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Dystonia

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161. Task-specificity in focal dystonia is shaped by aberrant diversity of a functional network kernel. (PubMed)

Task-specificity in focal dystonia is shaped by aberrant diversity of a functional network kernel. Task-specific focal dystonia selectively affects the motor control during skilled and highly learned behaviors. Recent data suggest the role of neural network abnormalities in the development of the pathophysiological dystonic cascade.We used resting-state functional MRI and analytic techniques rooted in network science and graph theory to examine the formation of abnormal subnetwork of highly (...) influential brain regions, the functional network kernel, and its influence on aberrant dystonic connectivity specific to affected body region and skilled motor behavior.We found abnormal embedding of sensorimotor cortex and prefrontal thalamus in dystonic network kernel as a hallmark of task-specific focal dystonia. Dependent on the affected body region, aberrant functional specialization of the network kernel included regions of motor control management in focal hand dystonia (writer's cramp, musician's

2018 Movement Disorders

162. Pallidal beta bursts in Parkinson's disease and dystonia. (PubMed)

Pallidal beta bursts in Parkinson's disease and dystonia. Exaggerated beta power has been discussed as a disease-specific biomarker for Parkinson's disease (PD) and has recently been suggested to rely on prolonged bursts of subthalamic beta synchronization.In this study, we test whether prolonged bursts are disease specific for beta activity in PD by comparison to oscillatory activity in dystonia.Pallidal local field potentials were recorded from 5 PD patients ON and OFF dopaminergic medication (...) and 5 dystonia patients. Synchronization of beta and low-frequency oscillations in bursts was compared between groups with respect to their duration, amplitude, and rate.Pallidal beta bursts were longer in PD-OFF than PD-ON or dystonia (P < .05). PD-ON and dystonia displayed similar beta burst dynamics. Low-frequency burst features showed no differences across groups.Prolonged burst duration appears as a disease-specific feature for beta activity in PD across the basal ganglia. With dopaminergic

2018 Movement Disorders

163. A single case of MRI-guided focused ultrasound ventro-oral thalamotomy for musician's dystonia. (PubMed)

A single case of MRI-guided focused ultrasound ventro-oral thalamotomy for musician's dystonia. Musician's dystonia (MD) is a type of focal hand dystonia that develops only while playing musical instruments and interferes with skilled and fine movements. Lesioning of the ventro-oral (Vo) nucleus of the thalamus (Vo-thalamotomy) using radiofrequency can cause dramatic improvement in MD symptoms. Focused ultrasound (FUS) can make intracranial focal lesions without an incision. The authors used

2018 Journal of Neurosurgery

164. Delineating cerebellar mechanisms in DYT11 myoclonus-dystonia. (PubMed)

Delineating cerebellar mechanisms in DYT11 myoclonus-dystonia. Recent research has highlighted the role of the cerebellum in the pathophysiology of myoclonus-dystonia syndrome as a result of mutations in the ɛ-sarcoglycan gene (DYT11). Specifically, a cerebellar-dependent saccadic adaptation task is dramatically impaired in this patient group.The objective of this study was to investigate whether saccadic deficits coexist with impairments of limb adaptation to provide a potential mechanism

2018 Movement Disorders

165. High frequency somatosensory stimulation in dystonia: Evidence fordefective inhibitory plasticity. (PubMed)

High frequency somatosensory stimulation in dystonia: Evidence fordefective inhibitory plasticity. Apart from motor symptoms, multiple deficits of sensory processing have been demonstrated in dystonia. The most consistent behavioural measure of this is abnormal somatosensory temporal discrimination threshold, which has recently been associated with physiological measures of reduced inhibition within the primary somatosensory area. High-frequency repetitive sensory stimulation is a patterned (...) dystonia.Somatosensory temporal discrimination and a number of electrophysiological measures of sensorimotor inhibition and facilitation were measured before and after 45 minutes of high-frequency repetitive sensory stimulation.As compared with a group of healthy volunteers of similar age, in whom high-frequency repetitive sensory stimulation increased inhibition and shortened somatosensory temporal discrimination, patients with cervical dystonia showed a consistent, paradoxical response: they had reduced

