Citations
Citations
Dystonia has a wide range of causes, but treatment of dystonia is limited to minimizing the symptoms as there is yet no successful treatment for its cause. One of the optimal treatment methods for dystonia is chemodenervation using botulinum toxin type A (BTX-A), alcohol injection, etc., but its success depends on how precisely the dystonic muscle is selected. Here, we reported a successful experience in a 49-year-old post-stroke female patient who showed paroxysmal repetitive contractions involving the right leg, which may be of dystonic nature. BTX-A and alcohol were injected into the muscles which were identified by dynamic polyelectromyography. After injection, the dystonic muscle spasm, cramping pain, and the range of motion of the affected lower limb improved markedly, and she was able to walk independently indoors. In such a case, dynamic polyelectromyography may be a useful method for selecting the dominant dystonic muscles.
Citations
Dystonia is a movement disorder characterized by involuntary muscle contractions. Patients with dystonia may experience uncontrollable twisting, repetitive movements, or abnormal posture. A 55-year-old man presented with an involuntary left forearm supination, which he had experienced for five years. There was no history of antecedent trauma to the wrist or elbow. Although conventional therapeutic modalities had been performed, the symptoms persisted. When he visited our hospital, electromyography was performed. Reduced conduction velocity was evident at the elbow-axilla segment of the left median nerve. We suspected that there was a problem on the median nerve between the elbow and the axilla. For this reason, we performed an ultrasonography and magnetic resonance imaging study. A spindle-shaped soft tissue mass was observed at the left median nerve that suggested the possibility of neurofibroma. Dystonia caused by traumatic or compressive peripheral nerve injury has often been reported, but focal dystonia due to a neurogenic tumor is extremely rare. Here, we report our case with a review of the literature.
Citations
Paroxysmal autonomic instability with dystonia (PAID) is a rare complication of brain injury. Symptoms of PAID include diaphoresis, hyperthermia, hypertension, tachycardia, and tachypnea accompanied by hypertonic movement. Herein, we present the case of a 44-year-old female patient, who was diagnosed with paraneoplastic limbic encephalopathy caused by thyroid papillary cancer. The patient exhibited all the symptoms of PAID. On the basis that the symptoms were unresponsive to antispastic medication and her liver function test was elevated, we performed alcohol neurolysis of the musculocutaneous nerve followed by botulinum toxin type A (BNT-A) injection into the biceps brachii and brachialis. Unstable vital signs and hypertonia were relieved after chemodenervation. Accordingly, alcohol neurolysis and BNT-A injection are proposed as a treatment option for intractable PAID.
Citations
To investigate the effect of deep brain stimulation (DBS) on reducing dystonia and disability in adults with cerebral palsy (CP) and to compare the therapeutic outcomes between primary dystonia patients and CP patients over two years after bilateral pallidal DBS.
Five patients with primary dystonia and seven CP patients with dystonia were recruited. All subjects received DBS surgery in both globus pallidus. Burke-Fahn-Marsden dystonia rating scale consisting of dystonia movement score and disability score and subjective satisfaction scale were assessed after 1 month and every 6 months over two years following DBS treatment.
On the dystonia movement scale, both groups of primary dystonia patients and CP patients showed a significant decrease over time following DBS. On the disability scale, patients with primary dystonia showed a significant decrease over time, whereas the disability score of CP patients did not change over the two years. Comparing the dystonia movement and disability scores of CP patients at each assessment, patients with primary dystonia showed a significant reduction after 6 months. Comparing the satisfaction scores of CP patients after DBS, patients with primary dystonia showed significantly higher subjective satisfaction.
Whereas dystonia can be significantly reduced in patients with primary dystonia, CP patients showed a modest improvement on the dystonia movement scale, but not on the disability scale. Therefore, DBS may be considered with caution as a treatment modality of CP patients with dystonia.
Citations
To examine whether the clinical severity of cervical dystonia (CD) significantly correlates with 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) findings as well as to determine the threshold of the clinical severity of CD for positive 18F-FDG PET/CT study findings.
Forty-seven subjects with torticollis as one of the symptoms of CD were included. The clinical severity of CD was evaluated with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) at the time of 18F-FDG PET/CT. The correlation between the clinical severity of CD and the highest SUVmax was examined. The threshold of the clinical severity of CD necessary for positive 18F-FDG PET/CT findings was determined using receiver operating characteristics curve analysis.
Thirty-three of the 47 subjects (70.21%) showed positive 18F-FDG PET/CT findings. The ipsilateral splenius capitis/cervicis, oblique capitis inferior, and longus colli/capitis were the rotators most frequently involved. The highest SUVmax of 18F-FDG PET/CT was significant correlated with the TWSTRS. Subjects with a total TWSTRS exceeding 39 showed positive 18F-FDG PET/CT findings, with those having a total TWSTRS ≤22 showing negative 18F-FDG PET/CT results. The cutoff value of the total TWSTRS for positive 18F-FDG PET/CT findings was set at 27.5 with 90.9% sensitivity and 64.3% specificity.
A significant correlation was evident between the clinical severity of CD and 18F-FDG PET/CT findings, providing a threshold of the clinical severity of CD for acquisition of positive 18F-FDG PET/CT findings.
Citations
To evaluate the usefulness of 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) in the management of cervical dystonia (CD) with botulinum toxin type A (BoNT-A) injection.
Thirty two subjects with CD were included. A BoNT-A injection was provided either by clinically targeting method (group 1) or by 18F-FDG PET/CT-assisted, clinically targeting method (group 2). In group 2, selection of target muscles and dosage of BoNT-A were determined according to the increased 18F-FDG uptake, in addition to physical examination and functional anatomy. The outcomes of BoNT-A injection was compared between the two groups, in terms of the number of subjects who had reinjection before and after 6 months, the number of reinjections, the interval of reinjections, the duration to the minimal Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS), the number of adverse events, the reduction rate of TWSTRS at 1-3 months and 3-6 months after injection, and the probability of reinjection-free living.
The number of subjects who had reinjection within 6 months was significantly lower in group 2 than in group 1 (10 in group 1 vs. 3 in group 2). The reduction rate of TWSTRS after 3-6 months (37.8±15.7% of group 1 vs. 63.3±28.0% of group 2) and the probability of reinjection-free living were significantly higher in group 2 than in group 1.
These findings suggest that 18F-FDG PET/CT study could be useful in management of CD in terms of the identification of dystonic muscles if there is an increase in the 18F-FDG uptake in the cervical muscle of the images.
Citations
Dystonia is a symptom defined by involuntary and irregular contractions of the muscles, which cause movement disorders and postural problems. Deep brain stimulation (DBS) in globus pallidus interna (GPi) is a good option for controlling dystonia. DBS has already been shown to have significant effects on primary dystonia as well as Parkinson's disease. Dystonia is very difficult to manage, as seen in cerebral palsy (CP) mixed with spasticity. As CP patients grow, their musculoskeletal problems may require orthopedic surgery. However, the outcome of orthopedic surgery is not usually suitable due to dystonia. Therefore, we attempted to control dystonia through DBS initially and perform orthopedic surgery to correct musculoskeletal deformities after treatment of dystonia. Herein, we report a case that showed remarkable improvement in terms of the dystonia rating scale and gait pattern after combined therapy of DBS and orthopedic surgery.
Citations