BackgroundHuntington's disease (HD) is a fatal inherited neurodegenerative disease, caused by a

The authors report the inter-rater reliability and factor structure of the Short Problem Behaviors Assessment (PBA-s), a semistructured interview to measure severity and frequency of behavioral problems in Huntington's disease. Video recordings of 410 PBA-s interviews were rescored by an independent rater, and Cohen's kappa calculated to assess inter-rater reliability. The mean kappa was 0.74 for severity and 0.76 for frequency scores, whereas weighted kappa (allowing scores to differ by 1 point) was 0.94 for severity and 0.92 for frequency scores. The results of factor analysis were consistent with previous studies using other measures. The authors conclude that the PBA-s is a reliable measure.

The effect of ambulatory oxygen use during pulmonary rehabilitation (PR) has not yet been clearly established, but many studies have shown benefit from oxygen acutely. Two small studies to date demonstrated no clear benefit when oxygen was used in patients who desaturated on exertion during a PR programme. One study showed the benefit of using oxygen during PR in patients who were normoxic at rest and desaturated to a minimum of 88% on exertion. We conducted a single-blind, randomised controlled trial comparing PR undertaken either with or without ambulatory oxygen, in those with demonstrable benefit from oxygen at baseline. Subjects from three PR services were recruited who, during baseline assessment, desaturated by more than 4% and to less than 90% on exertion, and walked 10% or more further with ambulatory oxygen on endurance shuttle walk test. Patients were randomised to either room air or oxygen via portable cylinder, titrated to optimise pulse oxygen saturation but up to a maximum flow rate of 6 L/min. All patients then completed a twice weekly, 6-to 7-week PR programme. Data were analysed as per protocol. Totally 51 patients completed the study. At the end of PR, both groups improved with PR, with patients in the oxygen group demonstrating a highly statistically significantly greater mean improvement in endurance walking distance than the controls, 490 m (95% confidence interval 228-750; p 0.001), as well as clinically, although not statistically, significant changes in quality of life. The use of ambulatory oxygen during a 6-to 7-week PR programme greatly improved endurance walking distance in patients who desaturated on exertion with a positive acute response to ambulatory oxygen at baseline.

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This study explores the structural relationship between self-report and interview measures of affect in Huntington's disease. The findings suggest continued use of both to recognize the multidimensionality within a single common consideration of distress.

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