PT  - JOURNAL ARTICLE
AU  - Junker, Johanna
AU  - Berman, Brian D
AU  - Hall, James
AU  - Wahba, Deena W
AU  - Brandt, Valerie
AU  - Perlmutter, Joel S
AU  - Jankovic, Joseph
AU  - Malaty, Irene A
AU  - Wagle Shukla, Aparna
AU  - Reich, Stephen G
AU  - Espay, Alberto J
AU  - Duque, Kevin R
AU  - Patel, Neepa
AU  - Roze, Emmanuel
AU  - Vidailhet, Marie
AU  - Jinnah, H.A.
AU  - Brüggemann, Norbert
TI  - Quality of life in isolated dystonia: non-motor manifestations matter
AID  - 10.1136/jnnp-2020-325193
DP  - 2021 Jun 01
TA  - Journal of Neurology, Neurosurgery & Psychiatry
PG  - 622--628
VI  - 92
IP  - 6
4099  - http://jnnp.bmj.com/content/92/6/622.short
4100  - http://jnnp.bmj.com/content/92/6/622.full
SO  - J Neurol Neurosurg Psychiatry2021 Jun 01; 92
AB  - Objective To evaluate the relationship between health-related quality of life (HR-QoL) and both physical and psychiatric factors in a large, international, multicentre cohort of patients with isolated dystonia, the Dystonia Coalition.Methods Natural history data from 603 patients with isolated dystonia (median age 57 years (IQR: 48 to 64 years), 67.0% women) were prospectively acquired and analysed. HR-QoL (RAND 36-Item Health Survey), severity of depressive symptoms, generalised anxiety (Hospital Anxiety and Depression Scale) and social anxiety (Liebowitz Social Anxiety Scale) were assessed. Dystonia severity (Burke-Fahn-Marsden Dystonia Rating Scale) and dystonic tremor were examined. Statistical predictors of HR-QoL were calculated using saturated path analysis.Results Reduced HR-QoL was strongly associated with the degree of depressive symptoms and generalised and social anxiety (8/8 RAND 36 subscales, p≤0.001). Increased dystonia severity was associated with worse physical functioning, physical and emotional role functioning and social functioning (all p≤0.001). The presence of tremor correlated with worse physical functioning and pain (all p≤0.006). Younger age was associated with reduced emotional well-being and vitality (all p≤0.006). There were no HR-QoL differences between sexes.Conclusion HR-QoL in isolated dystonia is strongly associated with psychiatric and physical features. While current standard of care focus on motor aspects of dystonia, comprehensive care should address both physical and mental aspects of health.Data are available upon reasonable request. Anonymised data (study protocol, statistical analysis) will be shared by request from any qualified investigator. Data will be available for 10 years.