Effectiveness of a standardized physical therapy program in cervical dystonia; a single blind randomized controlled trial.

Rationale and background
Cervical Dystonia (CD), or torticollis is a disabling neurological disorder
characterized by abnormal positions of the head due to involuntary muscle
contractions of the neck. The posture in cervical dystonia patient can feature
one or a combination of
postures: rotation (torticollis); lateral tilting (laterocollis); flexion
(anterocollis);extension (retrocollis); and lateral shift. Pain is experienced
by 75% of the patients and daily life activities are limited. With an estimated
prevalence of 5.7 patients per
100.000 persons in Western Europe, CD is the most common form of dystonia. The
pathophysiology of CD and dystonia in general, is largely unclear.
Neuro-imaging and electrophysiological studies point towards functional
abnormalities in the circuit
sensorimotor cortex, basal ganglia, brainstem and cerebellum. Reduced
inhibition in these circuits leads to over activity of the sensorimotor cortex
resulting in overflow of performed movements Over the last decade, increasing
evidence points towards maladaptive neuroplasticity of the central nervous
system in dystonia.
Neuroplasticity is the capacity of neurons and neural networks in the brain to
change connections and behaviour in response to new information, sensory
stimulation, development, or damage. Altered mechanisms for neuroplasticity due
to a genetic
pre-disposition combined with external triggering factors like trauma or
repetitive training form currently the main hypothesis in the pathophysiology
of dystonia.
The treatment options for CD are mainly symptomatic, aimed at reducing
involuntary movements, correcting abnormal head positions and reducing pain.
Currently, the best evidence based treatment option is to inject the dystonic
neck muscles with
Botulinum Toxin (BTX). BTX improved head postures and pain in 84% of the CD
patients with a mean improvement of 7.7 points on the TSUI-scale (severity) and
7.1 point on the TWSTRS disability scale.
In addition to BTX treatment, most CD patients are referred for PT. However,
theevidence for the effects of PT on CD are very limited.(10) Only two small
RCT*s with a short period of high intensity PT, investigated the effects of a
PT program on CD. The RCT by Tassoreli et al.(compared a combination of BTX
injections and a PT program to BTX injections alone in 40 patients after two
weeks of treatment. The outcome showed significant positive effects on pain and
disability, without differences on the severity of CD. The second RCT, compared
similar treatment
options and showed significant improvements on pain, active range of motion
(AROM) and disability scores in the group that received BTX combined with PT
after 6 weeks of treatment. In conclusion, both studies showed added value of
PT in combination with BTX injections mainly in the domain of disability. In
both studies, intensity of PT was high and duration was short, varying from 40
minutes per session every other day for six weeks up to 90 minutes a day for 2
weeks. It is difficult to extrapolate the positive results of both RCT*s to the
current regular care of chronic diseases given by physical therapists. For most
patients and therapists it will not be feasible to combine such an intensive
program with their daily lives and practice. Furthermore, a longer treatment
period seems more
appropriate for the long term effects in CD patients. A more practical and
international accepted approach towards the treatment of CD is
described by the physiotherapist J.P. Bleton. Unfortunately, the effect of the
Bleton treatment has never been investigated in a large randomized trial. The
hypothesis of his treatment is towards alteration of neuroplastic changes in
dystonia by massed practice methods i.e. to repetitively perform exercises to
learn new motor skills. The main goals of this method are to rehabilitate the
antagonist muscles and to control the dystonic movements by training frequently
in a functional way. Exercises are teached during one or two PT sessions a
week. After teaching the patients, intensive training is required in the
patient*s own environment (up to 10 times a day for 10 minutes). In addition,
patients are encouraged to correct the dystonic posture during their daily live
activities by turning their head in the opposite direction of the dystonic
posture. The Bleton method is unfortunately, not a standardized method.
We are currently developing an official Dutch physical therapy treatment
guideline physiotherapists and exercise therapists. It will be developed
according the AGREE standards in corporation with the Royal Dutch Society for
Physiotherapy (KNGF), the Society for Cesar therapy and Mensendieck (VvOCM) and
the HvA. The guideline is developed within the DystonieNet, a national
collaboration between neurologists
and paramedics in research, education and treatment of CD. The guideline will
be a standardized, best-evidence and best-practice guideline, adapted to
standard Dutch practice management. Shortly, the guideline recommends a one
year PT-program that
emphasizes independent practice in the patient*s own environment. During the
first six months patients receive weekly PT session to learn the new motor
skills with additional home based exercises. During the second half year
patients receive one session a month and will be learned how to maintain or
improve their newly gained motor skills independently. The definite treatment
guideline is expected in
September 2011.
The effect of this standardized PT program according the treatment guideline in
combination with BTX treatment will be tested and compared to BTX treatment
combined with regular physical therapy. Regular PT usually consists of one
session a week with interventions like massage, muscle stretching and training
of the dystonic muscles. Regular PT is mainly focused on training of the
dystonic muscles in contrast to the method described by Bleton aiming to
rehabilitate the antagonist muscles. The therapists of the regular therapy are
not trained to treat patients according the treatment guideline. The reason to
compare the guideline treatment with regular treatment instead of no treatment
in the control group is that we want to rule out the (placebo) effect of
attention due to therapy in general. By giving PT treatment to both groups all
the patients receive regular attention by a therapist.
In addition, an economic evaluation will be performed by comparing the costs
and health utility of the new standardized PT program with the regular PT
treatment. We expect the standardized PT program to be more cost effective
compared to regular PT as fewer sessions are required (34 sessions vs. 52
sessions), combined with the expectation that it*s more effective to improve
daily life activities and consequently,
employment status and productivity.

