Dystonia Awareness Month
September 2023
Dystonia is a movement disorder characterised by muscle contractions which can cause abnormal movements, twisting and postures of any part of the body. It may also be accompanied by tremor and pain. Dystonia Network of Australia Inc. (DNA) is the peak body in Australia for adults and children with all forms of dystonia Dystonia Network of Australia
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Articles
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Dystonia
What you need to know
BMJ 11 April 2022
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Research
The Pain in Dystonia Scale (PIDS)—Development and validation in Cervical Dystonia
The PIDS is the first specific questionnaire developed to evaluate pain in all patients with adult-onset idiopathic dystonia (AOID), here, demonstrating high-level psychometric properties in people with CD. Future work will validate PIDS in other forms of AOID. Movement disorders 25 May 2023
Increased frequency of self-reported obsessive-compulsive symptoms in patients with functional movement disorders
FMD patients reported higher rates of OCS compared to controls, along with higher rates of non-motor symptoms and lower quality of life. This finding may have clinical implications and raises the possibility of shared risk factors and common pathophysiological mechanisms in FMD and OCD. Movement disorders clinical practice 9 June 2023
Parkinsonism following SARS-CoV-2 infection unmasks a genetic twist
A broad spectrum of neurological sequelae has been associated with SARS-CoV-2 infection. Movement disorders remain uncommon, with higher prevalence of myoclonus, ataxia, and tremor. However, the observation of newly-diagnosed parkinsonism following SARS-CoV-2 infection has stimulated debate whether this corroborates causality versus coincidence. The authors present a case with SARS-CoV-2 infection unmasking parkinsonism in the context of a previously undiagnosed monogenic disorder, which suggests that post-SARS-CoV-2 parkinsonism should prompt clinicians to investigate alternative etiologies. Movement disorders clinical practice 15 May 2023
Transitional care for young people with movement disorders: consensus-based recommendations from the MDS task force on pediatrics
Recommendations on providing transitional care for patients with childhood onset movement disorders are provided. Nevertheless several challenges remain in the implementation of these recommendations, related to health infrastructure and the distribution of health resources, and the availability of knowledgeable and interested practitioners. Research on the influence of transitional care programs on outcomes in childhood onset movement disorders is much needed. Movement disorders clinical practice 20 March 2023
Thyroid disorders and movement disorders—A systematic review
In view of the treatability of movement disorders associated with thyroid disease, accurate diagnosis is important. The pathophysiology remains poorly understood. More detailed case documentation and systematic studies, along with experimental studies are needed. Movement disorders clinical practice 11 January 2023
Development of a patient journey map for people living with cervical dystonia
The authors present the first patient journey map for CD that can be used to guide local service mapping and to compare current provision with what patients say they want and need. Orphanet journal of rare diseases 22 March 2022
Under-recognition of leg dystonia in people with cerebral palsy
Leg dystonia is clinically under-documented in people with CP-associated spasticity, even when these people are evaluated by well-trained clinicians. Longitudinal evaluation and vigilance for leg dystonia is critical to address this diagnostic gap. Annals of the Child Neurology Society 5 April 2023
Multiple motor disorders in cerebral palsy
Multiple motor disorders in children and young people with CP are common and associated with more severe functional impairment. Accurate assessment of motor disorders is essential to guide prognosis and ensure personalized evidence-based interventions. Developmental medicine & child neurology 14 August 2023
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Treatment
A single-center real-life study on the use of medical cannabis in patients with dystonia
A subset of dystonia patients who use medical cannabis under clinical observation reported significant subjective improvement during 30 months of use in average. Further prospective randomized controlled trials are required to examine the effectiveness of cannabis in dystonia. Frontiers in neurology 29 June 2022
Trial of Globus Pallidus focused ultrasound ablation in Parkinson’s Disease
Unilateral pallidal ultrasound ablation resulted in a higher percentage of patients who had improved motor function or reduced dyskinesia than a sham procedure over a period of 3 months but was associated with adverse events. Longer and larger trials are required to determine the effect and safety of this technique in persons with Parkinson’s disease. NEJM 23 February 2023
Assessment of Botulinum Neurotoxin injection for dystonic hand tremor: A randomized clinical trial
In this randomized clinical trial, botulinum neurotoxin injections were superior to placebo in reducing tremor severity in upper-extremity dystonic tremor (DT). An individualized approach to muscle selection and dosing was beneficial without unacceptable adverse effects. JAMA 21 December 2020
Electrophysiological insights into deep brain stimulation of the network disorder dystonia
Deep brain stimulation (DBS), a treatment for modulating the abnormal central neuronal circuitry, has become the standard of care nowadays and is sometimes the only option to reduce symptoms of movement disorders such as dystonia. However, on the one hand, there are still open questions regarding the patho-mechanisms of dystonia and, on the other hand, the mechanisms of DBS on neuronal circuitry. That lack of knowledge limits the therapeutic effect and makes it hard to predict the outcome of DBS for individual dystonia patients. Finding electrophysiological biomarkers seems to be a promising option to enable adapted individualised DBS treatment. However, biomarker search studies cannot be conducted on patients on a large scale and experimental approaches with animal models of dystonia are needed. In this review, physiological findings of deep brain stimulation studies in humans and animal models of dystonia are summarised and the current pathophysiological concepts of dystonia are discussed. European journal of physiology 2 August 2023
Neurosurgical management of non‑spastic movement disorders
Non-spastic movement disorders are common in children and difcult to treat. Most of these patients are referred to neurosurgery for the management of dystonia, with modern neurosurgical management including pallidotomy, rhizotomy, and deep brain stimulation. Historically, pallidotomy has been effective and may still be preferred in subpopulations presenting either in status dystonicus or with high risk for hardware complications. Superiority of DBS over pallidotomy for secondary dystonia has not been determined. Rhizotomy is an underutilized surgical tool and more study characterizing efficacy and risk profile is indicated. Child's nervous system 31 July 2023
Bilateral globus pallidus internus (GPi) deep brain stimulation for cervical dystonia: Effects on motor and non-motor symptoms within 5 years follow
Highlights
Journal of the neurological sciences 15 September 2023
The challenge of choosing the right stimulation target for Dystonic Tremor—A series of instructive cases
The diagnosis of dystonic tremor alone is not a sufficient predictor to establish the differential indication of GPi- or Vim-DBS. Further criteria (eg, proximal-distal distribution of tremor/dystonia) are needed to avoid rescue surgery in the future. On the other hand, the course of our patients encourages rescue surgery in such severely disabled patients if the first target fails. Movement disorders clinical practice 29 July 2023
Long-term efficacy with deep brain stimulation of the globus pallidus internus in cervical dystonia: a retrospective monocentric study
GPI DBS is an effective procedure especially in severely affected patients with a positive 10-year outcome. It should be considered in more complex CD-forms or predominant torticollis. Neurological research and practice 3 October 2022
Video-based long-term follow up of musician’s dystonia in pianists reveals similar improvements following different treatment strategies: a retrospective observational study
This study provides a new perspective on the course of Musician’s Dystonia (MD) in keyboard players in a larger cohort using methods other than self-report. Video rating of scale-playing was shown to be a reliable and useful method to evaluate MD in keyboard players. Average improvement rates were different to previous studies using patient-subjective questionnaires. Treatment options showed different effects in the two rating criteria, with retraining showing the highest improvement rates in single and combined use. Journal of clinical movement disorders 25 January 2022
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