Functional Capacity Score Research Articles

Avaliação da qualidade de vida dos pacientes submetidos à hemiartroplastia do quadril

ObjectiveThis was a comparative analysis on the quality of life of patients who underwent hemiarthroplasty of the hip, assessed before the operation and three and six months after the operation, by means of the SF‐36 questionnaire (Medical Outcomes Study 36‐Item Short Form health survey). MethodsA prospective study was conducted on 12 patients with femoral neck fractures who underwent partial hip arthroplasty between June 2013 and July 2014. Female patients predominated (58.3%). The mean age was 83 years and, in 91.7%, the fracture was due to falling from a standing position. The SF‐36 questionnaire was applied before the operation and three and six months after the operation. ResultsWith regard to physical health, the patients presented low scores for functional capacity and physical aspects. They had good scores for the subitem of general state and high scores regarding pain. Vitality, social aspect and mental health had moderate scores and emotional aspects had a low score. ConclusionWith the sample analyzed here, we can say that the hip hemiarthroplasty procedure for cases of unstable femoral neck fractures, in patients with low functional demands analyzed over a postoperative period of six months, does indeed allow quality of life to be maintained.

Surgery or EUS-guided choledochoduodenostomy for malignant distal biliary obstruction after ERCP failure.

Background and Objectives:Endoscopic retrograde cholangiopancreatography (ERCP) is the method of choice for drainage in patients with distal malignant biliary obstruction, but it fails in up to 10% of cases. Percutaneous transhepatic cholangiography (PTC) and surgical bypass are the traditional drainage alternatives. This study aimed to compare technical and clinical success, quality of life, and survival of surgical biliary bypass or hepaticojejunostomy (HJT) and endoscopic ultrasound (EUS)-guided choledochoduodenostomy (CDT) in patients with distal malignant bile duct obstruction and failed ERCP.Patients and Methods:A prospective, randomized trial was conducted. From March 2011 to September 2013, 32 patients with malignant distal biliary obstruction and failed ERCP were studied. The HJT group consisted of 15 patients and the CDT group consisted of 14 patients. Technical and clinical success, quality of life, and survival were assessed prospectively.Results:Technical success was 94% (15/16) in the HJT group and 88% (14/16) in the CDT group (P = 0.598). Clinical success occurred in 14 (93%) patients in the HJT group and in 10 (71%) patients in the CDT group (P = 0.169). During follow-up, a statistically significant difference was seen in mean functional capacity scores, physical health, pain, social functioning, and emotional and mental health aspects in both techniques (P < 0.05). The median survival time in both groups was the same (82 days).Conclusion:Data relating to technical and clinical success, quality of life, and survival were similar in patients who underwent HJT and CDT drainage after failed ERCP for malignant distal biliary obstruction.

F01 Vestibular Ocular Reflex Dynamics During Passive Head-impulses: Slow Phase And Quick-eye Movements In Huntington's Disease

Background Angular vestibular ocular reflex (VOR) can be non-invasively and easily assessed with the video head-impulse test (vHIT), allowing the quantification of VOR dynamic parameters (latency and gain) at a physiological frequency domain, as well as the characterisation of quick eye movements (QEM) triggered with head impulses. Here we characterise these findings in HD patients. Methods We explored 18 genetically confirmed HD patients (44.7 ± 8.1 years; male=9), classified as Shoulson and Fahn severity stages 1 (n = 5; pre-symptomatic), 2 (n = 4), 3 (n = 8) and 4 (n = 1) based on their Total Functional Capacity (TFC) scores, and 40 healthy controls (39.9 ± 16.5 years; male=20). We calculated the VOR latency and gain and determined the latency, peak-velocity and occurrence rate of the QEM triggered during and after head impulses. All patients had an oculomotor evaluation (spontaneous nystagmus, smooth pursuit, gaze fixation and saccades) before vHIT testing. Results VOR latency and VOR gain were not different from controls (p > 0.29 for both comparisons). No differences were found between presymptomatic and symptomatic patients. QEM were present in 11 patients and 18 controls, always after the head impulse and compensatory (overt saccades). QEM latency (174.5 ± 41.2 ms), peak velocity (71.58 ± 34.48 o/s) and occurrence rate (0.45 ± 0.29) were not different from controls (p > 0.11 for all comparisons). Though not realising it, 5 of the symptomatic patients majorly failed to direct gaze in darkness, both horizontal as vertically, this correlating with TFC scores (Spearman r = 0.65, p = 0.005). Conclusions VOR seems to be preserved at physiological frequency domains in HD patients, even in more advanced stages of the disease. Although both voluntary saccades and quick phases of nystagmus are known to be slower in HD, quick eye movements triggered with impulses showed no differences in comparison to controls. Gaze failure in darkness may prove beneficial as a biological marker for HD.

