associated Services in Huntington’s disease: a data mining study

Background: People with Huntington’s disease (HD) often require tailored healthcare and support packages that develop as the disease progresses. The Client Service Receipt Inventory (CSRI) gathers retrospective information on service utilization. This study investigated the use of formal services and informal care as measured by the CSRI and explored associations between informal care, disease severity and functional ability as measured by the Unified Huntington’s Disease Rating Scale Total Motor Score (UHDRS-TMS) and functional scales. Methods: All monitored longitudinal data from annual clinical assessments of UHDRS-TMS and functional assessments and CSRI collected under the auspices of the European Huntington’s Disease Network (EHDN) REGISTRY study between the years 2004 and 2009 were utilised in the analyses. Disease severity was reflected by UHDRS-TMS. Functional ability was measured using the UHDRS functional scales. CSRI data were analysed according to percentage use of individual formal services and total estimated hours per week of informal care. Regression analyses were conducted to identify any associations between disease severity, functional ability and hours of informal care.Results: 451 HD patients (212 female; 239 male) completed one visit; 105 patients (54 females; 51 males) completed two visits and 47 patients (20 females; 27 males) completed three visits in total over the 5 year period. The mean time between visits was 1.2 years. At visit one, 74% of the participants reported being in receipt of at least one formal hospital-based service in the previous six months, and 89% reported receipt of formal primary and community care services. In contrast, at the third visit, 62% of people had used hospital based services and 94% formal community based services in the previous six months. Fifty % of individuals required some form of informal care in the home at visit 1; this increased to 68% at visits 2 and 3. The mean (SD) estimated weekly total informal care hours at visits 1, 2 and 3 were 32.8 (49.4); 21.6 (53.6) and 21.3 (62.4) respectively. Only the scores on the Functional Assessment Scale (FAS) accounted for the variance in the weekly total informal care hours at each visit. Conclusions: Although it must be acknowledged that service use is supply driven, most HD patients across Europe surveyed as part of this study were in receipt of formal primary and community care services and to a lesser extent formal hospital based services. There was however a large reliance on informal care in the home. The FAS appear to have predictive value on informal care requirements and may have utility in facilitating pro-active service provision and in particular when managing carer burden in this population.

F09 Progression of motor symptoms prior to diagnosis in HD-gene carriers

BackgroundIdentifying sensitive, specific, and reliable indicators of disease progression in Huntington's disease (HD) is key to selecting the appropriate population for clinical trials that assess novel treatments that may slow...

Tongue force analysis assesses motor phenotype in premanifest and symptomatic Huntington's disease

Motor symptoms in Huntington's Disease (HD) are commonly assessed by the Unified Huntington's Disease Rating Scale-Total Motor Score (UHDRS-TMS). However, the UHDRS-TMS is limited by interrater variability, its categorical nature, and insensitivity in premanifest subjects. More objective and quantitative measures of motor phenotype may complement the use of the UHDRS-TMS as outcome measure and increase the power and sensitivity of clinical trials. Deficits in tongue protrusion are well acknowledged in HD and constitute a subitem of the UHDRS-TMS. We, therefore, investigated whether objective and quantitative assessment of tongue protrusion forces (TPF) provides measures that (1) correlate to the severity of motor phenotype detected in the UHDRS-TMS in symptomatic HD, (2) detect a motor phenotype in premanifest HD gene-carriers, and (3) exhibit a correlation to the genotype as assessed by a disease burden score (based on CAG-repeat length and age). Using a precalibrated force transducer, the ability of premanifest gene carriers (n = 15) and subjects with symptomatic HD (n = 20) to generate and maintain isometric TPF at three target force levels (0.25, 0.5, and 1.0 N) was assessed and compared with age-matched controls (n = 20) in a cross-sectional study. Measures of variability of TPF and tongue contact time distinguished controls, premanifest, and symptomatic HD groups and correlated to the UHDRS-TMS and disease burden score, suggesting a strong genotype-phenotype correlation. Group distinction was most reliable at the lowest target force level. We conclude that assessment of TPF may be a useful objective and quantitative marker of motor dysfunction in premanifest and symptomatic HD.

