STUDY DESIGN: Observational population-based cohort study. OBJECTIVES: The main aim of this study was to examine the relative effect of hereditary spastic paraplegia (HSP) on the health-related quality of life (HRQoL). METHODS: HRQoL was evaluated using a RAND 36-Item Health Survey 1.0 questionnaire. Fifty-eight patients received a questionnaire through mail and signed an informed consent. The results for the control group were obtained from the RAND-36 data collected in 2004 in the European Social Survey. R2.9.0 and Statistica 6.1 were used to analyze the RAND-36 data. SETTING: The study was performed in Estonia, a country with a population of 1.3 million. RESULTS: Completed questionnaires were received from 49 participants (response rate was 84.5%). The control group consisted of 549 individuals from the Estonian population. Patients with HSP had lower mean scores in all categories as compared with the control group. Six of the eight categories showed significant differences, with P<0.0001. For the vitality category, the P-value ranged from 0.000006 from 0.002, and the P-value for the mental health category ranged from 0.001 to 0.055. CONCLUSIONS: The HRQoL in patients with HSP was found to be significantly worse than that for the general population. The level of education might affect the HRQoL experienced by HSP patients.
STUDY DESIGN: Observational population-based cohort study. OBJECTIVES: The main aim of this study was to examine the relative effect of hereditary spastic paraplegia (HSP) on the health-related quality of life (HRQoL). METHODS: HRQoL was evaluated using a RAND 36-Item Health Survey 1.0 questionnaire. Fifty-eight patients received a questionnaire through mail and signed an informed consent. The results for the control group were obtained from the RAND-36 data collected in 2004 in the European Social Survey. R2.9.0 and Statistica 6.1 were used to analyze the RAND-36 data. SETTING: The study was performed in Estonia, a country with a population of 1.3 million. RESULTS: Completed questionnaires were received from 49 participants (response rate was 84.5%). The control group consisted of 549 individuals from the Estonian population. Patients with HSP had lower mean scores in all categories as compared with the control group. Six of the eight categories showed significant differences, with P<0.0001. For the vitality category, the P-value ranged from 0.000006 from 0.002, and the P-value for the mental health category ranged from 0.001 to 0.055. CONCLUSIONS: The HRQoL in patients with HSP was found to be significantly worse than that for the general population. The level of education might affect the HRQoL experienced by HSP patients.
Authors: Krister W Fjermestad; Øivind J Kanavin; Eva E Næss; Lise B Hoxmark; Grete Hummelvoll Journal: Orphanet J Rare Dis Date: 2016-07-13 Impact factor: 4.123
Authors: Bas J H van Lith; Hans C J W Kerstens; Laura A C van den Bemd; Maria W G Nijhuis-van der Sanden; Vivian Weerdesteyn; Rob J E M Smeets; Klemens Fheodoroff; Bart P C van de Warrenburg; Alexander C H Geurts Journal: Orphanet J Rare Dis Date: 2020-03-04 Impact factor: 4.123