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dc.contributor.authorFerner, RE
dc.contributor.authorShaw, A
dc.contributor.authorEvans, DG
dc.contributor.authorMcAleer, D
dc.contributor.authorHalliday, D
dc.contributor.authorParry, A
dc.contributor.authorRaymond, FL
dc.contributor.authorDurie-Gair, J
dc.contributor.authorHanemann, Clemens Oliver
dc.contributor.authorHornigold, R
dc.contributor.authorAxon, P
dc.contributor.authorGolding, JF
dc.date.accessioned2015-10-06T13:24:47Z
dc.date.available2015-10-06T13:24:47Z
dc.date.issued2014-05
dc.identifier.issn0340-5354
dc.identifier.issn1432-1459
dc.identifier.urihttp://hdl.handle.net/10026.1/3574
dc.description.abstract

Advances in molecular biology have resulted in novel therapy for neurofibromatosis 2-related (NF2) tumours, highlighting the need for robust outcome measures. The disease-focused NF2 impact on quality of life (NFTI-QOL) patient questionnaire was assessed as an outcome measure for treatment in a multi-centre study. NFTI-QOL was related to clinician-rated severity (ClinSev) and genetic severity (GenSev) over repeated visits. Data were evaluated for 288 NF2 patients (n = 464 visits) attending the English national NF2 clinics from 2010 to 2012. The male-to-female ratio was equal and the mean age was 42.2 (SD 17.8) years. The analysis included NFTI-QOL eight-item score, ClinSev graded as mild, moderate, or severe, and GenSev as a rank order of the number of NF2 mutations (graded as mild, moderate, severe). The mean (SD) 8.7 (5.4) score for NFTI-QOL for either a first visit or all visits 9.2 (5.4) was similar to the published norm of 9.4 (5.5), with no significant relationships with age or gender. NFTI-QOL internal reliability was good, with a Cronbach's alpha score of 0.85 and test re-test reliability r = 0.84. NFTI related to ClinSev (r = 0.41, p < 0.001; r = 0.46 for all visits), but weakly to GenSev (r = 0.16, p < 0.05; r = 0.15 for all visits). ClinSev related to GenSev (r = 0.41, p < 0.001; r = 0.42 for all visits). NFTI-QOL showed a good reliability and ability to detect significant longitudinal changes in the QOL of individuals. The moderate relationships of NFTI-QOL with clinician- and genetic-rated severity suggest that NFTI-QOL taps into NF2 patient experiences that are not encompassed by ClinSev rating or genotype.

dc.format.extent963-969
dc.format.mediumPrint-Electronic
dc.languageen
dc.language.isoeng
dc.publisherSpringer Science and Business Media LLC
dc.subjectNeurofibromatosis 2
dc.subjectNF2
dc.subjectNFTI-QOL
dc.subjectVestibular schwannoma
dc.titleLongitudinal evaluation of quality of life in 288 patients with neurofibromatosis 2
dc.typejournal-article
dc.typeEvaluation Study
dc.typeJournal Article
dc.typeMulticenter Study
dc.typeResearch Support, Non-U.S. Gov't
plymouth.author-urlhttps://www.webofscience.com/api/gateway?GWVersion=2&SrcApp=PARTNER_APP&SrcAuth=LinksAMR&KeyUT=WOS:000335772500015&DestLinkType=FullRecord&DestApp=ALL_WOS&UsrCustomerID=11bb513d99f797142bcfeffcc58ea008
plymouth.issue5
plymouth.volume261
plymouth.publication-statusPublished
plymouth.journalJournal of Neurology
dc.identifier.doi10.1007/s00415-014-7303-1
plymouth.organisational-group/Plymouth
plymouth.organisational-group/Plymouth/Faculty of Health
plymouth.organisational-group/Plymouth/Faculty of Health/Peninsula Medical School
plymouth.organisational-group/Plymouth/REF 2021 Researchers by UoA
plymouth.organisational-group/Plymouth/REF 2021 Researchers by UoA/UoA01 Clinical Medicine
plymouth.organisational-group/Plymouth/Research Groups
plymouth.organisational-group/Plymouth/Research Groups/Institute of Translational and Stratified Medicine (ITSMED)
plymouth.organisational-group/Plymouth/Research Groups/Institute of Translational and Stratified Medicine (ITSMED)/CBR
plymouth.organisational-group/Plymouth/Users by role
plymouth.organisational-group/Plymouth/Users by role/Academics
plymouth.organisational-group/Plymouth/Users by role/Researchers in ResearchFish submission
dc.publisher.placeGermany
dcterms.dateAccepted2014-02-25
dc.identifier.eissn1432-1459
dc.rights.embargoperiodNot known
rioxxterms.versionofrecord10.1007/s00415-014-7303-1
rioxxterms.licenseref.urihttp://www.rioxx.net/licenses/all-rights-reserved
rioxxterms.licenseref.startdate2014-05
rioxxterms.typeJournal Article/Review


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