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New Multiomic Studies Shed Light on Cellular Diversity and Neuronal Susceptibility in Parkinson's Disease.
IF 7.4 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2025-01-15 DOI: 10.1002/mds.30097
Marianna Liang, Linh Chu, Zhenyu Yue

Parkinson's disease is a complex neurodegenerative disorder characterized by degeneration of dopaminergic neurons, with patients manifesting varying motor and nonmotor symptoms. Previous studies using single-cell RNA sequencing in rodent models and humans have identified distinct heterogeneity of neurons and glial cells with differential vulnerability. Recent studies have increasingly leveraged multiomics approaches, including spatial transcriptomics, epigenomics, and proteomics, in the study of Parkinson's disease, providing new insights into pathogenic mechanisms. Continued advancements in experimental technologies and sophisticated computational tools will be essential in uncovering a network of neuronal vulnerability and prioritizing disease modifiers for novel therapeutics development. © 2025 International Parkinson and Movement Disorder Society.

{"title":"New Multiomic Studies Shed Light on Cellular Diversity and Neuronal Susceptibility in Parkinson's Disease.","authors":"Marianna Liang, Linh Chu, Zhenyu Yue","doi":"10.1002/mds.30097","DOIUrl":"https://doi.org/10.1002/mds.30097","url":null,"abstract":"<p><p>Parkinson's disease is a complex neurodegenerative disorder characterized by degeneration of dopaminergic neurons, with patients manifesting varying motor and nonmotor symptoms. Previous studies using single-cell RNA sequencing in rodent models and humans have identified distinct heterogeneity of neurons and glial cells with differential vulnerability. Recent studies have increasingly leveraged multiomics approaches, including spatial transcriptomics, epigenomics, and proteomics, in the study of Parkinson's disease, providing new insights into pathogenic mechanisms. Continued advancements in experimental technologies and sophisticated computational tools will be essential in uncovering a network of neuronal vulnerability and prioritizing disease modifiers for novel therapeutics development. © 2025 International Parkinson and Movement Disorder Society.</p>","PeriodicalId":213,"journal":{"name":"Movement Disorders","volume":" ","pages":""},"PeriodicalIF":7.4,"publicationDate":"2025-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142982087","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Step Width Haptic Feedback for Gait Stability in Spinocerebellar Ataxia: Preliminary Results.
IF 7.4 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2025-01-13 DOI: 10.1002/mds.30117
Hong Wang, Zakir Ullah, Eran Gazit, Marina Brozgol, Jeffrey M Hausdorff, Peter B Shull, Penina Ponger

Background: Wider step width and lower step-to-step variability are linked to improved gait stability and reduced fall risk. It is unclear if patients with spinocerebellar ataxia (SCA) can learn to adjust these aspects of gait to reduce fall risk.

Objectives: The aims were to examine the possibility of using wearable step width haptic biofeedback to enhance gait stability and reduce fall risk in individuals with SCA.

Methods: Thirteen people with SCA type 3 performed step width training (single session) using real-time feedback.

Results: Step width increased post-training (19.3 cm, interquartile range [IQR] 16.3-20.2 cm) and at retention (16.6 cm, IQR 16.2-21.1 cm), compared to baseline (11.0 cm, IQR 5.2-15.2 cm; P < 0.001). Step width variability decreased during post-training (19.7%, IQR 17.4%-26.2%) and at retention (22.3%, IQR 18.6%-30.2%), compared to baseline (44.5%, IQR 28.5%-71.2%; P < 0.001). Crossover steps, another mark of instability, decreased after training (P < 0.031).

Conclusions: These pilot results suggest that patients with SCA can use a novel, wearable biofeedback system to improve their gait stability. © 2025 International Parkinson and Movement Disorder Society.

