Movement Disorders Clinical Practice最新文献

Comprehensive management of Parkinson's disease (PD) symptoms should include addressing both motor and nonmotor symptoms (NMS). Gastrointestinal (GI) manifestations are the most common NMS in PD and can lead to significant impairment in quality of life (QoL), impaired medication absorption, and response to treatment. Therefore, the approach to managing GI symptoms in PD ideally should emphasize interdisciplinary collaboration, bringing together experts across several disciplines to provide cohesive and comprehensive treatment. Our teams at Oregon Health & Science University (OHSU) and University of Florida (UF) have developed a novel approach to incorporating gastroenterology and allied services to the delivery of neurologic care in our "PDGI clinic." In this review, we provide recommendations on how to establish an interdisciplinary clinic for the care of patients with PD (PwPD) based on our experience, including team building, operations, logistics, and curriculum building for education. Despite the emphasis in this review on management of GI symptoms, this interdisciplinary approach could be adapted for expert care of any NMS.

Background: Dystonia in pediatric patients often coexists with other movement disorders and neurodevelopmental issues. Current rating scales for evaluating pediatric deep brain stimulation (DBS) candidates are not universally applicable and often require a non-validated combination of the existing scales.

Objectives: To evaluate dystonia scales used in pediatric patients, focusing on their application in DBS.

Methods: A scoping review identified the most widely used scales in pediatric DBS candidates.

Results: The Fahn-Marsden Dystonia Rating Scale was the most frequently chosen (94.3%), often as a sole scale (78.2%). The Barry Albright Dystonia Scale followed (12.9%). The Unified Dystonia Rating Scale, Global Dystonia Rating Scale, Dyskinesia Impairment Scale and Movement Disorders in Childhood Rating Scale, were used less frequently (5.6%, 4%, 2.4% and 0.8%, respectively). Only 12% of studies included a quality-of-life scale.

Conclusions: There is a need for a new pediatric dystonia scale for evaluating DBS candidates considering developmental and functional challenges.

Background: There are limited data on the long-term survival of patients with Parkinson's disease (PD) undergoing subthalamic nucleus deep brain stimulation (STN-DBS).

Objectives: The aim of the study was to estimate the long-term survival and its predictors in patients who underwent STN-DBS.

Methods: The baseline demographic and clinical features of patients who underwent STN-DBS for PD at our center and had at least 5 years of follow-up were analyzed. Survival status, the date of demise, and the cause of death were obtained by telephonic interview with family members.

Results: A total of 186 patients who underwent STN-DBS between 1999 and 2018 (age at surgery: 56.7 ± 9.3 years) and were contactable in 2024 were included; 85 had died by 2024. Progressive worsening of PD, intercurrent infections, and cardiovascular diseases were the leading causes of death. The median survival after surgery was 13.1 years (interquartile range [IQR]: 8.9-17.1). Baseline postural instability and gait dysfunction subtype (hazard ratio [HR]: 2.3; 95% confidence interval [CI]:1.4-3.8, P = 0.001), higher levodopa-equivalent daily dosage (HR: 2.5; 95%CI: 1.4-4.5; P = 0.002), and higher Unified Parkinson's Disease Rating Scale Part I (UPDRS I) score in the ON state (HR: 2.8; 95% CI: 1.8-4.9; P < 0.001) were independent predictors of shorter survival, whereas higher levodopa response was an independent predictor of longer survival (HR: 0.19; 95% CI: 0.07-0.48; P = 0.001).

Conclusions: STN-DBS offers a median survival of around 13 years; predominant axial features, neurocognitive changes, poor levodopa response, and higher medication requirement at baseline portend shorter survival. These findings could influence surgical candidate selection and long-term care strategies in resource-limited settings.

Background: Quantitative susceptibility mapping (QSM) is a promising neuroimaging biomarker for detecting abnormal iron deposition in progressive supranuclear palsy (PSP); however, the QSM profile and its accuracy in autopsy-confirmed PSP remain unexplored.

Objective: The aim of the study was to elucidate and validate the QSM profiles in autopsy-confirmed PSP.

Methods: Eight patients with a clinically diagnosed PSP syndrome (PSPS) underwent antemortem 3 T magnetic resonance imaging (MRI) scanning and subsequent pathological diagnosis, confirming 6 PSP pathology and 2 multiple system atrophy (MSA) pathology cases. QSM analysis and Prussian blue staining were performed across subcortical regions.

Results: PSP cases showed higher susceptibility than age-matched controls in the pallidum, subthalamic nucleus, substantia nigra, and cerebellar dentate (false discovery rate [FDR]-corrected P < 0.05). PSP had higher susceptibility in the subthalamic nucleus and lower susceptibility in the putamen than the clinically atypical MSA (P = 0.08). Subcortical susceptibilities correlated with Prussian blue-stained areas (r = 0.63, P < 0.001).

Conclusions: QSM could be a reliable measure to evaluate abnormal iron accumulation patterns in PSP.

