Mauro César Quintão E Silva Cunningham, Sarah Teixeira Camargos, Vinícius Ribeiro Jeunon, Natalia Pessoa Rocha, Antônio Lúcio Teixeira, Thiago de Oliveira Maciel, Elisa de Paula França Resende, Francisco Eduardo Costa Cardoso, Paulo Caramelli, Leonardo Cruz de Souza
{"title":"Does Midbrain Atrophy Distinguish Progressive Supranuclear Palsy from Frontotemporal Dementia?","authors":"Mauro César Quintão E Silva Cunningham, Sarah Teixeira Camargos, Vinícius Ribeiro Jeunon, Natalia Pessoa Rocha, Antônio Lúcio Teixeira, Thiago de Oliveira Maciel, Elisa de Paula França Resende, Francisco Eduardo Costa Cardoso, Paulo Caramelli, Leonardo Cruz de Souza","doi":"10.1002/mdc3.70058","DOIUrl":"10.1002/mdc3.70058","url":null,"abstract":"<p><strong>Background: </strong>The diagnostic value of midbrain atrophy for distinguishing behavioral variant frontotemporal dementia (bvFTD) from progressive supranuclear palsy (PSP) is unclear.</p><p><strong>Objective: </strong>To investigate whether measures of midbrain atrophy differentiate PSP from bvFTD.</p><p><strong>Methods: </strong>We included four groups: healthy controls (n = 19), PSP-Richardson syndrome (n = 20), bvFTD (n = 19) and Parkinson's disease (PD; n = 12). The following quantitative and qualitative measures were calculated: Hummingbird sign rating scale [HBS-RS], global midbrain atrophy [GMA], midbrain area, midbrain/pons ratio, the Magnetic Resonance Parkinsonism Index (MRPI), the MRPI 2.0 and brainstem volume.</p><p><strong>Results: </strong>Compared to controls, PSP and bvFTD had lower values of midbrain area, HBS-RS and GMA, and higher MRPI and MRPI 2.0. HBS-RS, GMA, midbrain/pons ratio, midbrain area, MRPI, MRPI 2.0 and brainstem volume distinguished PSP from bvFTD with 73%, 67%, 75%, 83%, 71%, 69% and 82% accuracies, respectively.</p><p><strong>Conclusions: </strong>Both quantitative and qualitative measures of midbrain atrophy provided modest accuracy in distinguishing PSP from bvFTD.</p>","PeriodicalId":19029,"journal":{"name":"Movement Disorders Clinical Practice","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-04-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143764522","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Abigail Braun, Dion Basson, Shahida Moosa, Jonathan Carr, Soraya Bardien, Riaan van Coller
{"title":"Deep Brain Stimulation Improves Symptoms in an Individual with Alpha-Synuclein-Gene-Associated Parkinson's Disease.","authors":"Abigail Braun, Dion Basson, Shahida Moosa, Jonathan Carr, Soraya Bardien, Riaan van Coller","doi":"10.1002/mdc3.70057","DOIUrl":"https://doi.org/10.1002/mdc3.70057","url":null,"abstract":"","PeriodicalId":19029,"journal":{"name":"Movement Disorders Clinical Practice","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143743278","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Case Report of Myoclonus-Ataxia Syndrome in an Indian Patient Due to SCNA1 Gene Mutation.","authors":"Anjali Chouksey","doi":"10.1002/mdc3.70067","DOIUrl":"https://doi.org/10.1002/mdc3.70067","url":null,"abstract":"","PeriodicalId":19029,"journal":{"name":"Movement Disorders Clinical Practice","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143743183","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Daniel G Di Luca, Mwiza Ushe, Scott A Norris, Isabel Alfradique-Dunham, Matthew Glasser, Arash Nazeri, Paul Kotzbauer, Jon T Willie
