This study of patients affected by CD assessed, through TWSTRS and SF-36 scores, the impact of a new rehabilitation program provided together with BTX injections. Patients with CD have many disabilities but in this study the PT program focused specifically on muscle weakness, limited range of motion, pain, and central motor and control deficits. The patients who completed the PT program reported improvements in pain, disease severity, and QoL.
A major aspect of CD is deficient muscle relaxation leading to a fixed dystonic posture and limited range of motion (2
). Impaired muscle relaxation may be caused by increased activity of agonist and antagonist muscles (co-contraction) and reduced voluntary and sequential movements (17
). Therefore, our proposed PT protocol including kinesiotherapy and tissue release techniques may help improve these patients’ QoL. It should be pointed out that this protocol can be easily reproduced by other therapists.
Tassorelli et al. (6
) reported significant differences between groups treated with BTX plus PT and BTX only in daily life abilities and subjective pain. Subjects receiving PT, consisting of kinesiotherapy, postural control exercises and biofeedback, showed improvements in both areas. These patients required a smaller dose of BTX at the next injection and also reported a longer-lasting toxin effect (days). These findings are partially consistent with our results since these authors did not find any improvements in TWSTRS scores for the pain subscale, whereas we did in G1 (BTX plus PT). One possible explanation for the improvement seen in our G1, as measured by the TWSTRS, is that our PT protocol duration was twice as long as that of Tassorelli et al. (6
Smania et al. (5
) investigated four patients and divided them into two groups to receive two different protocols. The first protocol consisted of biofeedback and the second one of muscle stretching and postural reeducation. Both groups showed improvements with similar results suggesting that the PT program showed therapeutic effects comparable to those of biofeedback.
In these two studies (5
), biofeedback was used because it acts on central mechanisms of motor learning. The basal ganglia play an important role in motor control, and patients with CD have dysfunctional motor planning, programming and execution, resulting in dystonic movements and a distorted body image (3
Although biofeedback is used worldwide, access to this treatment modality in Brazil is quite limited and for this reason we developed a program of repetitive motor learning exercises. All the exercises were performed under similar conditions and in a repetitive manner in order to stimulate conditioning mechanisms and create a more solid internal model, which would ensure better adaptation and task generation. Hauptmann and Karni (18
) reported that measurement of motor learning performance can be based on behavioral concepts. This was corroborated in our study as we found improved scores, especially on the TWSTRS, in G1 patients. But we were unable to find other similar studies for comparison of our results.
Another important aspect to underline is that patients with CD have weakened muscle strength compared with normal individuals. When pain is present in CD, it is also associated with impaired muscle strength (19
This study proposed the application of FES for the treatment of patients with CD. Muscle dystonia and abnormal postures impair effective and functional contraction of antagonist muscles, which may lead to muscle hypotonia due to disuse. In addition to muscle contraction of target non-dystonic cervical muscles, the external electrical current used in FES also causes, through reciprocal innervation, relaxation of dystonic muscles that have already been treated with BTX.
FES electrodes were placed on the surface of muscles contralaterally to dystonic muscles so that muscle contraction would move the head to its normal position and induce functional movement. The aim was to provide an adequate input for head positioning. We did not find other articles in English on FES used in patients with CD or other dystonic conditions, although this treatment approach has been applied in other neurological disorders characterized by muscle weakness or failed motor programming resulting in abnormal muscle contraction, such as stroke or other conditions causing spasticity (20
) reported a protocol applied to one patient with CD which resulted in improvement of dystonic posture, according to the TWSTRS scale, after manual strengthening of dystonic antagonist muscle groups. Zetterberg et al. (7
) also included manual muscle strengthening in a treatment protocol and improved QoL was seen in five out of six patients with CD. Three patients reported improved pain and better TWSTRS scores. Our study corroborates these findings.
Another way to evaluate patient response to treatment is by assessing self-perceived QoL. The assessment of QoL is recognized as an important component of the evaluation and management of patients with dystonia. In the provision of adequate healthcare to patients with dystonia, it is necessary to focus not only on reducing disease severity but also on managing key factors for improving QoL (21
General perceptual measures of QoL are useful tools in the treatment of patients with CD because functional quantitative scales are not able to detect significant changes achieved by BTX, neurological rehabilitation, and other therapies. The effect of a multidisciplinary approach to CD may be assessed by means of scales that combine motor function measures with an assessment of the impact of the disease on the patient’s daily life activities, and emotional and social functioning. Therefore, the SF-36 is a good tool and has been used in many studies to assess QoL in patients with CD (22
Most studies published on CD and PT did not include a QoL assessment in their analyses (4
). Although Zetterberg et al. (7
) reported improved QoL in five out of six patients, this improvement was exclusively attributed to the PT program, since no patients received BTX injections for at least three months before their enrollment in the study and there was no effect of BTX from previous injections.
Consistent with the results of Zetterberg et al. (7
), we found improvements on six of the eight SF-36 subscales in the patients treated with PT, while those who received BTX only did not show any improvement on any QoL-related subscales.
Although some studies have shown consistent improvements in QoL indicators after BTX treatment (22
), other investigators, like us, found mild or no improvement in QoL perception in controls treated with BTX only (15
). These conflicting findings may be due to the fact that the effect of BTX treatment varies over time, reaching a plateau right after injection and decreasing after that. Not all injections achieve the same effect; furthermore this is a long-term treatment and it needs to be repeated often because dystonic symptoms recur after a few months. These factors may reduce patient compliance with the treatment and have a negative impact on their QoL.
One limitation of this study is its non-random design. Since CD is not a very common condition, the number of patients with CD managed at our institution was small (n=70), which precluded the use of a randomized design. All the patients were interviewed and invited to participate in the study and our final sample size consisted of 20 subjects in the treatment group and 20 in the control group. The use of a non-random sample may have introduced bias. However, as there are few studies on CD and PT, this study may help physical therapists to make decisions about the management of CD and may encourage further randomized controlled studies on this approach.
Another aspect to be emphasized is treatment duration. In this a non-random sample of patients, the two groups, G1 and G2, were very similar in all respects, except treatment duration. This may have introduced some bias because the longer the treatment is, the less inclined the patient will be to participate in new treatment modalities such as PT, and the less prone to perceive any improvement following treatment. Patients enrolled in G2 also had longer disease duration than those in G1 (16 versus 9 years, respectively). This difference was not statistically significant, but it may have also introduced some bias, because the patients in G2 were more chronically ill and may have felt more despondent about their condition. Regardless of disease or treatment duration, it is worth mentioning that PT was a “new” treatment for all the patients enrolled in this study.
This study suggests that the combination of BTX injections and a PT program including kinesiotherapy, motor learning exercises, and muscle strengthening techniques, may improve disease severity, disability, pain, and QoL in patients with CD. This finding is in agreement with other studies on rehabilitation, although a direct comparison was not possible as each protocol had a different methodology and different techniques. Other studies are needed to further explore these findings and support neurological rehabilitation as an effective approach in the management of patients with CD.