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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. © 2013 International Parkinson and Movement Disorder Society

Objective: To investigate the relationships between cognitive impairment and apathy in patients with early Huntington’s disease (HD) and to further explore the influence of depression on the outcome of cognitive changes associated with apathy. Methods: We included 36 early HD patients, among them 20 were apathetic (HDA) and 16 were not (HDnA). The two groups were matched by age, education and severity of disease. Cognitive functions were evaluated by a comprehensive neuropsychological battery that measures memory, attention, executive function, language and visuospatial abilities. Results: The HDA patients had significantly lower scores on memory, attention and executive function tests when compared with the HDnA patients (p values Discussion: The presence of apathy is associated with more severe deficits of attention, executive function and episodic memory in early HD patients. Furthermore, the findings suggest that depression has little or no effect on cognitive deficits. Finally, apathy increased in parallel with both motor and cognitive dysfunction.

PURPOSE: Huntington's disease is a rare condition. Patients are commonly treated with antipsychotics and tetrabenazine. The evidence of their effect on disease progression is limited and no comparative study between these drugs has been conducted. We therefore compared the effectiveness of antipsychotics on disease progression. METHODS: 956 patients from the Huntington French Speaking Group were followed for up to 8 years between 2002 and 2010. The effectiveness of treatments was assessed using Unified Huntington's Disease Rating Scale (UHDRS) scores and then compared using a mixed model adjusted on a multiple propensity score. RESULTS: 63% of patients were treated with antipsychotics during the survey period. The most commonly prescribed medications were dibenzodiazepines (38%), risperidone (13%), tetrabenazine (12%) and benzamides (12%). There was no difference between treatments on the motor and behavioural declines observed, after taking the patient profiles at the start of the drug prescription into account. In contrast, the functional decline was lower in the dibenzodiazepine group than the other antipsychotic groups (Total Functional Capacity: 0.41 ± 0.17 units per year vs. risperidone and 0.54 ± 0.19 vs. tetrabenazine, both p

Short-term blood pressure (BP) variability predicts cardiovascular complications in hypertension, but its association with large-artery stiffness is poorly understood and confounded by methodologic issues related to the assessment of BP variations over 24 hours. Carotid-femoral pulse wave velocity (cfPWV) and 24-hour ambulatory BP were measured in 911 untreated, nondiabetic patients with uncomplicated hypertension (learning population) and in 2089 mostly treated hypertensive patients (83% treated, 25% diabetics; test population). Short-term systolic BP (SBP) variability was calculated as the following: (1) SD of 24-hour, daytime, or nighttime SBP; (2) weighted SD of 24-hour SBP; and (3) average real variability (ARV), that is, the average of the absolute differences between consecutive SBP measurements over 24 hours. In the learning population, all of the measures of SBP variability showed a direct correlation with cfPWV (SD of 24-hour, daytime, and nighttime SBP, r =0.17/0.19/0.13; weighted SD of 24-hour SBP, r =0.21; ARV, r =0.26; all P P

Few studies have evaluated exhaled NO measurement during acute asthma.To evaluate exhaled NO fraction (FE(NO)) and peak expiratory flow (PEF) time-courses during acute asthma treatment (beta 2-agonist plus systemic steroid) and to assess whether FE(NO) time-course predicts subsequent asthma control.Sixty-five asthmatic patients (mean +/- SD, 34 +/- 10 years) were prospectively enrolled in three Emergency Departments.Sixteen patients were excluded (failure of offline FE(NO) measurement at 100 mL/s [FE(NO 0.1)], n = 4, and early discharge). The 49 remaining patients performed FE(NO 0.1) and PEF on admission, at the 2nd (H2) and 6th hour (H6). Follow-up using an Asthma Control Diary was obtained in 27 of 49 patients, whether they were hospitalized (n = 9) or discharged (n = 18). All but 2 patients had elevated FE(NO) on admission (median [interquartile], 49 [26-78] ppb). Unlike PEF, mean FE(NO 0.1) of our sample was not significantly modified by treatment. No significant relationship was evidenced between exhaled NO and PEF variations. The variation of FE(NO 0.1) [H0 minus H6] was different in patients who were hospitalized (decrease of 8 +/- 20 ppb) versus discharged (increase of 5 +/- 20 ppb, p = 0.04). This variation of FE(NO 0.1) was correlated with the Diary score (control of subsequent week), an initial increase in FE(NO 0.1) being associated with better asthma control. Nevertheless, neither exhaled NO nor PEFR were good predictors of asthma control.An increase in FE(NO) is observed in almost all patients with acute asthma, and its subsequent increase within 6 hours is associated with a better degree of asthma control in the subsequent week.