2018 Movement Disorders

166. Deep brain stimulation for pediatric dystonia: a meta-analysis with individual participant data. (PubMed)

Deep brain stimulation for pediatric dystonia: a meta-analysis with individual participant data. We performed a meta-analysis with individual participant data of deep brain stimulation (DBS) for dystonia in children and young people.Three databases (PubMed, Embase, and Web of Science) were queried from January 1999 to August 2017 with no language restrictions to identify case studies and cohort studies reporting on pediatric patients (age ≤21y) with dystonia. The primary outcomes were changes (...) in Burke-Fahn-Marsden (BFM) or Barry-Albright Dystonia Scale scores. A mixed-effects regression was used to identify associations between clinical covariates and outcomes.Of 2509 citations reviewed, 72 articles (321 children) were eligible. At last follow-up (median 12mo, 25th centile=9.0; 75th centile=32.2), 277 (86.3%) patients showed improvement in dystonia, while 66.1 percent showed clinically significant (>20%) BFM Dystonia Rating Scale-motor improvement. On multivariable hierarchical regression

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2018 Developmental Medicine and Child Neurology

167. Long-term GPi-DBS improves motor features in myoclonus-dystonia and enhances social adjustment. (PubMed)

Long-term GPi-DBS improves motor features in myoclonus-dystonia and enhances social adjustment. Good short-term results of pallidal deep brain stimulation have been reported in myoclonus-dystonia. Efficacy and safety in the long term remain to be established. In addition, the actual impact of DBS treatment on social inclusion is unknown. The objective of this study was to assess the long-term clinical outcome, quality of life, and social adjustment of GPi-DBS in patients with ε-sarcoglycan (...) (DYT11)-positive myoclonus-dystonia.Consecutive myoclonus-dystonia patients with ε-sarcoglycan mutations who underwent GPi-DBS were evaluated at least 5 years postoperatively. Motor symptoms were assessed using the Burke-Fahn-Marsden Dystonia Rating Scale including the Disability Scale, a composite score combining the rest and action parts of the Unified Myoclonus Rating Scale and modified Abnormal Involuntary Movement Scale. Standardized video-protocols were assessed by a blinded and external

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2018 Movement Disorders

168. Pain processing in functional and idiopathic dystonia: An exploratory study. (PubMed)

Pain processing in functional and idiopathic dystonia: An exploratory study. Pain is often experienced by patients with functional dystonia and idiopathic cervical dystonia and is likely to be determined by different neural mechanisms.In this exploratory study, we tested the sensory-discriminative and cognitive-emotional component of pain in patients with functional and idiopathic dystonia.Ten patients with idiopathic cervical dystonia, 12 patients with functional dystonia, and 16 age- and sex (...) thresholds assessed in hands and feet. Pain tolerance was significantly increased in all body regions only in functional dystonia. Patients with continuous functional dystonia had higher pain tolerance compared to subjects with paroxysmal functional dystonia and idiopathic cervical dystonia. There was no correlation between pain tolerance and pain scores, depression, anxiety, disease duration, and motor disability in both groups.Patients with functional dystonia have a dissociation between the sensory

2018 Movement Disorders

169. Deep brain stimulation treated dystonia-trajectory via status dystonicus. (PubMed)

Deep brain stimulation treated dystonia-trajectory via status dystonicus. Status dystonicus (SD) is a life-threatening condition.In a dystonia cohort who developed status dystonicus, we analyzed demographics, background dystonia phenomenology and complexity, trajectory previous to-, via status dystonicus episodes, and evolution following them.Over 20 years, 40 of 328 dystonia patients who were receiving DBS developed 58 status dystonicus episodes. Dystonia was of pediatric onset (95

2018 Movement Disorders

170. Safety and long-term efficacy of ventro-oral thalamotomy for focal hand dystonia: A retrospective study of 171 patients. (PubMed)

