Introduction
Methodology
Protocol
Search Strategy
Inclusion and exclusion criteria
Synthesis of results
Assessment of bias
Compliance with ethical guidelines
Results
Selected studies
Total number of papers included in this review
| 75 |
Type of paper (%)
| |
Case report | 4 (5.3) |
Case series | 49 (65.3) |
Case-controlled study | 13 (17.3) |
Randomized controlled trial | 7 (9.3) |
Pharmaco-economic | 2 (2.7) |
Total number of patients
| 6,528 |
Mean number of patients, per paper (SD)
| 87.0 (145.5) |
Mean age of dystonia onset, in years
| 47.6 |
Male to female ratio
| 1:2 |
Type of dystonia studied, number of papers (%)
| |
Blepharospasm | 10 (13.3) |
Cervical | 25 (33.3) |
Laryngeal | 4 (5.3) |
Musician’s | 1 (1.3) |
Oromandibular | 3 (4.0) |
Segmental | 3 (4.0) |
Generalized | 3 (4.0) |
Various types | 26 (34.7) |
Number of publications per decade
| |
Until 2000 | 4 (5.3) |
2000–2009 | 33 (44.0) |
2010–2018 | 38 (50.7) |
Patient demographics
Type of dystonia | Number of papers | Number of patients | Male:female | Mean age of dystonia onset |
---|---|---|---|---|
Blepharospasm | 10 | 378 | 2:5 | 46.9 years |
Cervical | 25 | 2771 | 1:2 | 45.7 years |
Laryngeal | 4 | 75 | 1:4 | N/A |
Musician’s | 1 | 243 | 2:7 | 34.6 years |
Oromandibular | 3 | 72 | 1:2 | 52.0 years |
Segmental | 3 | 42 | 4:3 | 45.9 years |
Generalized | 3 | 49 | 1:1 | N/A |
Assessment of QoL
Questionnaire | N (%)a |
---|---|
30-item Hemifacial Spasm Quality of Life (HSF-30) | 1 (1.3) |
7-item Hemifacial Spasm Quality of Life (HFS-7 QOL) | 1 (1.3) |
Cervical Dystonia Impact Profile-58 (CDIP-58) | 4 (5.3) |
Clinical Global Impression Scale | 2 (2.7) |
Craniocervical dystonia questionnaire (CDQ24) | 6 (8.0) |
Dry Eye-Related Quality of Life Score (DEQS) | 1 (1.3) |
EQ-5D | 9 (12.0) |
Fugl-Meyer questionnaire | 1 (1.3) |
Glasgow Benefit Inventory | 4 (5.3) |
Nottingham Health Profile (NHP) | 2 (2.7) |
Oromandibular dystonia questionnaire (OMDQ-25) | 2 (2.7) |
PDQ-39 | 1 (1.3) |
Questionnaire— not specified | 3 (4.0) |
Questionnaire on Life Satisfaction | 1 (1.3) |
SF-20 | 1 (1.3) |
SF-36 | 39 (52.0) |
Spitzer Quality of Life Index (SQLI) | 1 (1.3) |
Visual Function Questionnaire (VFQ-25) | 2 (2.7) |
Voice Handicap Index (VHI) | 2 (2.7) |
Voice-Related Quality of Life (V-RQOL) | 2 (2.7) |
WHOQOL-BREF | 1 (1.3) |
Cervical Dystonia
Determinants of QoL in CD
Age
Disease severity
Comorbidity with mood disorders
QoL aspects in CD
Psychiatric features
Pain
Impact on work and employment
Fatigue
Sleep disturbances
Effect of treatment on QoL in CD
Botox in CD
BTX combined with physical therapy
Deep brain stimulation of globus pallidus internus in CD
DBS of subthalamic nucleus in CD
Peripheral denervation in CD
Blepharospasm
Determinants of QoL in BEB
Disease duration
Gender
Cognitive impairment
Psychiatric features
Effect of treatment on QoL in BEB
BTX in BEB
Laryngeal dystonia
Focal hand dystonia: writer’s cramp and musician’s dystonia
Lingual and oromandibular dystonia
Segmental dystonia
Generalized dystonia
Pharmaco-economic studies and the burden of poor QoL
Conclusions
-
Research into QoL in dystonia is increasing. The increasing research interest reflects in part the advancement in understanding QoL is an equally important endpoint for patients with dystonia as objective outcomes.
-
Implications of this clinically, are these elements affecting QoL may help us refine our treatment options and reorient our treatment goals.
-
Overall findings suggest patients with any form of dystonia have a reduced QoL compared to healthy controls. The reasons underpinning this vary not exclusively to the clinical characteristics of the dystonia, but to other factors such as social and demographic variables, and non-motor symptoms. Inherently, the stigma attached to dystonia requires significant social adaptation and coping mechanisms by patients, in turn impacting on QoL. This calls for further clinical research in the field as well as regular routine monitoring and assessment of QoL in dystonic patients.
-
The review highlighted the majority of studies in the literature focus on QoL in CD and BEB, reflecting the respective higher prevalence of these subtypes. This is a prerequisite for more research looking into QoL in the other subtypes of dystonia.
Limitations
-
Outcome measures such as the SF-36 scale are self-reported questionnaires; thus there is potential for an inherent response bias. This questions whether or not the QoL scales are really reflecting the true nature of the QoL of patients who have dystonia.
-
A more comprehensive search using other databases, rather than PubMed alone, may have produced a greater number of articles suitable for final analysis.
-
Studies should not be excluded due to language, rather they should be professionally translated, which was not possible in this case due to financial and time constraints.