Movement Disorders Clinical Practice最新文献

Background: Essential tremor (ET) and dystonic tremor syndrome (DTS) can be treated using botulinum toxin (BoNT) injections. Previous reviews lacked an assessment of the certainty of evidence and focused solely on clinician-reported outcomes. Additionally, studies have demonstrated interindividual variability in BoNT efficacy.

Objective: The aim of the study was to assess the efficacy and safety of BoNT injections for ET and DTS of the upper limbs, and to identify factors associated with BoNT efficacy.

Methods: We systematically searched Pubmed, Embase, Cochrane Library, and Web of Science databases for studies on BoNT injections in ET and DTS of the upper limbs. The certainty of evidence was rated using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) approach. Outcomes from randomized controlled trials (RCTs) were pooled as standardized mean differences (SMDs).

Results: We identified 5 RCTs, 6 open-label trials, and 6 retrospective cohort studies. Meta-analysis of post-intervention scores showed a moderate effect on patient-reported change (SMD: 0.58 [95% confidence interval [CI]: 0.39, 0.78]), no effect on clinically rated tremor severity (SMD: 1.69 [95% CI: -3.80, 0.42]), and no effect on grip strength (SMD: -0.63 [95% CI: -1.37, 0.10]). In contrast, meta-analysis using change-from-baseline scores showed an improvement of clinically rated tremor severity (SMD: -1.12 [95% CI: -1.70, -0.54]). Certainty of evidence ranged from low to very low. No clear associations between BoNT efficacy and tremor phenotypes or injection strategies were identified.

Conclusions: Patient-tailored BoNT injections may be effective and safe for ET and DTS. More trials are needed to confirm efficacy and safety, identify which tremor phenotypes benefit the most, and optimize injection strategies.

Background: The diagnosis of movement disorders relies on identifying the salient phenomenology during clinical examination. Besides a thorough in-person examination, the clinician may rely on video recording, particularly in cases of transient, fluctuating, or paroxysmal movement disorders. As such, this subspecialty has been profoundly influenced by the advent of and access to film, video, and digital documentation.

Objective: The aim was to review the historical development of video technology in the field of movement disorders, tracing its origins from early observational methods to its modern-day applications in telemedicine, clinical research, and neurology education.

Methods: We conducted interviews with 10 experts in movement disorders, many of whom actively played a role in formalizing movement disorders as a clinical discipline.

Results: Our understanding of movement disorders was greatly advanced by the adoption of film at first and video later. The growing relative ease of access to videos allowed easy clinical use in the clinic and its display during video rounds and conferences. A session at the American Academy of Neurology led by David Marsden and Stanley Fahn played a particularly pivotal role in formalizing phenomenology and establishing video as a tool.

Conclusions: The use of patient recordings paralleled the growth of movement disorders as a field as they played a foundational role in the description of phenomenology, formulation of rating scales, and the education of neurologists. The potential of video recordings continues to be realized, most recently through telemedicine and the adoption of machine learning algorithms in research.

Background: Early identification of pathological α-synuclein deposition (αSynD) may improve understanding of Lewy body disorder (LBD) progression and enable timely disease-modifying treatments.

Objectives: We investigated αSynD using a seed amplification assay and assessed prodromal LBD symptoms in individuals with idiopathic olfactory dysfunction (iOD).

Methods: In this cross-sectional, case-control study, we included iOD participants and normosmic healthy controls (HC) aged 55 to 75 years without diagnoses of dementia with Lewy bodies, Parkinson's disease (PD), or other major neurological disorders. iOD was defined as hyposmia without any known causes. The primary outcome was αSynD detection in skin and olfactory mucosa. Secondary outcomes included prodromal LBD symptoms: cognitive dysfunction, rapid eye movement sleep behavior disorder (RBD), motor and nonmotor symptoms (Movement Disorders Society-Unified Parkinson's Disease Rating Scale [MDS-UPDRS] Parts I-III), and prodromal PD risk.

Results: We recruited 44 iOD participants (mean age 65) and 50 HCs (mean age 67). Eighteen iOD participants (41%) were αSynD positive in skin and/or olfactory mucosa compared to 1 HC (2%, P < 0.0001). iOD participants had higher rates of cognitive dysfunction (48% vs. 24%, P = 0.02), possible RBD (41% vs. 16%, P = 0.01), and elevated MDS-UPDRS I-III (median [interquartile range]: 15 [7-22] vs. 5.5 [3-11], P < 0.0001). Dysautonomia symptoms did not differ significantly. In the iOD group, 45% met probable/possible prodromal PD criteria versus 1 control (P < 0.0001). However, αSynD-αSynD-negative iOD participants had a nonsignificantly higher prodromal PD risk compared to αSynD-positive individuals.

Conclusions: iOD exhibits high αSynD prevalence and prodromal LBD symptoms, supporting its role as an early LBD marker and potential model for early intervention and mechanistic studies.

Background: There is limited data regarding the role of pallidothalamic tract (PTT) lesioning after failure of pallidotomy.

Objectives: To report the role of unilateral PTT lesioning in refractory generalized dystonia following bilateral pallidotomy and hemi-dystonia following unilateral pallidotomy.

Methods: A single-center retrospective case series.

Results: Five patients underwent unilateral PTT lesioning after the failure of pallidotomy to control dystonia, including status dystonicus in one case. The interval between pallidotomy and PTT lesioning ranged from 2 to 90 months. Pre-PTT surgery, BFMDRS-M scores ranged from 20 to 87, and BFMDRS-D scores ranged from 8 to 23. Immediately post-PTT surgery, improvement was observed in four patients, with objective improvements ranging between 57.5 and 93.3% in the BFMDRS-M score. Two patients developed adverse effects: one experienced a mild worsening of post-pallidotomy dysarthria, and another developed impaired hand dexterity.

Conclusions: Our initial experience suggests that unilateral PTT lesioning holds promise as an option for lesional surgery following pallidotomy, in refractory dystonia.