Safety and long-term efficacy of ventro-oral thalamotomy for focal hand dystonia: A retrospective study of 171 patients. To report the safety and long-term efficacy of ventro-oral thalamotomy for 171 consecutive patients with task-specific focal hand dystonia.Between October 2003 and February 2017, 171 consecutive patients with task-specific focal hand dystonia underwent unilateral ventro-oral thalamotomy. Etiologies included writer's cramps (n = 92), musician's dystonias (n = 58), and other (...) occupational task-related dystonias (n = 21). The task-specific focal hand dystonia scale was used to evaluate patients' neurologic conditions (range 1-5, high score indicated a better condition). The scores before surgery; at 1 week, 3 months, and 12 months postoperatively; and the last available follow-up period were determined. Postoperative complications and postoperative recurrence were also evaluated.The scores before surgery; at 1 week (1.72 ± 0.57, 4.33 ± 0.85 [p < 0.001]), 3 months (4.30 ± 1.06 [p

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2018 Neurology

171. Oromandibular Dystonia Related to Sertraline Treatment in a Child. (PubMed)

Oromandibular Dystonia Related to Sertraline Treatment in a Child. In children, similar to adults, the agents that cause movement disorders are most commonly dopaminergic and antidopaminergic drugs. However, it is also known that selective serotonin reuptake inhibitors (SSRIs), which are frequently prescribed in child psychiatry practice, may rarely lead to tremor, akathisia, dystonia, dyskinesia, and parkinsonism. In this study, we report a rare side effect of SSRIs, namely oromandibular (...) dystonia related to sertraline, in a pediatric patient who was diagnosed with posttraumatic stress disorder.

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2018 Journal of Child and Adolescent Psychopharmacology

172. Randomised controlled trial of escitalopram for cervical dystonia with dystonic jerks/tremor. (PubMed)

Randomised controlled trial of escitalopram for cervical dystonia with dystonic jerks/tremor. Trials for additional or alternative treatments for cervical dystonia (CD) are scarce since the introduction of botulinum neurotoxin (BoNT). We performed the first trial to investigate whether dystonic jerks/tremor in patients with CD respond to the selective serotonin reuptake inhibitor (SSRI) escitalopram.In a randomised, double-blind, crossover trial, patients with CD received escitalopram (...) and placebo for 6 weeks. Treatment with BoNT was continued, and scores on rating scales regarding dystonia, psychiatric symptoms and quality of life (QoL) were compared. Primary endpoint was the proportion of patients that improved at least one point on the Clinical Global Impression Scale for jerks/tremor scored by independent physicians with experience in movement disorders.Fifty-threepatients were included. In the escitalopram period, 14/49 patients (29%) improved on severity of jerks/tremor versus 11

2018 Journal of neurology, neurosurgery, and psychiatry

173. Randomised controlled trial of escitalopram for cervical dystonia with dystonic jerks/tremor. (PubMed)

Randomised controlled trial of escitalopram for cervical dystonia with dystonic jerks/tremor. Trials for additional or alternative treatments for cervical dystonia (CD) are scarce since the introduction of botulinum neurotoxin (BoNT). We performed the first trial to investigate whether dystonic jerks/tremor in patients with CD respond to the selective serotonin reuptake inhibitor (SSRI) escitalopram.In a randomised, double-blind, crossover trial, patients with CD received escitalopram (...) and placebo for 6 weeks. Treatment with BoNT was continued, and scores on rating scales regarding dystonia, psychiatric symptoms and quality of life (QoL) were compared. Primary endpoint was the proportion of patients that improved at least one point on the Clinical Global Impression Scale for jerks/tremor scored by independent physicians with experience in movement disorders.Fifty-threepatients were included. In the escitalopram period, 14/49 patients (29%) improved on severity of jerks/tremor versus 11

2018 Neurosurgery and Psychiatry

174. MINGO Supplemental Trial in X-linked Dystonia-Parkinsonism Patients

MINGO Supplemental Trial in X-linked Dystonia-Parkinsonism Patients MINGO Supplemental Trial in X-linked Dystonia-Parkinsonism Patients - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. MINGO Supplemental (...) Trial in X-linked Dystonia-Parkinsonism Patients (MINGO) The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our for details. ClinicalTrials.gov Identifier: NCT03019458 Recruitment Status : Completed First Posted : January 12, 2017 Last Update Posted : June 27, 2017 Sponsor: Sunshine Care Foundation Collaborators: Jose R. Reyes Memorial Medical Center

