Quality of life in isolated dystonia: non-motor manifestations matter

Abstract

ObjectiveTo 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.MethodsNatural 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.ResultsReduced 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.ConclusionHR-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.

The Dystonia Coalition: A Multicenter Network for Clinical and Translational Studies

“…This scale had known deficiencies in its clinimetric properties including inconsistent scoring among items, double weighting of duration factors, and variable approach to different aspects of the disorder (74,75). Additionally, the scale neglected non-motor features such as depression and anxiety, which are known to have a strong impact on quality of life (9,51,(76)(77)(78)(79)(80)(81)(82). This project was designed to address these shortcomings by producing and clinimetrically validating the Comprehensive Cervical Dystonia Rating Scale, which has three modules addressing motor features, non-motor features, and quality of life.…”

“…Dystonia causes substantial disability (8,9). For example, cervical dystonia is associated with neck muscle spasms that make it difficult for patients to control head movements for basic activities of daily living such as looking straight ahead to drive a car, read, see a computer or television screen, or even walk.…”

“…These abnormalities and limitations of mobility and pain degrade quality of life. In fact, standardized tests for quality of life in dystonia fall in the same range as patients with Parkinson's disease, multiple sclerosis, and stroke (8,9).…”

Study protocol: multimodal physiotherapy as an add-on treatment to botulinum neurotoxin type A therapy for patients with cervical dystonia: DysPT-multi—a prospective, multicentre, single-blind, randomized, controlled study

“…In the case of CD, this affects cervical and neck muscles, which results in an abnormal head or neck position or motion [ 3 ]. The majority of all patients also suffer from pain [ 4 ] and further from non-motor symptoms like depression or social phobia [ 5 , 6 ]. Therapeutic options are purely symptomatic and not causal, meaning that only an alleviating of the symptoms is possible.…”

Patients' Perspective of Dystonia Symptoms during the SARS‐CoV‐2 Pandemic

“…The present study suggests that the pandemic's exceptional circumstances worsened both motor and nonmotor symptoms in most dystonia patients, in line with previous reports. 1,2 Interestingly, nonmotor symptoms have been increasingly recognized in dystonia but are still poorly understood, 3,4 which were most frequently reported and contributed to clinical worsening. Our results suggest that exercise and rehabilitation may also impact on both motor and nonmotor symptoms, and their role in dystonia warrants further study.…”