Longitudinal evaluation of quality of life in 288 patients with Neurofibromatosis 2

Ferner, R.E., Shaw, A., Evans, D.G., McAleer, D., Halliday, D., Parry, A., Raymond F, L., Durie-Gair, J., Hanemann C, O., Hornigold, R., Axon, P.R. and Golding, J.F. 2014. Longitudinal evaluation of quality of life in 288 patients with Neurofibromatosis 2. Journal of Neurology. 26 (5), pp. 936-969. https://doi.org/10.1007/s00415-014-7303-1

TitleLongitudinal evaluation of quality of life in 288 patients with Neurofibromatosis 2
AuthorsFerner, R.E., Shaw, A., Evans, D.G., McAleer, D., Halliday, D., Parry, A., Raymond F, L., Durie-Gair, J., Hanemann C, O., Hornigold, R., Axon, P.R. and Golding, J.F.
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.

JournalJournal of Neurology
Journal citation26 (5), pp. 936-969
ISSN0340-5354
Year2014
PublisherSpringer
Publisher's version
Digital Object Identifier (DOI)https://doi.org/10.1007/s00415-014-7303-1
Publication dates
Published12 Mar 2014

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Predicting individual differences in motion sickness susceptibility by questionnaire
Golding, J.F. 2006. Predicting individual differences in motion sickness susceptibility by questionnaire. Personality and Individual Differences. 41 (2), pp. 237-248. https://doi.org/10.1016/j.paid.2006.01.012

Motion sickness susceptibility fluctuates through the menstrual cycle
Golding, J.F., Kadzere, P. and Gresty, M.A. 2005. Motion sickness susceptibility fluctuates through the menstrual cycle. Aviation, Space, and Environmental Medicine. 76 (10), pp. 970-973.

Can tamoxifen relieve motion sickness?
Gianni, M.C., Golding, J.F. and Goldhirsch, A. 2005. Can tamoxifen relieve motion sickness? Annals of Oncology. 16 (10), pp. 1713-1714. https://doi.org/10.1093/annonc/mdi304

Motion sickness
Golding, J.F. and Gresty, M.A. 2005. Motion sickness. Current Opinion in Neurology. 18 (1), pp. 29-34.

Effect of a novel motion desensitization training regime and controlled breathing on habituation to motion sickness
Yen Pik Sang, F., Billar, J., Gresty, M.A. and Golding, J.F. 2005. Effect of a novel motion desensitization training regime and controlled breathing on habituation to motion sickness. Perceptual and Motor Skills. 18 (1), pp. 29-34.

Perceived control, locus of control and preparatory information: effects on the perception of an acute pain stimulus
Williams, D., Golding, J.F., Phillips, K. and Towell, A. 2004. Perceived control, locus of control and preparatory information: effects on the perception of an acute pain stimulus. Personality and Individual Differences. 36 (7), pp. 1681-1691. https://doi.org/10.1016/j.paid.2003.07.009

We are most aware of our place in the world when about to fall
Bray, A., Subanandan, A., Isableu, B., Ohlmann, T., Golding, J.F. and Gresty, M.A. 2004. We are most aware of our place in the world when about to fall. Current Biology. 14 (15), pp. R609-R610. https://doi.org/10.1016/j.cub.2004.07.040

Suppression of sickness by controlled breathing during mildly nauseogenic motion
Yen Pik Sang, F., Golding, J.F. and Gresty, M.A. 2003. Suppression of sickness by controlled breathing during mildly nauseogenic motion. Aviation, Space, and Environmental Medicine. 74 (9), pp. 998-1002.

Effect of breathing supplemental oxygen on motion sickness in healthy adults
Ziavra, N., Yen Pik Sang, F., Golding, J.F., Bronstein, A.M. and Gresty, M.A. 2003. Effect of breathing supplemental oxygen on motion sickness in healthy adults. Mayo Clinic Proceedings. 78 (5), pp. 574-578.

Behavioral methods of alleviating motion sickness: effectiveness of controlled breathing and a music audiotape
Yen Pik Sang, F., Billar, J., Golding, J.F. and Gresty, M.A. 2003. Behavioral methods of alleviating motion sickness: effectiveness of controlled breathing and a music audiotape. Journal of Travel Medicine. 10 (2), pp. 108-112.

Impairment of spatial cognitive function with preservation of verbal performance during spatial disorientation
Gresty, M.A., Waters, S., Bray, A., Bunday, K. and Golding, J.F. 2003. Impairment of spatial cognitive function with preservation of verbal performance during spatial disorientation. Current Biology. 13 (21), pp. R829-R830. https://doi.org/10.1016/j.cub.2003.10.013

Motion sickness and tilts of the inertial force environment: active suspension systems vs. active passengers
Golding, J.F., Bles, W., Bos, E., Haynes, T. and Gresty, M.A. 2003. Motion sickness and tilts of the inertial force environment: active suspension systems vs. active passengers. Aviation, Space, and Environmental Medicine. 74 (3), pp. 220-227.

A motion sickness maximum around the 0.2 Hz frequency range of horizontal translational oscillation
Golding, J.F., Mueller, A.G. and Gresty, M.A. 2001. A motion sickness maximum around the 0.2 Hz frequency range of horizontal translational oscillation. Aviation, Space, and Environmental Medicine. 72 (3), pp. 188-192.

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