J Neurol (2012) 259:1793–1800 contact with the patient, inﬂuenced the level of proxy rat- ings. However, the patient’s own psychological state (SF-12v2 Mental Component score) inﬂuenced proxy scores on the more ‘subjective’ Speciﬁc Scales of the HDQoL—Speciﬁc Hopes and Worries, Speciﬁc Mood State, Speciﬁc Self and Vitality—even after the patient’s disease stage was taken into account. Therefore, proxies’ ratings of psychosocial aspects of HrQoL were sensitive to patients’ psychological state, as well as symptom severity. In summary, this cross-sectional study addressed parameters of patient–proxy agreement on HrQoL for the ﬁrst time in HD, by comparing proxy assessment with patient self-report, using the disease-speciﬁc HDQoL. There was substantial agreement for the Summary Scale and also the Speciﬁc Scales and this suggests that the HDQoL proxy version yields generally similar scores to patients’ own, particularly on physical and functional aspects but also on more ‘subjective’ scales. On the Speciﬁc Hopes and Worries Scale, proxies on average rated HrQoL as better than patients’ self-ratings, while on both the Spe- ciﬁc Cognitive and Speciﬁc Physical and Functional Scales proxies tended to rate HrQoL more poorly than patients themselves. As more modest patient–proxy agreement was found for moderate-stage patients, proxy report should not be used as a substitute for patient-reported rating for these patients. As there was good patient–proxy agreement for advanced-stage patients, proxy evaluation using this ques- tionnaire could be a particularly useful complementary tool to self-report in advanced HD. Acknowledgments This study was funded by a Grant from the European Huntington’s disease Network to Aileen K Ho. The authors would like to thank all of the study participants and their family members who participated in the project and HDA UK for their kind support. The authors would also like to thank the European Hun- tington’s Disease Network by the Quality of Life Working Group: Walter Bucher, Alyson Bradbury, Beatrice de Schepper, John Eden/ Sue Beevers and team, George El-Nimr, Victor Hendrikx, Alis Hughes, Diana King, Ursula Kleibrink, Rita Kuttruff-Wilschut, Bernhard Landwehermeyer, Anne Lenon-Bird/Catherine Paradise and team, Christiane Lohkamp, Rhona MacLeod, Helene Padieu, Marie- Odile Perrousseaux, Asuncion Martinez, Lilliane Rapaille, Helen Santini, Pavla Sasinkova, Beverley Soltysiak, Steve Smith, Hans van der Leer, Lucienne van der Meer, Jo Anne Watton and team, Marleen van Walsem, Michael Wooldridge, Paola Zinzi. 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