Health-Related Quality of Life in Multiple System Atrophy and Progressive Supranuclear Palsy

Abstract

Objective: Multiple system atrophy (MSA) and progressive supranuclear palsy (PSP), known as atypical parkinsonian syndromes (APS), are neurodegenerative disorders with severe disability and decreased life expectancy. Little is known about the health-related quality of life (HrQoL) and its determinants in patients with those disorders. The objective of our cross-sectional study was to evaluate the HrQoL in patients with APS and to identify the determinants of HrQoL. Methods: A total of 101 consecutive patients with MSA (n = 54) and PSP (n = 47) were recruited in four German neurological centers. Disease severity was assessed using the Hoehn and Yahr stages and the Unified MSA Rating Scale. The HrQoL was evaluated using the EuroQol instrument (EQ-5D and EQ-VAS). Independent determinants of HrQoL were identified in multiple regression analyses. Results: The mean EQ-VAS score was 52% lower than that reported for the general population (36.9 ± 18.3 vs. 77.4 ± 19.0). Of the study participants, 63% reported severe problems in at least one dimension of the EQ-5D. Cerebellar dysfunction was associated with a more considerable reduction of HrQoL. Independent determinants of reduced HrQoL were female gender, <12 years of education, disease severity, a decreased number of persons in the household and depression. Conclusions: The HrQoL in MSA and PSP is considerably reduced. While therapeutic options in the treatment of motor symptoms remain restricted, greater attention should be paid to the treatment of depression, which was identified among independent determinants of HrQoL. Independent determinants of HrQoL should be considered when developing healthcare programs aimed at improving the HrQoL in APS.

Challenges Faced by Patients With Progressive Supranuclear Palsy and their Families

“…A quality-of-life study published after the completion of our focus groups suggests that educating patients about PSP could improve health-related quality of life because a low level of education was an independent determinant of decreased quality of life. 13 Our findings support the call for patient education, but also point to the need to expand efforts to include education of health care providers.…”

Clinical characteristics and quality of life in Chinese patients with multiple system atrophy

“…The comparison of the SF-36 and EQ-5D between the two subtypes, after adjusting for disease duration, showed that MSA-P patients had worse PCS and MCS, as indicated by their scores on the SF-36, and worse pain/discomfort and anxiety/depression, according to their scores on the EQ-5D. The presence of parkinsonism seemed to bring about a more serious decline in HrQoL than the presence of cerebellar dysfunction, which is inconsistent with the results of another study (Winter et al, 2011). HrQoL was negatively associated with increased motor impairment and disease severity, as assessed by the HY staging scale and the UMSARS, indicating that disease progression contributed to the deterioration of HrQoL, previous studies have also confirmed these results (Chen et al, 2014;Schrag et al, 2006Schrag et al, , 2010Winter et al, 2011).…”

“…HrQoL was negatively associated with increased motor impairment and disease severity, as assessed by the HY staging scale and the UMSARS, indicating that disease progression contributed to the deterioration of HrQoL, previous studies have also confirmed these results (Chen et al, ; Schrag et al, ; Winter et al, ). Depression and anxiety scores were moderately correlated with HrQoL scores; other studies have reported a close association between depression and HrQoL (Schrag et al, ; Winter et al, ; Zhang et al, ). Autonomic dysfunction was correlated with HrQoL in one study (Schrag et al, ), but only a weak correlation with the HrQoL was found in the present study.…”

Functional impairment in progressive supranuclear palsy

“…1 As in other neurodegenerative conditions, patients with PSP also display difficulties with cognition, 2 psychiatric functioning, 3 and quality of life. 4 Functional disability observed in these patients is less clear, however.…”