J25 Performance Of The 36-item And 12-item Whodas 2.0 In Prodromal Huntington Disease

Background The fifth edition of the Diagnostic and Statistical Manual for Mental Disorders (DSM-5) recommends the World Health Organisation Disability Assessment Schedule (WHODAS) 2.0 for routine clinical use. We tested the utility of the 36-item and 12-item WHODAS 2.0 in prodromal Huntington disease. Aims The aims of the current study are (1) to test disease progression group differences in baseline values and longitudinal change in a separate analysis of participant and companion ratings on the 36-item and 12-item WHODAS 2.0; (2) to compare longitudinal participant and companion ratings on the 36-item and 12-item WHODAS 2.0, and; (3) to compare the 36-item and 12-item WHODAS 2.0 with the Total Functional Capacity (TFC) score, in terms of detecting baseline and longitudinal differences. Methods Using data from 726 participants and 630 companions over a 3-year follow-up, linear mixed regression models were fitted and compared by Akaike’s information criterion. Participants were classified into four groups based on their gene status and baseline disease progression (control, low, medium, and high progression). Results For both the 36-item and 12-item WHODAS, participants and companions in the high group reported significantly worse functioning at baseline compared to controls. For longitudinal changes, companions but not participants in the medium and high groups reported significantly worse functional decline over time compared to controls. Participant and companion longitudinal trajectories showed divergence in the high group, suggesting reduced validity of self-report. The 12-item WHODAS detected longitudinal change better than the 36-item version and the TFC in the medium group. Conclusions Both the 36-item and 12-item WHODAS 2.0 can detect baseline and longitudinal differences in prodromal HD, and may be useful in HD clinical trials. Companions may provide more accurate data as the disease progresses.

D02 Associations Between Disease Stage And Levels Of Cytokines In Huntington's Disease

Background Several studies have demonstrated increased levels of pro-inflammatory cytokines in the serum of HD mutation carriers compared with healthy controls. Aims To investigate the association between disease stage as reflected in Total Functional Capacity score and motor score on the one hand, and levels of pro-inflammatory and anti-inflammatory cytokines on the other hand in the serum of HD mutation carriers, both cross-sectionally and longitudinally. Method Blood samples suitable for determination of cytokine levels were withdrawn from a cohort initially comprising 152 HD mutation carriers and 54 healthy controls at two follow-up measurements with an average interval of 2 years. Global daily functioning was assessed using the Total Functional Capacity (TFC) scale and motor symptoms were assessed by trained neurologists using the motor section of the Unified Huntington’s Disease Rating Scale (UHDRS). Using multilevel regression analyses, cross-sectional associations between disease stage and levels of cytokines were assessed. Longitudinal changes in levels of cytokines were assessed using paired t-tests. Results Compared to controls, mutation carries did not differ in their cytokine level increase over time. The pro-inflammatory cytokines IL-6 and TNF-alpha were higher in motorsymptomatic mutation carriers compared with pre-motorsymptomatic mutation carriers. The anti-inflammatory cytokine IL-5 was lower in motorsymptomatic mutation carriers compared with pre-motorsymptomatic mutation carriers. IL-6 increases significantly across progressing disease reflected in TFC score. Conclusion We found evidence for increasing levels of pro-inflammatory cytokines across progressing disease. In addition, levels of anti-inflammatory cytokines may decrease in later disease stages.

Changes in functional capacity in older adults living alone: a three-year longitudinal study in a rural area of Japan.

The purposes of this research were to clarify three-year changes in functional capacity and to investigate related physical health, psychological/mental health, and social health factors in older adults living alone in a rural area of Japan. Home visit interviews were conducted in 2000 (the baseline survey: n = 128) and in 2003 (the follow-up survey: n = 101) with older adults living alone in a town in Ishikawa prefecture. Seventy nine subjects were analyzed using logistic regression analysis at the follow-up survey. Of these 79 subjects, 40 persons had scores for functional capacity decreased by one or more points, the "Lowered" group (50.6%), while 27 persons were "Unchanged" (34.2%), and 12 persons were "Improved" (15.2%). Factors at the baseline survey were compared between the "Lowered" group and the "Unchanged/Improved" group. Significant factors that caused a decreased functional capacity were age (75 years old and over), no participation in social activities, and contact with friends/neighbors over the phone less than once a week. This research has shown that participation in social activities and contact with friends/neighbors maintain a higher functional capacity in older adults living alone.

