{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T10:45:10Z","timestamp":1788259510087,"version":"build-2803163510"},"reference-count":13,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2011,6,1]],"date-time":"2011-06-01T00:00:00Z","timestamp":1306886400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2012,1]]},"abstract":"<jats:p><jats:bold>Background and purpose:\u2002<\/jats:bold> Hereditary spastic paraplegia (HSP) causes progressive gait disturbance because of degeneration of the corticospinal tract. To assess its impact on Health\u2010Related Quality of Life (HRQoL), we analyzed the correlation of HRQoL with disease severity and clinical symptoms in HSP.<\/jats:p><jats:p><jats:bold>Methods:\u2002<\/jats:bold> HRQoL was assessed by the Short\u2010Form 36 (SF\u201036) Mental and Physical Component summary scores (MCS and PCS) in 143 German patients with HSP. Disease severity was assessed by the Spastic Paraplegia Rating Scale (SPRS) and landmarks of walking ability. Patients with \u2018pure\u2019 or \u2018complicated\u2019 HSP were compared.<\/jats:p><jats:p><jats:bold>Results:\u2002<\/jats:bold> Higher SPRS scores indicating higher disease severity correlated significantly with lower PCS (<jats:italic>r\u2003<\/jats:italic>=\u2003\u22120.63; <jats:italic>P\u2003<\/jats:italic>&lt;\u20030.0005) and MCS (<jats:italic>r\u2003<\/jats:italic>=\u2003\u22120.38; <jats:italic>P\u2003<\/jats:italic>&lt;\u20030.0005) scores. MCS and PCS were reduced in patients with \u2018complicated\u2019 forms compared to \u2018pure\u2019 HSP and with decreasing walking ability.<\/jats:p><jats:p><jats:bold>Conclusion:\u2002<\/jats:bold> HRQoL is substantially impaired in patients with HSP and decreases with disease severity and the presence of \u2018complicating\u2019 symptoms. Patients are most affected by the physical restraints of their disease, but mental health is impaired as well. HRQoL is a valid parameter in HSP that should be considered in upcoming therapeutical trials.<\/jats:p>","DOI":"10.1111\/j.1468-1331.2011.03443.x","type":"journal-article","created":{"date-parts":[[2011,6,1]],"date-time":"2011-06-01T12:36:26Z","timestamp":1306931786000},"page":"168-171","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":36,"title":["Disease severity affects quality of life of hereditary spastic paraplegia patients"],"prefix":"10.1111","volume":"19","author":[{"given":"S.","family":"Klimpe","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"Sch\u00fcle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Kassubek","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Otto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Z.","family":"Kohl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Klebe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T.","family":"Klopstock","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Ratzka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K.","family":"Karle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Sch\u00f6ls","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2011,6]]},"reference":[{"key":"e_1_2_7_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(83)92879-9"},{"key":"e_1_2_7_3_2","doi-asserted-by":"publisher","DOI":"10.1038\/15472"},{"key":"e_1_2_7_4_2","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000228242.53336.90"},{"key":"e_1_2_7_5_2","first-page":"AS264","article-title":"Comparison of methods for the scoring and statistical analysis of SF\u201036 health profile and summary measures: summary of results from the Medical Outcomes Study","volume":"33","author":"Ware JE","year":"1995","journal-title":"Med Care"},{"key":"e_1_2_7_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/0920-1211(96)00024-1"},{"key":"e_1_2_7_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jclinepi.2005.11.001"},{"key":"e_1_2_7_8_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2003.033100"},{"key":"e_1_2_7_9_2","doi-asserted-by":"publisher","DOI":"10.1080\/17482960701553949"},{"key":"e_1_2_7_10_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2003.029660"},{"key":"e_1_2_7_11_2","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100005989"},{"key":"e_1_2_7_12_2","doi-asserted-by":"publisher","DOI":"10.1038\/sc.2008.17"},{"key":"e_1_2_7_13_2","doi-asserted-by":"publisher","DOI":"10.1038\/sc.2010.61"},{"key":"e_1_2_7_14_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.57.9.1319"}],"container-title":["European Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1468-1331.2011.03443.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1468-1331.2011.03443.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,30]],"date-time":"2023-10-30T08:11:50Z","timestamp":1698653510000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1468-1331.2011.03443.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,6]]},"references-count":13,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2012,1]]}},"alternative-id":["10.1111\/j.1468-1331.2011.03443.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1468-1331.2011.03443.x","archive":["Portico"],"relation":{},"ISSN":["1351-5101","1468-1331"],"issn-type":[{"value":"1351-5101","type":"print"},{"value":"1468-1331","type":"electronic"}],"subject":[],"published":{"date-parts":[[2011,6]]},"assertion":[{"value":"2011-06-01","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}