Objective: The aim of the study was to determine if available quality of life related scales are useful to detect the deterioration of quality of life in patients with early stage of the disease.

Background: Huntington’s Disease (HD) is neurodegenerative disorder that impairs severely everyday functioning of affected individuals.

Methods: We evaluated data of 22 patients, aged 51.1 years ±5.7, 15 females with early and moderately advanced HD (disease duration: 34.02 months, range: 0-75 months) enrolled in Registry and Enroll-HD studies during years 2007-2015. SF-36 scale, Total Functional Capacity (TFC), Functional Assessment Scale (FAS) and Independence Scale were performed at initial visit and follow-up visit after mean 34.6 months (range:12-72 months). We calculated correlations of these scales with motor symptoms measured with Unified Huntington’s Disease Rating Scale (UHDRS) and cognitive state evaluated with Stroop test, verbal fluency test, and symbol digit modality test.

Results: We did not observe significant deterioration of quality of life measured with all used scales. There was an average decrease of 3.4±0.9 in SF-36 (p=0.49), 2.0±0.7 in TFC (p=0.17), 2.0±0.7 in FAS (p=0.23). UHDRS increased from 37.6 to 42.2 (mean: 4.7±41.0, p=0.54), verbal fluency domain declined from 19.7 to 12.8 (mean: 7.0±0.8, p=0.1) and Stroop Word Reading Test from 57.4 to 52.2 (mean: 5.2.±7.8, p=0.37), Symbol Digit Modality Test from 25.7 to 24.2 (mean: 1.5± 9.9, p=0.72). None of the quality of life related scales did correlate significantly with UHDRS and psychological tests.

Conclusions: All applied the quality of life related scales are not sensitive enough to detect significant deterioration of quality of life in patients with early and moderately advanced disease.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/the-assessment-of-quality-of-life-in-patients-with-early-and-moderately-advanced-huntington-disease/