Decline in functional capacity is unaffected by diet quality alone or in combination with physical activity among generally healthy older adults with T2D from the NuAge cohort

BackgroundBoth diet quality (DQ) and physical activity (PA) have been shown to play a role in the prevention of functional capacity (FC) decline. Because older adults (OA) with T2D are at a higher risk of FC decline compared to their non-diabetic counterparts, our aim was to determine if DQ alone, or combined with PA is associated with FC decline in OA with T2D over a 3-year follow-up in a secondary analysis of the NuAge cohort. MethodsIn 159 OA with T2D (mean age=75 years), FC change was calculated as the difference in FC scores at T1 and T4 measured by the SMAF (Système de Mesure de l’Autonomie Fonctionnelle). Baseline DQ was calculated from three non-consecutive 24-h dietary recalls collected at T1 using the validated Canadian Healthy Eating Index (C-HEI). PA change was calculated from Physical Activity Scale for the Elderly (PASE) as T4−T1. Associations were evaluated between FC decline and four combinations of variables: C-HEI score < or ≥70 with PASE change < or > median and analyzed by GLM while controlling for covariates. ResultsNeither DQ alone nor DQ combined with PA change were associated with FC decline over follow-up. ConclusionsThe absence of effect may be explained by characteristics of this healthy sample of OA with T2D who showed relatively good adherence to dietary recommendations (mean C-HEI=70) and were highly functional shown by minimal, clinically non-significant FC decline over 3 years. More research is needed to confirm the role of DQ in preventing FC decline in a larger diabetic sample showing clinically significant FC decline.

AB0794 Intra-Articular Injections in Thumb Osteoarthritis. A Systematic Review and Meta-Analysis of Randomized Controlled Trials

BackgroundIntra-articular injections of corticosteroids (CS) or hyaluronic acid (HA) are reported to be efficacious in hip and knee osteoarthritis (OA). In base of thumb OA (rhizarthrosis), information concerning intra-articular injections...

Neuropsychiatric symptoms in a European Huntington's disease cohort (REGISTRY)

BackgroundThe majority of Huntington's disease (HD) mutation carriers experience some psychopathology during their lifetime, varying from irritability to psychosis, but prevalences of particular symptoms vary widely due to diverse study...

The most appropriate primary outcomes to design clinical trials on Huntington's disease: meta‐analyses of cohort studies and randomized placebo‐controlled trials

Huntington's disease (HD) is a rare multifactorial neurodegenerative disease. Both its natural course and any placebo effect are poorly known. All are obstacles to design randomized controlled trials (RCTs). We conducted meta-analyses of RCTs and cohorts on all parameters of the Unified Huntington's Disease Rating Scale to determine the most appropriate outcomes and to minimize the number of patients required to design RCTs in HD. Twenty-four RCTs were included, involving 838 patients with a mean age of 50.0 ± 2.3 years and a mean total functional capacity (TFC) score of 9.8 ± 0.6. Nineteen cohorts were included involving 1939 patients with a mean age of 48.9 ± 2.3 years and a mean TFC of 10.1 ± 0.7. Significant deterioration was observed in RCTs for all scores except behavioral score. Effect sizes were comparable between RCTs and cohorts for each test except that there was a significant difference for TFC. The weighted mean deterioration per year on the TFC scale was -0.5 (0.2) in RCTs and -0.8 (0.2) in cohorts. The lowest number of patients required per group in a RCT was for TFC (19 per group), whereas 30 patients would be required per group for the total motor score (TMS). For cognition, the verbal fluency test required the smallest number of patients: 104 per group. In conclusion, TMS and TFC are the most appropriate outcomes to design RCTs on HD likewise the verbal fluency test for cognition. Our results suggest an effect of placebo administration on the total functional capacity.

Optimising mobility outcome measures in Huntington's disease.