{"title":"Long-Term Outcome of Unilateral Magnetic Resonance Imaging-Guided Focused Ultrasound for Fragile X Tremor Ataxia Syndrome.","authors":"Daniel G Di Luca, Mwiza Ushe, Scott A Norris, Isabel Alfradique-Dunham, Matthew Glasser, Arash Nazeri, Paul Kotzbauer, Jon T Willie","doi":"10.1002/mdc3.70068","DOIUrl":"https://doi.org/10.1002/mdc3.70068","url":null,"abstract":"","PeriodicalId":19029,"journal":{"name":"Movement Disorders Clinical Practice","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143743284","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Daniel Weiss, Wolfgang H Jost, József Attila Szász, Zvezdan Pirtošek, Ivan Milanov, Volker Tomantschger, Norbert Kovács, Harry Staines, Bharat Amlani, Niall Smith, Teus van Laar
{"title":"Levodopa-Entacapone-Carbidopa Intrajejunal Infusion in Advanced Parkinson's Disease - Interim Analysis of the ELEGANCE Study.","authors":"Daniel Weiss, Wolfgang H Jost, József Attila Szász, Zvezdan Pirtošek, Ivan Milanov, Volker Tomantschger, Norbert Kovács, Harry Staines, Bharat Amlani, Niall Smith, Teus van Laar","doi":"10.1002/mdc3.70046","DOIUrl":"https://doi.org/10.1002/mdc3.70046","url":null,"abstract":"<p><strong>Background: </strong>Levodopa-entacapone-carbidopa intestinal gel (LECIG) was introduced in 2018 as a device-aided therapy for advanced Parkinson's disease (PD).</p><p><strong>Objectives: </strong>The ELEGANCE study (NCT05043103) is gathering real-world data on long-term efficacy, safety and patient-reported outcomes with LECIG from 13 European countries. This article reports data from the planned interim analysis.</p><p><strong>Methods: </strong>The study enrolled patients prescribed LECIG as part of routine clinical care. We evaluated patients at V1 before starting LECIG treatment (in seven patients V1 data were obtained retrospectively), and thereafter at V2 (3-6 months) or V3 (6-12 months).</p><p><strong>Results: </strong>This analysis includes 167 patients from 37 centers. Three patients from this analysis set (1.8%) discontinued the study. Mean (±SD) daily OFF-time hours (MDS-UPDRS IV item 4.3) were substantially reduced by 3.47 ± 3.56 h at V2 (baseline: 5.15 ± 3.05; P < 0.0001). Similarly, MDS-UPDRS part IV total scores were reduced by 4.24 ± 4.08 at V2 (baseline: 10.77 ± 3.83); (P = 0.0001) and MDS-UPDRS part II scores by 3.63 ± 7.76 at V2 (baseline: 20.65 ± 8.17; P = 0.0004). PDSS-2 total scores were sustainably improved (reduction of 7.38 ± 10.72 at V2 [baseline: 25.21 ± 10.62]; P < 0.0001), as was the PDQ-8 summary index score indicating an improvement in quality of life (QoL) (reduction of 13.3 ± 19.05 at V2 [baseline: 46.34 ± 20.09]; P < 0.0001). For all parameters improvements were maintained at V3. Patient-reported satisfaction with the LECIG pump was high. Most adverse events were related to the procedure or the device.</p><p><strong>Conclusions: </strong>Routine use of LECIG for up to 12 months provided sustained control of motor symptoms, and was well tolerated with a positive impact on QoL and high patient satisfaction.</p>","PeriodicalId":19029,"journal":{"name":"Movement Disorders Clinical Practice","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143701051","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Correlations between Dysphagia Severity Scale Scores and Clinical Indices in Individuals with Multiple System Atrophy.","authors":"Ryunosuke Nagao, Yasuaki Mizutani, Kazuya Kawabata, Junichiro Yoshimoto, Yoko Inamoto, Seiko Shibata, Mizuki Ito, Yohei Otaka, Hirohisa Watanabe","doi":"10.1002/mdc3.70055","DOIUrl":"https://doi.org/10.1002/mdc3.70055","url":null,"abstract":"<p><strong>Background: </strong>Dysphagia significantly impacts prognosis in individuals with multiple system atrophy (MSA). While video-based assessments are practical, their limited availability highlights the need for a simple tool such as the Dysphagia Severity Scale (DSS) in clinical practice.