I20 The assessment of quality of life in patients with moderately advanced huntington’s disease

Background Huntington’s disease (HD) is neurodegenerative disorder that impairs severely everyday functioning. Aim The aim of the study was to determine if avalaible quality of life scales are useful to detect the deterioration of quality of life in patients with moderate stage of the disease. Methods We evaluated data of 22 patients with early and moderately advanced HD (average disease duration of 34 months) enrolled in REGISTRY and Enroll-HD studies. SF-36, Total Functional Capacity (TFC), Functional Assessment Scale (FAS) and Independence Scale were performed at initial visit and follow-up after mean 34.6 months. We calculated correlations of these scales with motor symptoms measured with Unified Huntington’s Disease Rating Scale (UHDRS) and cognitive state. Results We did not observe significant deterioration of quality of life measured with any of scales we used. There was an average decrease of 3.38 ± 0.9 in SF-36 (p = 0.49), 2.01 ± 0.71 in TFC (p = 0.17), 1.98 ± 0.71 in FAS (p = 0.23). UHDRS increased from 37.55 to 42.24 (mean 4.69 ± 41, p = 0.54), Reading Test from 57.4 to 52.2 (mean 5.23 ± 7.78, p = 0.37), Symbol Digit Modality Test from 25.68 to 24.18 (mean 1.54 ± 9.9, p = 0.72) Conclusions All applied quality of life related scales are not sensitive enough to detect significant deterioration of quality of life in patients with short disease duration.