Early treatment benefits of ropinirole prolonged release in Parkinson's disease patients with motor fluctuations

We performed a retrospective analysis of the Efficacy And Safety Evaluation in Parkinson's Disease (EASE-PD) Adjunct Study, assessing the minimum time to symptom improvement after initiation of ropinirole prolonged release (2-24 mg/day) versus placebo in patients with moderate-to-advanced PD not optimally controlled with levodopa. Ropinirole prolonged release was superior to placebo at Week 2 for change from baseline in "off" time (adjusted mean treatment difference [AMTD] - 0.7 hours; 95% confidence interval [CI], -1.1, -0.2; P = 0.0029), and "on" time without troublesome dyskinesia (0.4 hours; 95%CI, 0.01, 0.88; P = 0.0444). At Week 4, improvements were seen in change from baseline in Unified Parkinson's Disease Rating Scale total motor score (AMTD, -3.1; 95%CI, -4.4, -1.8; P < 0.0001), activities of daily living score (AMTD, -1.1; 95%CI, -1.7, -0.5; P = 0.0004), and the cardinal symptoms of PD compared with placebo. These analyses indicate that once-daily, adjunctive ropinirole prolonged release can offer PD symptom control 2 weeks after treatment initiation.

A pilot study using nabilone for symptomatic treatment in Huntington's disease

Pilot study of nabilone in Huntington's disease (HD). Double-blind, placebo-controlled, cross-over study of nabilone versus placebo. Primary outcome, Unified Huntington's Disease Rating Scale (UHDRS) total motor score. Secondary measures: UHDRS subsections for chorea, cognition and behavior, and neuropsychiatric inventory (NPI). 44 randomized patients received either nabilone (1 or 2 mg) followed by placebo (n = 22), or placebo followed by nabilone (n = 22). Recruiting was straightforward. Nabilone safe and well tolerated, no psychotic episodes. Assessment of either dose of nabilone versus placebo showed a treatment difference of 0.86 (95% CI: -1.8 to 3.52) for total motor score; 1.68 (95% CI: 0.44 to 2.92) for chorea; 3.57 (95% CI: -3.41 to 10.55) for UHDRS cognition; 4.01 (95% CI: -0.11 to 8.13) for UHDRS behavior, and 6.43 (95% CI: 0.2 to 12.66) for the NPI. Larger longer RCT of nabilone in HD is feasible and warranted.

A teaching film, video library and online certification for the Unified Huntington's Disease Rating Scale Total Motor Score

Background: Clinical assessment of motor symptoms in Huntington's Disease (HD) is commonly performed using the Unified Huntington's Disease Rating Scale-Total Motor Score (UHDRS-TMS) (HSG 1996). Clinical diagnosis of HD is based on the presence of characteristic motor symptoms in the UHDRS-TMS. Therefore results of clinical trials are limited by (1) possible subjective error of raters (inter-rater and intra-rater variability), (2) the limited sensitivity of the categorical rating scale (non-continuous measure), and (3) insensitivity in pre-manifest subjects (de Boo et al., 1998).

Pharmacokinetic and Pharmacodynamic Comparison of Ropinirole 24-Hour Prolonged Release and Ropinirole Immediate Release in Patients With Parkinson's Disease

To explore the pharmacokinetic-pharmacodynamic relationship between systemic exposure to ropinirole and the primary efficacy end points from two phase 3 studies. Efficacy And Safety Evaluation in Parkinson's Disease (EASE-PD) Monotherapy (101468/168) compared ropinirole 24-hour prolonged release with ropinirole immediate release in patients with early Parkinson's disease (PD). Efficacy And Safety Evaluation in Parkinson's Disease Adjunct (101468/169) compared ropinirole 24-hour prolonged release or placebo in patients with advanced PD not optimally controlled with L-dopa. Sparse blood samples were collected for pharmacokinetic evaluations through population analysis. The relationship between ropinirole systemic exposure [steady-state area under the curve between time zero and 24 hours after dose, AUC(0-24,ss)] and change from baseline in Unified Parkinson's Disease Rating Scale (UPDRS) total motor score, and awake time spent off was investigated. In EASE-PD Monotherapy, the relationship between the decrease in UPDRS motor score and AUC(0-24,ss) was similar for both formulations, with a 60% to 80% probability of response for the exposure range studied. In patients with early PD, similar clinical benefit was achieved at AUC(0-24,ss) values associated with doses of 8 to 12 mg and higher doses (up to 24 mg). In EASE-PD Adjunct, the predicted probability of an off-time response for a patient on placebo was approximately 0.4, increasing to a near total probability of response rate (approximately 0.9) at higher systemic exposures of ropinirole 24-hour prolonged release. Characterization of the exposure-response relationship has identified that a dose range of 8 to 12 mg provides clinical benefit for the improvement in UPDRS total motor score in patients with early PD. Similarly, pharmacokinetic-pharmacodynamic analysis showed that, in patients with advanced PD, the probability of response increased with increasing exposures to ropinirole, indicating that doses more than 8 to 12 mg may lead to an improved benefit in parallel with reductions in L-dopa dose.