</p><p><strong>Objectives: </strong>To evaluate the utility of the DSS in assessing dysphagia in MSA patients and its correlations with clinical indices.</p><p><strong>Methods: </strong>We examined 43 MSA patients using the DSS and other clinical measures, including the Unified MSA Rating Scale (UMSARS) and cerebrospinal fluid 5-hydroxyindoleacetic acid levels. As a follow-up, 11 of 43 patients underwent a secondary DSS evaluation. Spearman's correlation and linear mixed models were used to analyze cross-sectional and longitudinal relationships.</p><p><strong>Results: </strong>DSS scores were significantly correlated with UMSARS Parts 1, 2, and 4, as well as disease duration and blood pressure changes. This indicates that the DSS is sensitive to MSA-related motor and autonomic dysfunctions, and that the DSS could provide a more detailed assessment of swallowing function compared with the UMSARS Part 1 swallowing subscore. Additionally, DSS score was correlated with cerebrospinal fluid 5-hydroxyindoleacetic acid levels. Our longitudinal analysis further supported the role of DSS score as a reliable marker of dysphagia progression over time.</p><p><strong>Conclusions: </strong>The DSS is a sensitive and practical tool for evaluating dysphagia. Thus, combining the DSS and UMSARS could improve dysphagia monitoring in individuals with MSA. Our data support the use of the DSS as a valuable clinical and research tool in MSA management.</p>","PeriodicalId":19029,"journal":{"name":"Movement Disorders Clinical Practice","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143701049","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Jack Anderson, Nicholas K H Chiu, Jonathon E Pye, Maria Comas-Soberats, Aaron Lam, Ronald R Grunstein, Simon J G Lewis, Elie Matar
{"title":"Rest-Activity Disturbances Correlate with Core Features in Dementia with Lewy Bodies.","authors":"Jack Anderson, Nicholas K H Chiu, Jonathon E Pye, Maria Comas-Soberats, Aaron Lam, Ronald R Grunstein, Simon J G Lewis, Elie Matar","doi":"10.1002/mdc3.70052","DOIUrl":"https://doi.org/10.1002/mdc3.70052","url":null,"abstract":"<p><strong>Background: </strong>Sleep-wake disturbances are a clinically important and poorly studied feature of dementia with Lewy bodies (DLB) due to the challenges of in-laboratory polysomnography in this population.</p><p><strong>Objectives: </strong>To compare rest-activity rhythms in DLB, Parkinson's disease (PD), and age-matched controls using home-based wrist actigraphy and examine their relationship with core clinical DLB features.</p><p><strong>Methods: </strong>Eleven DLB patients, 12 PD patients, and 11 age-matched controls underwent clinical assessment. Actigraphy data were obtained over 14 days and analyzed using nonparametric methods.</p><p><strong>Results: </strong>DLB patients demonstrated higher rest-activity rhythm fragmentation (P = 0.002) than controls and lower circadian amplitude (P = 0.011) than both PD and controls. Instability of rest-activity rhythm was positively correlated with hallucinations (P = 0.009) and cognitive fluctuations (P = 0.016) in DLB, and reduced daytime activity correlated with severity of motor parkinsonism (P = 0.013). No such correlations were observed in PD.</p><p><strong>Conclusions: </strong>Actigraphy detects distinct rest-activity rhythm disruptions in DLB, differentiating it from PD and controls. These measures are associated with the severity of core clinical features in DLB.</p>","PeriodicalId":19029,"journal":{"name":"Movement Disorders Clinical Practice","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143701054","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}