Many of the performance-based mobility measures that are currently used in Huntington's disease (HD) were developed for assessment in other neurological conditions such as stroke. We aimed to assess the individual item-response of commonly used performance-based mobility measures, with a view to optimizing the scales for specific application in Huntington's Disease (HD). Data from a larger multicentre, observational study were used. Seventy-five people with HD (11 pre-manifest & 64 manifest) were assessed on the Six-Minute Walk Test, 10-Meter Walk Test, Timed "Up & Go" Test (TUG), Berg Balance Scale (BBS), Physical Performance Test (PPT), Four Square Step Test, and Tinetti Mobility Test (TMT). The Unified Huntington's Disease Rating Scale (UHDRS) Total Motor Score, Functional Assessment Scale and Total Functional Capacity scores were recorded, alongside cognitive measures. Standard regression analysis was used to assess predictive validity. Individual item responses were investigated using a sequence of approaches to allow for gradual removal of items and the subsequent creation of shortened versions. Psychometric properties (reliability and discriminant ability) of the shortened scales were assessed. TUG (β 0.46, CI 0.20-3.47), BBS (β -0.35, CI -2.10-0.14), and TMT (β -0.45, CI -3.14-0.64) were good disease-specific mobility measures. PPT was the best measure of functional performance (β 0.42, CI 0.00-0.43 for TFC & β 0.57 CI 0.15-0.81 for FAS). Shortened versions of BBS and TMT were developed based on item analysis. The resultant BBS and TMT shortened scales were reliable for use in manifest HD. ROC analysis showed that shortened scales were able to discriminate between manifest and pre-manifest disease states. Our data suggests that the PPT is appropriate as a general measure of function in individuals with HD, and we have identified shortened versions of the BBS and TMT that measure the unique gait and balance impairments in HD. These scales, alongside the TUG, may therefore be important measures to consider in future clinical trials.

Relationship between the Level of Education and Functional Capacity in Active Elderly Adults.

Both the level of education and functional capacity seems to be associated with the level of physical activity in the elderly. However, the relationship between the level of education and functional capacity in active elderly adults is poorly understood. To examine the association between the level of education and the functional capacity profile of active elderly adults. Cross-sectional. One hundred and four elderly men and 198 postmenauposal women (mean age: 62.7 ± 7.6 years old) were recruited among registered members of the YMCAs of Montreal who practiced at least one hour of structured physical activity per week. Participants were then divided in two groups based on their level of education (the cut-off point was the high-school diploma). Body composition (DXA), muscle strength (knee extensors, handgrip), estimated maximal oxygen consumption (2-km walk test), perceived health (SF-36) and functional capacity (timed up and go, alternate step and one-leg stance tests) were measured. The level of education of the participants was assessed by questionnaire. Body composition was similar between groups. We observed that all functional capacity tests as well as the global functional capacity score were significantly higher in the most educated group compared to the least educated group (p<0.05). In addition, the most educated group had significantly higher levels of knee extensors strength, estimated maximal oxygen consumption and perception of physical functioning. A higher level of education was associated with a favourable functional capacity profile in our cohort of active elderly adults. However, the mechanism(s) which could mediate this association remain(s) unknown.

The unified huntington's disease rating scale for advanced patients: Validation and follow‐up study

The Unified Huntington's Disease Rating Scale (UHDRS) adequately measures decline in patients at early and moderate stages of Huntington's disease (HD). In patients with advanced HD, floor effects hamper the evaluation, thus calling for an adjusted scale. We designed the UHDRS-For Advanced Patients (UHDRS-FAP) to improve longitudinal assessment of patients at the advanced disease stage. Sixty-nine patients with a Total Functional Capacity score ≤ 5 were recruited in France and the Netherlands. Among them, 45 patients were followed longitudinally (mean ± standard deviation, 1.6 ± 1.2 years) with the UHDRS-FAP; 30 patients also were assessed with the UHDRS. In cross-sectional analyses, the psychometric properties and inter-rater reliability of the scale were evaluated. Longitudinal analyses were used to evaluate the sensitivity to decline of the UHDRS-FAP compared with the UHDRS. Internal consistency was higher for motor (0.84) and cognitive (0.91) scores than for somatic (0.70) and behavioral (0.49) scores. Inter-rater reliability was ≥ 0.88 for all scores. The somatic score, which was specific to the UHDRS-FAP, declined over time along with motor and cognitive performance on both scales. Although performance with the two scales was correlated, the UHDRS-FAP appeared to be more sensitive to change and was the only scale that detected decline in patients with a Total Functional Capacity score ≤ 1. Neither scale detected a significant decline in behavioral scores. The results indicate that the UHDRS-FAP is reliable and more sensitive to change than the original UHDRS for cognitive and motor domains. It offers items that are relevant for daily care. Behavioral scores tended to decline, but this may reflect the decline in patients' communicative abilities.