Primary objective
The primary objective is to evaluate the effectiveness of a standardized PT
program according to the treatment guideline on improvement of the ability to
perform daily life tasks compared to regular PT as add-on treatment to
BTX-injections in CD patients.

Secondary objectives
The secondary objectives are to investigate the effectiveness of a standardized
PT program according to the treatment guideline in comparison to regular PT as
add-on treatment to BTX-injections on:
- Severity of dystonia
- Pain
- Quality of life
- Anxiety and depression
- Healthcare costs and health utility

Data will be collected at baseline, after six months of physical therapy and
after one year of physical therapy on the same day as patients receive BTX
injections by the principal investigator.

Experimental group
The experimental group receives a one year PT program according the guideline
in combination with BTX injections. The PT program will start two weeks after
the injections, when the beneficial effects of the BTX are optimal. The PT
program will be given by a specially trained physical therapist. The intensity
of the PT program will be higher during the first six months in order to
stimulate plastic changes in the central nervous system. During the first
month, patients will receive two PT sessions of 30 minutes each week. During
the following five months patients receive a PT session of 30 minutes once a
week. During the second six months, patients will receive one PT session each
month focused on coaching the patient and independent continuation of the
exercises. Daily home based exercises have to be performed up to 5 times a day
for 10-15 minutes for maintaining and increasing the made progression during
the PT sessions. The emphasis of the PT program is on the functional
performance of the exercises adapted to daily life situations. Reverse Movement
Rehabilitation for example, is aimed at placing the head in the opposite
direction of the dystonic pull to reactivate the corrector muscles and gain
more voluntary control over the dystonic movements. Daily activities like
watching TV, computer work and household tasks need to be adapted so the head
is positioned in the opposite direction of the dystonic pull. Exercises will
become more difficult during the course of the PT program so the patient will
have complete voluntary control through the complete range of motion of the
head at the end of the study. Passive mobilizations and stretching of the
dystonic muscles are used to create the optimal conditions for free and
controlled movement of the head.

Control Group
The control group will receive BTX injections and regular PT for one year. The
weekly sessions will be retrieved from the local PT*s.

Physical therapy according the guideline does not have any extra risks compared
to standard physical therapy.