2.242 Relationship between Unified Parkinson's disease Rating Scale total motor score and systemic exposure of ropinirole 24-hour prolonged release in patients with early stage Parkinson's disease

2.242 Relationship between Unified Parkinson's disease Rating Scale total motor score and systemic exposure of ropinirole 24-hour prolonged release in patients with early stage Parkinson's disease

2.230 Pramipexole monotherapy in the treatment of early Parkinson's disease: Results of a 3 year follow up of a randomized controlled study

Objective: The aim of the study is to determine the effectiveness of pramipexole monotherapy in providing symptomatic relief in patients with early PD Method: The efficacy and tolerability of pramipexole is evaluated in a double blind, randomized, 3 year trial versus placebo. Patients with a diagnosis of idiopathic PD, with a modified Hoehn & Yahr score I−III, and a score more than 14 points in UPDRS part III at baseline were enrolled to the study. There were 20 patients in the treatment arm (mean age 61.7 yrs) and 20 age matched patients in the placebo group. The primary endpoint-the mean reduction in the Unified Parkinson’s disease Rating Scale (UPDRS) total motor score and change in Global Clinical Impression Scale (GCIS). Results: Patient characteristics were similar in the two groups at the beginning of the study. The pramipexole group showed a significantly important percent of responders (defined as a −40% decrease in UPDRS part III score at end point) compared to placebo (65% versus 11%; p< 0.005). Pramipexole treated group have a significantly greater improvement than placebo treated patients (p< 0.0001) in UPDRS and GCIS. At the end of 3 years the mean pramipexole dose was 4.7mg/day.12 patients in the placebo group were significantly disabled at the end of 2.8 years and were started either an agonist or levodopa at the end of the trial period (3 years). 19 patients in the pramipexole group are still taking pramipexole monotherapy, although some required dose adjustments. One patient in the pramipexole group required levodopa at the end of the trial. Side effects were mild, becouse all the patients used domperidon started at the beginning of the trial. There were no drop outs due to the side effects of the treatment. Conclusion: The results of this study reveals that pramipexole monotherapy can be considered as an effective and well tolerated first line treatment in patients with early PD

Physical Therapy for People with Huntington Disease

Huntington disease (HD) is a neurodegenerative disease that results in a progressive loss of functional abilities. Impairments in voluntary movements and the presence of involuntary movements are a contributing factor to functional decline. Despite the discovery of the gene that causes HD in 1993, effective interventions to retard disease progression continue to elude scientists and clinicians. Physical therapy is sometimes underutilized in people with HD, possibly due to the progressive nature of the disease, and the complexity of cognitive and psychiatric problems that are common in people with HD. Physical therapists can enable people with HD to optimize their quality of life and overall functional abilities by addressing health and wellness issues, educating them about falls prevention, teaching them how to minimize the effects of primary impairments such as dystonia and bradykinesia, and teaching them how to counteract the effects of secondary impairments on activity and participation. Case study: A 49-year-old male living at home, who was diagnosed with HD 17 years ago, participated in a 14-week exercise program to reduce impairments and activity limitations related to balance and postural control. Physical therapy consisted of a home program, which included exercises and tasks designed to promote balance, coordination, and flexibility. Over the course of the program, disability was reduced on numerous aspects of the SF-36, the number of falls decreased, self-selected walking speed increased by 0.77 ft/sec; performance on the Berg Balance Scale improved by 9 points, and the Unified Huntington Disease Rating Scale (UHDRS) total motor score decreased by 12 points. In light of these findings, physical therapy examination and treatment strategies are recommended as a basis for minimizing impairments and maintaining functional abilities in people with this progressive neurological condition.