A single-blind randomized controlled trial to evaluate the effect of 6 months of progressive aerobic exercise training in patients with uraemic restless legs syndrome

Uraemic restless legs syndrome (RLS) affects a significant proportion of patients receiving haemodialysis (HD) therapy. Exercise training has been shown to improve RLS symptoms in uraemic RLS patients; however, the mechanism of exercise-induced changes in RLS severity is still unknown. The aim of the current randomized controlled exercise trial was to investigate whether the reduction of RLS severity, often seen after training, is due to expected systemic exercise adaptations or it is mainly due to the relief that leg movements confer during exercise training on a cycle ergometer. This is the first randomized controlled exercise study in uraemic RLS patients. Twenty-four RLS HD patients were randomly assigned to two groups: the progressive exercise training group (n = 12) and the control exercise with no resistance group (n = 12). The exercise session in both groups included intradialytic cycling for 45 min at 50 rpm. However, only in the progressive exercise training group was resistance applied, at 60-65% of maximum exercise capacity, which was reassessed every 4 weeks to account for the patients' improvement. The severity of RLS symptoms was evaluated using the IRLSSG severity scale, functional capacity by a battery of tests, while sleep quality, depression levels and daily sleepiness status were assessed via validated questionnaires, before and after the intervention period. All patients completed the exercise programme with no adverse effects. RLS symptom severity declined by 58% (P = 0.003) in the progressive exercise training group, while a no statistically significant decline was observed in the control group (17% change, P = 0.124). Exercise training was also effective in terms of improving functional capacity (P = 0.04), sleep quality (P = 0.038) and depression score (P = 0.000) in HD patients, while no significant changes were observed in the control group. After 6 months of the intervention, RLS severity (P = 0.017), depression score (P = 0.002) and daily sleepiness status (P = 0.05) appeared to be significantly better in the progressive exercise group compared with the control group. A 6-month intradialytic progressive exercise training programme appears to be a safe and effective approach in reducing RLS symptom severity in HD patients. It seems that exercise-induced adaptations to the whole body are mostly responsible for the reduction in RLS severity score, since the exercise with no applied resistance protocol failed to improve the RLS severity status of the patients.

Cardiac magnetic resonance-derived right ventricular outflow tract systolic flow acceleration: a novel index of right ventricular function and prognosis in patients with pulmonary arterial hypertension

To validate the right ventricular outflow tract systolic flow acceleration (RVOTACC; peak flow velocity/time-to-peak velocity) measured by phase-contrast (PC)-cardiovascular magnetic resonance (CMR) as a novel index of right ventricular (RV) function, and to investigate its clinical implications in patients with pulmonary arterial hypertension (PAH). Thirty (38% male, 43 ± 15 years old) out of 55 consecutive patients who were initially diagnosed with PAH at the referral center were prospectively enrolled between March 2009 and July 2010 and were followed for PAH-related cardiovascular events for 2 years. The invasively measured maximum dP/dt (dP/dtmax) was used as an index of RV contractility. The PC-CMR-derived RVOTACC was compared with well-known prognostic parameters. The PC-CMR-derived RVOTACC correlated strongly with the dP/dtmax and estimated RV function more accurately than the CMR-derived RV ejection fraction. The CMR-derived RVOTACC level (HR = 0.87, 95% CI 0.78-0.98, p = 0.038) could be another powerful prognostic index compared with the functional capacity (hazard ratio [HR] = 0.88, 95% confidence interval [CI] 0.78-0.97, p = 0.035) and REVEAL Registry risk score (HR = 0.83, CI 0.56-0.95, p = 0.012). Furthermore, receiver-operating characteristic analysis identified ≥0.4 m/s2 as the optimal RVOTACC cut-off for predicting subsequent cardiovascular events. PC-CMR-derived RVOTACC is a promising non-invasively measured index of RV function and prognosis in patients with PAH.