2017 Clinical Trials

175. Brain Networks in Dystonia

Brain Networks in Dystonia Brain Networks in Dystonia - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. Brain Networks in Dystonia The safety and scientific validity of this study is the responsibility (...) Study Details Study Description Go to Brief Summary: Task-specific focal dystonias are characterized by selective activation of dystonic movements during performance of highly learned motor tasks, such as writing or playing a musical instrument. To date, there is only limited knowledge about the distinct neural abnormalities that lead to the development of task-specificity in focal dystonias, which affect similar muscle groups but result in different clinical manifestations, such as writer's cramp

2017 Clinical Trials

176. Efficacy and Safety of DBS in Patients With Primary Dystonia

Efficacy and Safety of DBS in Patients With Primary Dystonia Efficacy and Safety of DBS in Patients With Primary Dystonia - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. Efficacy and Safety of DBS (...) in Patients With Primary Dystonia The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. of clinical studies and talk to your health care provider before participating. Read our for details. ClinicalTrials.gov Identifier: NCT03017586 Recruitment Status : Recruiting First Posted : January 11, 2017 Last Update Posted : March 15, 2018 See Sponsor: Beijing Pins Medical Co

2017 Clinical Trials

177. Outcome of Botulinum Toxin Treatment for Oromandibular Dystonia

Outcome of Botulinum Toxin Treatment for Oromandibular Dystonia Outcome of Botulinum Toxin Treatment for Oromandibular Dystonia - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. Outcome of Botulinum Toxin (...) Treatment for Oromandibular Dystonia (rétroBODOM) The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our for details. ClinicalTrials.gov Identifier: NCT03363113 Recruitment Status : Completed First Posted : December 6, 2017 Last Update Posted : December 6, 2017 Sponsor: Fondation Ophtalmologique Adolphe de Rothschild Information provided by (Responsible

2017 Clinical Trials

178. Study of Speech Disorders , Voice and Swallowing in Primary Dystonia Oromandibular

Study of Speech Disorders , Voice and Swallowing in Primary Dystonia Oromandibular Study of Speech Disorders , Voice and Swallowing in Primary Dystonia Oromandibular - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before (...) adding more. Study of Speech Disorders , Voice and Swallowing in Primary Dystonia Oromandibular (DOM) The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our for details. ClinicalTrials.gov Identifier: NCT03380676 Recruitment Status : Completed First Posted : December 21, 2017 Last Update Posted : December 21, 2017 Sponsor: University Hospital, Lille

2017 Clinical Trials

179. Task-dependent Operation of a Mechanism Intracortical Inhibition in Dystonia

Task-dependent Operation of a Mechanism Intracortical Inhibition in Dystonia Task-dependent Operation of a Mechanism Intracortical Inhibition in Dystonia - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. Task (...) -dependent Operation of a Mechanism Intracortical Inhibition in Dystonia (LICIdystonie) The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our for details. ClinicalTrials.gov Identifier: NCT03381456 Recruitment Status : Completed First Posted : December 22, 2017 Last Update Posted : December 22, 2017 Sponsor: University Hospital, Lille Information provided

2017 Clinical Trials

180. It's not just the basal ganglia: Cerebellum as a target for dystonia therapeutics. (PubMed)

It's not just the basal ganglia: Cerebellum as a target for dystonia therapeutics. Dystonia is a common movement disorder that devastates the lives of many patients, but the etiology of this disorder remains poorly understood. Dystonia has traditionally been considered a disorder of the basal ganglia. However, growing evidence suggests that the cerebellum may be involved in certain types of dystonia, raising several questions. Can different types of dystonia be classified as either a basal (...) ganglia disorder or a cerebellar disorder? Is dystonia a network disorder that involves the cerebellum and basal ganglia? If dystonia is a network disorder, how can we target treatments to alleviate symptoms in patients? A recent study by Chen et al, using the pharmacological mouse model of rapid-onset dystonia parkinsonism, has provided some insight into these important questions. They showed that the cerebellum can directly modulate basal ganglia activity through a short latency cerebello-thalamo

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2017 Movement Disorders

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