Objective: To analyze predictors and progression of quality of life in patients with Huntington’s disease (HD), on a longitudinal assessment over two years.

Background: HD is an inherited neurodegenerative disease that affects motor, cognitive and neuropsychiatric functions. Quality of life (QoL) of HD patients is influence by these three domains.

Methods: We performed a longitudinal study (three consecutive evaluations over a two-year period) of 22 HD patients followed in the Neurogenetics Department of a University Hospital. The following data was collected: demographic (age, sex and education level), clinical (age of onset, duration of illness, presentation symptom) and genetic (CAG repeats and genetic transmission). Each patient was evaluated according to: Unified Huntington Disease Rating Scale (UHDRS) – motor score; Cognitive performance (symbol-digit, verbal fluency and stroop interference); Neuropsychological score (depression, drive/executive, irritability, psychosis and apathy); Functional assessment (Total Functional Capacity Score, independence and functional scale) and quality of life (Short Form Health Survey). Statistical analysis was performed using bivariate analysis. A p<0,05 was considered statistically significant.

Results: 59% of patients were female, the mean age of onset was 38.9 years, with a mean disease duration of 10.9 years. Neuropsychiatric symptoms were the most common form of presentation (45.5%). By univariate analysis we found that depression was the main determinant of QoL at the baseline evaluation (p<0,004). Apathy (p<0,009), drive/executive (p<0,024) and worse cognitive scores (p<0,018) were the major determinants in long-term evaluation. In our sample, functional capacities and motor score do not appear to have a significant impact on quality of life.

Conclusions: Early in the disease, neuropsychiatric symptoms seem to have a greater impact on QoL. On the other hand, worsening of cognitive scores throughout the evaluations becomes more important in determining QoL in later stages of the disease. Despite cognitive and behavioral impact on QoL, there are still no specific interventions in this area.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/predictors-of-quality-of-life-in-huntingtons-disease-a-longitudinal-observational-study/