Hallux Metatarsophalangeal Joint Arthrodesis With a Hybrid Locking Plate and a Plantar Neutralization Screw

Many techniques have been described for arthrodesis of the first metatarsophalangeal (MTP) joint. The purpose of this study was to determine the results of fixation using a low-profile dorsal titanium plate with locking screws in the phalanx, nonlocking screws in the metatarsal, and a plantar neutralization screw. Forty-nine consecutive patients (51 feet) underwent a first MTP joint arthrodesis during an enrollment period of 1 year from October 2010 to November, 2011. All patients were evaluated preoperatively for primary pathology, pain, function, radiographic findings, AOFAS scores, and physical exam findings. First MTP joint arthrodesis was performed with a precontoured dorsal titanium plate with preset valgus and dorsiflexion after the joint surfaces were prepared with dome-shaped power reamers to achieve congruous cancellous bone surfaces. At a minimum of 1-year follow-up, patients returned for postoperative evaluation of pain, function, radiographic findings, satisfaction, AOFAS scores, and physical exam findings. Forty-six of 49 (48 feet) patients returned for final follow-up examination at least 12 months after operative intervention. Forty-one patients (89%) reported good to excellent results. Visual analog pain scores improved from an average of 6.6 preoperatively to an average of 1.6 postoperatively (t = -9.3339, df = 45, P < .001). Functional capacity scores improved from a preoperative mean of 2.5 to a postoperative mean of 1.4 (t = -5.2648, df = 46, P < .001). AOFAS hallux MTP joint scores improved from a preoperative mean of 45 to a postoperative mean of 77 (t = 9.9498, df = 46, P < .003). Eighteen of 48 great toes (38%) had preoperative pronation whereas, 2 of 48 great toes (4%) had postoperative pronation. Eleven of 46 patients (24%) were unable to perform preoperative toe rise, and 8 of 46 (17%) were unable to perform postoperative toe rise. Twenty-five of 46 patients (54%) had gait improvement, while 19 patients (44%) had no change in gait, and 2 patients (4%) had gait deterioration. The mean preoperative hallux valgus angle of 27 degrees improved to a mean postoperative angle of 13 degrees (t = -6.1982, df = 46, P < .001). The mean preoperative 1-2 intermetatarsal angle of 12 degrees improved to a mean postoperative angle of 9 degrees (t = -5.2614, df = 46, P < .001). There was 1 delayed union (2%) and 1 nonunion (2%). Our outcome scores indicate that first MTP joint arthrodesis with a precontoured dorsal titanium plate with locking screws in the phalanx and nonlocking screws in the first metatarsal is both reliable and reproducible with a very high bony union rate. Level IV, prospective case series.

THU0523 High Impact of Obesity and Smoking Status on Patient Reported Outcomes of Ra Patients

BackgroundSmoking is a proven risk factor for RA severity, and overweight has a negative impact on global health.ObjectivesTo compare the patient-reported disease burden in relation to smoking status and body...

Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington's disease in the TRACK-HD study: analysis of 36-month observational data

TRACK-HD is a multinational prospective observational study of Huntington's disease (HD) that examines clinical and biological findings of disease progression in individuals with premanifest HD (preHD) and early-stage HD. We aimed to describe phenotypic changes in these participants over 36 months and identify baseline predictors of progression. Individuals without HD but carrying the mutant huntingtin gene (classed as preHD-A if ≥10·8 years and preHD-B if <10·8 years from predicted onset), participants with early HD (classed as HD1 if they had a total functional capacity score of 11-13 and HD2 if they had a score of 7-10), and healthy control individuals were assessed at four study sites in the Netherlands, the UK, France, and Canada. We measured 36-month change for 3T MRI, clinical, cognitive, quantitative motor, and neuropsychiatric assessments and examined their prognostic value. We also assessed the relation between disease progression and the combined effect of CAG repeat length and age. All participants were analysed according to their baseline subgroups. Longitudinal results were analysed using a combination of repeated-measure weighted least squares models and, when examining risk of new diagnosis, survival analysis. At baseline, 366 participants were enrolled between Jan 17, and Aug 26, 2008, and of these 298 completed 36-month follow-up: 97 controls, 58 participants with preHD-A, 46 with preHD-B, 66 with HD1, and 31 with HD2. In the preHD-B group, several quantitative motor and cognitive tasks showed significantly increased rates of decline at 36 months, compared with controls, whereas few had at 24 months. Of the cognitive measures, the symbol digit modality test was especially sensitive (adjusted mean loss 4·11 points [95% CI 1·49-6·73] greater than controls; p=0·003). Among psychiatric indicators, apathy ratings specifically showed significant increases (0·34 points [95% CI 0·02-0·66] greater than controls; p=0·038). There was little evidence of reliable change in non-imaging measures in the preHD-A group, with the exception of the speeded tapping inter-tap interval (0·01 s [95% CI 0·01-0·02] longer than controls; p=0·0001). Several baseline imaging, quantitative motor, and cognitive measures had prognostic value, independent of age and CAG repeat length, for predicting subsequent clinical diagnosis in preHD. Of these, grey-matter volume and inter-tap interval were particularly sensitive (p=0·013 and 0·002, respectively). Longitudinal change in these two measures was also greater in participants with preHD who received a diagnosis of HD during the study compared with those who did not, after controlling for CAG repeat length and age-related risk (p=0·006 and 0·0003, respectively). In early HD, imaging, quantitative motor, and cognitive measures were predictive of decline in total functional capacity and tracked longitudinal change; also, neuropsychiatric changes consistent with frontostriatal pathological abnormalities were associated with this loss of functional capacity (problem behaviours assessment composite behaviour score p<0·0001). Age and CAG repeat length explained variance in longitudinal change of multimodal measures, with the effect more prominent in preHD. We have shown changes in several outcome measures in individuals with preHD over 36 months. These findings further our understanding of HD progression and have implications for clinical trial design. CHDI Foundation.

