RT Journal Article
SR Electronic
T1 Quality of life in isolated dystonia: non-motor manifestations matter
JF Journal of Neurology, Neurosurgery & Psychiatry
JO J Neurol Neurosurg Psychiatry
FD BMJ Publishing Group Ltd
SP 622
OP 628
DO 10.1136/jnnp-2020-325193
VO 92
IS 6
A1 Johanna Junker
A1 Brian D Berman
A1 James Hall
A1 Deena W Wahba
A1 Valerie Brandt
A1 Joel S Perlmutter
A1 Joseph Jankovic
A1 Irene A Malaty
A1 Aparna Wagle Shukla
A1 Stephen G Reich
A1 Alberto J Espay
A1 Kevin R Duque
A1 Neepa Patel
A1 Emmanuel Roze
A1 Marie Vidailhet
A1 H.A. Jinnah
A1 Norbert Brüggemann
YR 2021
UL http://jnnp.bmj.com/content/92/6/622.abstract
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.