Background: Several movement disorders (MD) have been reported to occur in neuromyelitis optica spectrum disorder (NMOSD). No extensive review has addressed the whole spectrum of MD in NMOSD.

Objective: This article aims to review MD in NMOSD, describing its prevalence and features.

Methods: A systematic review and prevalence meta-analysis were conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) reporting guidelines. We included articles on NMOSD patients with MD as defined by the 2015 international consensus criteria. Meta-analysis was considered feasible if the prevalence of certain MDs was evaluated for at least 4 studies with more than 5 patients.

Results: Ninety-six articles were selected from an initial pool of 5441, involving 1751 patients, of whom 487 had MD. The prevalence of anti-aquaporin-4 antibodies (AQP4-IgG) was 79.9% in general and 78.7% in NMOSD-MD patients. Tonic spasms/paroxysmal dystonia and ataxia were the most prevalent MD in NMOSD, with 39% and 26% prevalence, respectively. Tremor, parkinsonism, myoclonus, chorea, and other hyperkinetic disorders were more rarely reported.

Conclusions: A wide range of MDs in NMOSD were found, each with distinct features and frequency in literature. This knowledge might help to identify patients with NMOSD, which presents MD as a clinical feature and improves outcomes.

Background: Dysphagia remains a major clinical concern in multiple system atrophy (MSA), and so far, lacks relevant treatment options.

Objective: To systematically assess levodopa-responsiveness of dysphagia in MSA.

Methods: 19 MSA-patients underwent endoscopic swallowing evaluation in Off- and On-state, following an adapted FEES-levodopa-test-protocol. Findings were compared between states and correlated to disease-specific and demographic factors.

Results: All 19 MSA-patients 70 [66-72] years, 15/4 MSA-P/ C, 10 women exhibited dysphagia. Levodopa-responsiveness of motor-impairment was poor (UPDRS-3 Off 46 [34-52] vs On 43 [40-48], P = 0.31). Dysphagia severity varied greatly. Sum-scores ranged between 9-21 in Off and 6-20 in On. MSA-P-patients were more likely to have higher sum-scores (rpb 0.41, P = 0.01). Sum-scores otherwise were independent of disease-specific and demographic factors. 3/19 MSA-patients showed marked score-improvement by ≥30% through reduced leaking and aspiration but remained unchanged for the majority. Scores worsened in 2/19 patients. In the group analysis, the median dysphagia-score remained unchanged (2 [1, 2], P = 0.28). The occurrence of leaking, pharyngeal residue, penetration, and aspiration was not changed (P = 0.13-0.19). Severity of leaking was reduced (3 [2-4] vs. 0[0.5-1], P < 0.0001), while severity of pharyngeal residue, penetration, and aspiration remained unchanged (P = 0.29-0.53). Liquids were more often associated with leaking (P < 0.05) and aspiration (P < 0.005). All patients exhibited laryngeal movement disorders, with irregular arytenoid cartilage movements (iACM) being observed in all patients, regardless of levodopa-state.

Conclusion: The levodopa-response in MSA-related dysphagia varies greatly. Some patients might improve markedly, necessitating individual assessments to tailor patient-specific therapies. Non-pharmacological measures should be investigated to address dysphagia in MSA. IACM again proved to be highly prevalent in MSA.

Background: Only little is known about the prognosis of functional tic-like behaviors (FTLB), especially in the subgroup of patients with mass social media-induced illness (MSMI-FTLB).

Objectives: To provide data of long-term follow-up (FU) of a carefully characterized group of patients with MSMI-FTLB to identify influencing factors such as treatment, exposure to social media, and comorbidities.

Methods: At FU (mean = 26.3 (range = 11-51) months after baseline), 30 patients (mean age = 22.5 years, n = 18 (60%) female) underwent an extensive semi-structured interview.

Results: The majority of patients reported symptom improvement (n = 19, 63%) or complete remission (n = 7, 23%) of MSMI-FTLB, while only a minority reported no change (n = 4, 13%), and none worsening of symptoms. Factors associated with better prognosis were younger age, male sex, diagnosis early after disease onset, less lifetime psychiatric comorbidities, absence of depression, and discontinuation of secondary gain, while diagnostic acceptance or changes in daily social media time had no influence on prognosis. Of n = 26 patients (87%) receiving psychotherapy, n = 15 (58%) rated it as "helpful," but neither the presence of therapy nor the type of therapeutic approach was associated with significant differences in symptom improvement. At FU, n = 13 (43%) patients received medication, which most (n = 11, 85%) felt ineffective. N = 11 patients (36.7%) indicated that simply knowing the correct diagnosis was helpful in terms of symptom improvement.

Conclusions: In line with previous reports in FTLB, patients with MSMI-FTLB also have a good prognosis overall, especially in the case of younger age, male sex, early diagnosis, discontinuation of secondary gain, less psychiatric comorbidities, and absence of depression.