Better global and cognitive functioning in choreatic versus hypokinetic‐rigid Huntington's disease

Understanding the relation between predominantly choreatic and hypokinetic-rigid motor subtypes and cognitive and general functioning may contribute to knowledge about different motor phenotypes in Huntington's disease. In the European Huntington's Disease Network Registry study, 1882 subjects were classified as being predominantly choreatic (n=528) or hypokinetic-rigid (n=432), according to their scores on items of the total motor score a priori labeled as choreatic or hypokinetic-rigid; the other 922 patients were of a mixed type. The relationship between motor type and cognitive (verbal fluency, symbol digit modalities, Stroop color, word and interference tests) and functional (total functional capacity) capacity was investigated using multiple linear regression. Motor subtype contributed significantly to the total functional capacity score (partial r(2) : 7.8%; P<.001) and to the 5 cognitive scores (partial r(2) ranged from 2.0% to 8.4%; all P<.001). Patients with a predominantly choreatic motor phenotype performing better in all areas than patients with a hypokinetic-rigid motor phenotype.

Short-term effects of ivabradine in patients with chronic stable ischemic heart disease.

Introduction:Ivabradine is a novel selective If current inhibitor with anti-ischemic and antianginal activity.Objectives:To assess the effect of the selective If current inhibitor ivabradine on heart rate, angina pectoris, and functional capacity in stable patients with chronic coronary artery disease on maximally tolerated medical therapy.Materials and Methods:Consecutive patients from the out-patient cardiology clinic with stable coronary artery disease documented by coronary angiography were included. Patients had to be on maximally tolerated medical therapy with β-blockers, angiotensin-converting enzyme inhibitors or receptor blockers (ACE-I or ARB), antiplatelets, statins, nitrates, and anti-metabolics with a baseline heart rate of at least 70 beats per minute. All patients underwent assessment of angina (Canadian Cardiovascular Society Angina Class: CCS I to IV) and functional capacity (using a validated self-administered questionnaire), at baseline and after 4 months of ivabradine therapy.Results:Twenty patients were enrolled (mean age 47 ± 7 years, all male, 60% with hypertension, 30% with diabetes mellitus). Patients were on optimal medical regimen of aspirin (100%), β-blocker (100%), statins (100%), clopidogrel (90%), nitrates (35%), anti-metabolics (90%), and ACE-I or ARB (95%). At baseline, the majority of patients (90%) were in CCS class II-IV. All patients were started on ivabradine 5 mg twice daily, and in 12 patients the dose was increased to 7.5 mg twice daily. After 4 months of treatment, the heart rate was significantly reduced from an average of 82 ± 8 to 68 ± 6 bpm (P < 0.001). The reduction in heart rate was accompanied by a significant improvement in functional capacity (score 3.5 ± 0.9 to 4.7 ± 0.7, P < 0.001) and angina classification; at baseline 10% of the patients were in CCS class I compared to 50% after 4 months of therapy (P = 0.01). No symptomatic bradycardia was reported with ivabradine.Conclusion:The addition of ivabradine to optimal medical therapy in patients with stable coronary artery disease is associated with significant improvement in anginal symptoms and functional capacity.