Ferreira_EU Neurology 10/03/2010 10:38 Page 75
Pain and Quality of Life in the Treatment of Cervical Dystonia
10 and 20%.
14,15
In fact, in 20–32% of patients CD may progress to Table 1: Quality of Life Domains Most Affected in
segmental or generalised dystonia.
14,15 Cervical Dystonia Patients
Most cases of CD are idiopathic. Nevertheless, the finding of a family
Domains of SF-36
history of dystonia (12% of cases)
3
or of any movement disorder (44%
Role limitation physical
of cases)
16
suggests a genetic susceptibility. Secondary causes of CD
Role limitation emotional
include tardive and acute dystonia due to dopamine receptor blocking
Mental health
Social functioning
agents, neck trauma (central and peripheral trauma are less frequent),
Physical functioning
metabolic disorders such as Wilson’s disease and pantothenate-
As assessed by the Medical Outcomes Study Short-Form 36 (SF-36) scale.
kinase-associated neurodegeneration or Parkinson’s disease.
Pain and Quality of Life in Cervical Dystonia
Table 2: Main Determinants of Poor Quality of Life
in Cervical Dystonia
Pain
The presence of pain in CD is frequent and constitutes a distinctive
Determinants
feature not usually present in other focal dystonias. Patients tend to
Pain
report a diffuse pain over the neck and shoulders that usually
Depression
irradiates to the side of head deviation.
17
Anxiety
In one study of 266 patients with idiopathic CD, pain occurred in 75%
of the patients and had a significant impact on disability,
3
while it
Table 3: Assessment Tools for Pain and Quality of Life
in Cervical Dystonia
was reported by 68% in another series of 300 patients.
9
Pain was
associated with more prolonged and/or severe head turning along
Pain Quality of Life
with the presence of transient spasms of the head.
3
Patient self-assessment analogue scales SF-36
TWSTRS pain subscale EQ-5D
The mechanism of pain in CD remains mostly unknown. A small study
CDQ24
has suggested a decreased threshold of pain perception in CD
CDIP-58
patients,
18
which could point to the involvement of central processes
TWSTRS = Toronto Western Spasmodic Torticollis Rating Scale; SF-36 = Medical Outcomes
in addition to local muscle involvement.
17,19
Other potential causes of
Study Short-Form 36; EQ-5D = EuroQol questionnaire; CDQ24 = The Craniocervical Dystonia
Questionnaire; CDIP-58 = The Cervical Dystonia Impact Profile.
pain in CD are orthopaedic complications including cervical spine
degeneration, spondylosis, disc herniation, vertebral subluxations and
fractures, radiculopathies and myelopathies.
19–22
The association of major impact of CD on pain
25,28,29,32
and in domains of role limitation
these orthopaedic lesions with pain is not clear, despite their (physical and emotional), mental health and social and physical
prevalence in CD ranging between 18 and 41% and patients with CD functioning,
24,25,28,32
as measured by the Medical Outcomes Study
having an increased risk of secondary lesions of the upper cervical Short-Form 36 (SF-36), a generic QoL measure (see Table 1).
spine.
19,20
Studies have also failed to find a correlation between the Depression and anxiety are consistently reported as the main
presence or severity of pain and the presence or degree of spinal determinants of QoL in people with CD, in addition to pain
27–29,30,32,33
changes.
17,19
Nevertheless, the presence of spinal lesions was (see Table 2). Additional variables reported as contributing to poor
identified as a predictor of less improvement in pain and dystonia QoL were older age, low education level, unemployment, being
after selective peripheral denervation surgery.
19
One study did not find separated/divorced and severity of dystonia; on the other hand,
a conclusive association between the presence of pain and muscle longer duration of disease, physical activity and treatment
tenderness; the results were significant when tenderness was satisfaction were associated with a better QoL.
24,25,27–30,34
Two studies
assessed by muscle palpation, but not when measured by pressure have also identified self-perceived disfigurement and self-esteem
algometry.
17
In cases of CD treated with botulinum toxin, pain was also as contributors to QoL, although with weaker effect estimates in
associated with decreased muscle strength of the neck.
23
multivariable analysis.
28,30
Quality of Life The QoL of CD patients is comparable to that seen in patients with
Dystonias have a negative impact on quality of life (QoL).
24–28
A study multiple sclerosis or Parkinson’s disease, or long-term survivors of
on focal, segmental and generalised dystonias found that the QoL of stroke;
24
however, it is better than the QoL reported by patients with
dystonic patients was worse in all domains, but mainly in those generalised forms of dystonia.
28,31
Compared with other focal
related to physical and social functioning, compared with the norms dystonias, such as blepharospasm or limb dystonia, CD patients score
for the general UK population.
28
Poor QoL is observed even when poorer in the domain of pain, but perform similarly otherwise.
25,29
using different assessment tools, comprising both generic and
disease-specific instruments.
24–28
The results regarding gender effect How to Evaluate Pain and Quality of Life in
on QoL differ, but they suggest either no difference between males Cervical Dystonia
and females
28,29
or a worse QoL for females.
24,30
Several scales have been used to evaluate CD, incorporating both
objective and subjective measures (see Table 3). The most widely
CD has a negative impact on QoL compared with age- and gender- used is the Toronto Western Spasmodic Torticollis Rating Scale
matched healthy control subjects.
24,25–29,31
Several studies have tried (TWSTRS),
35
along with the Burke-Fahn-Marsden Dystonia Scale
36
and
to identify the most affected domains and the major determinants the Tsui Scale.
37
The TWSTRS (range 0–87 points) consists of three
of QoL in patients with CD.
24,25,27–34
Many studies have reported a subscales – severity (range 0–35), disability (range 0–23) and pain
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