{"title":"Step Width Haptic Feedback for Gait Stability in Spinocerebellar Ataxia: Preliminary Results.","authors":"Hong Wang, Zakir Ullah, Eran Gazit, Marina Brozgol, Jeffrey M Hausdorff, Peter B Shull, Penina Ponger","doi":"10.1002/mds.30117","DOIUrl":"https://doi.org/10.1002/mds.30117","url":null,"abstract":"<p><strong>Background: </strong>Wider step width and lower step-to-step variability are linked to improved gait stability and reduced fall risk. It is unclear if patients with spinocerebellar ataxia (SCA) can learn to adjust these aspects of gait to reduce fall risk.</p><p><strong>Objectives: </strong>The aims were to examine the possibility of using wearable step width haptic biofeedback to enhance gait stability and reduce fall risk in individuals with SCA.</p><p><strong>Methods: </strong>Thirteen people with SCA type 3 performed step width training (single session) using real-time feedback.</p><p><strong>Results: </strong>Step width increased post-training (19.3 cm, interquartile range [IQR] 16.3-20.2 cm) and at retention (16.6 cm, IQR 16.2-21.1 cm), compared to baseline (11.0 cm, IQR 5.2-15.2 cm; P < 0.001). Step width variability decreased during post-training (19.7%, IQR 17.4%-26.2%) and at retention (22.3%, IQR 18.6%-30.2%), compared to baseline (44.5%, IQR 28.5%-71.2%; P < 0.001). Crossover steps, another mark of instability, decreased after training (P < 0.031).</p><p><strong>Conclusions: </strong>These pilot results suggest that patients with SCA can use a novel, wearable biofeedback system to improve their gait stability. © 2025 International Parkinson and Movement Disorder Society.</p>","PeriodicalId":213,"journal":{"name":"Movement Disorders","volume":" ","pages":""},"PeriodicalIF":7.4,"publicationDate":"2025-01-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142968816","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Reply to: “Neutrophil‐Rich Infusion Site Reactions after Continuous Subcutaneous Application of Foslevodopa/Foscarbidopa”
IF 8.6 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2025-01-11 DOI: 10.1002/mds.30120
Nagisa Yoshihara, Rei Watanabe, Noriko Nishikawa, Nobutaka Hattori
{"title":"Reply to: “Neutrophil‐Rich Infusion Site Reactions after Continuous Subcutaneous Application of Foslevodopa/Foscarbidopa”","authors":"Nagisa Yoshihara, Rei Watanabe, Noriko Nishikawa, Nobutaka Hattori","doi":"10.1002/mds.30120","DOIUrl":"https://doi.org/10.1002/mds.30120","url":null,"abstract":"","PeriodicalId":213,"journal":{"name":"Movement Disorders","volume":"41 1","pages":""},"PeriodicalIF":8.6,"publicationDate":"2025-01-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142961449","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Neutrophil‐Rich Infusion Site Reactions After Continuous Subcutaneous Application of Foslevodopa/Foscarbidopa
IF 8.6 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2025-01-11 DOI: 10.1002/mds.30121
David Weise, Sebastian Haferkamp
{"title":"Neutrophil‐Rich Infusion Site Reactions After Continuous Subcutaneous Application of Foslevodopa/Foscarbidopa","authors":"David Weise, Sebastian Haferkamp","doi":"10.1002/mds.30121","DOIUrl":"https://doi.org/10.1002/mds.30121","url":null,"abstract":"","PeriodicalId":213,"journal":{"name":"Movement Disorders","volume":"45 1","pages":""},"PeriodicalIF":8.6,"publicationDate":"2025-01-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142961438","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
TDP‐43 Cryptic RNAs in Perry Syndrome: Differences across Brain Regions and TDP‐43 Proteinopathies
IF 8.6 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2025-01-10 DOI: 10.1002/mds.30104
Sarah R. Pickles, Jesus Gonzalez Bejarano, Anand Narayan, Lillian Daughrity, Candela Maroto Cidfuentes, Madison M. Reeves, Mei Yue, Paula Castellanos Otero, Virginia Estades Ayuso, Judy Dunmore, Yuping Song, Jimei Tong, Michael DeTure, Bailey Rawlinson, Monica Castanedes‐Casey, Jaroslaw Dulski, Catalina Cerquera‐Cleves, Yongjie Zhang, Keith A. Josephs, Dennis W. Dickson, Leonard Petrucelli, Zbigniew K. Wszolek, Mercedes Prudencio
BackgroundPerry syndrome (PS) is a rare and fatal hereditary autosomal dominant neurodegenerative disorder caused by mutations in dynactin (DCTN1). PS brains accumulate inclusions positive for ubiquitin, transactive‐response DNA‐binding protein of 43 kDa (TDP‐43), and to a lesser extent dynactin.ObjectivesLittle is known regarding the contributions of TDP‐43, an RNA binding protein that represses cryptic exon inclusion, in PS. Therefore, we sought to identify the degree of TDP‐43 dysfunction in two regions of PS brains.MethodsWe evaluated the levels of insoluble pTDP‐43 and TDP‐43‐regulated cryptic RNAs and protein in the caudate nucleus and substantia nigra of 7 PS cases, 12 cases of frontotemporal lobar degeneration (FTLD) with TDP‐43 pathology, and 11 cognitively healthy controls without TDP‐43 pathology.ResultsInsoluble pTDP‐43 protein levels were detected in PS brains to a similar extent in the caudate nucleus and substantia nigra but lower than those in FTLD brains. The caudate nucleus of PS showed accumulation of eight TDP‐43‐regulated cryptic RNAs (ACTL6B, CAMK2B, STMN2, UNC13A, KCNQ2, ATG4B, GPSM2, and HDGFL2) and cryptic protein (HDGFL2) characteristic of FTLD. Conversely, only one cryptic target, UNC13A, reached significance in the substantia nigra despite similar pTDP‐43 levels.ConclusionWe detected TDP‐43 cryptic RNAs and protein in PS caudate nucleus. Given the importance of cryptic exon biology in the development of biomarkers, and the identification of novel targets for therapeutic intervention, it is imperative we understand the consequences of TDP‐43 dysfunction across different brain regions and determine the targets that are specific and common to TDP‐43 proteinopathies. © 2025 The Author(s). Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.
{"title":"TDP‐43 Cryptic RNAs in Perry Syndrome: Differences across Brain Regions and TDP‐43 Proteinopathies","authors":"Sarah R. Pickles, Jesus Gonzalez Bejarano, Anand Narayan, Lillian Daughrity, Candela Maroto Cidfuentes, Madison M. Reeves, Mei Yue, Paula Castellanos Otero, Virginia Estades Ayuso, Judy Dunmore, Yuping Song, Jimei Tong, Michael DeTure, Bailey Rawlinson, Monica Castanedes‐Casey, Jaroslaw Dulski, Catalina Cerquera‐Cleves, Yongjie Zhang, Keith A. Josephs, Dennis W. Dickson, Leonard Petrucelli, Zbigniew K. Wszolek, Mercedes Prudencio","doi":"10.1002/mds.30104","DOIUrl":"https://doi.org/10.1002/mds.30104","url":null,"abstract":"BackgroundPerry syndrome (PS) is a rare and fatal hereditary autosomal dominant neurodegenerative disorder caused by mutations in dynactin (<jats:italic>DCTN1</jats:italic>). PS brains accumulate inclusions positive for ubiquitin, transactive‐response DNA‐binding protein of 43 kDa (TDP‐43), and to a lesser extent dynactin.ObjectivesLittle is known regarding the contributions of TDP‐43, an RNA binding protein that represses cryptic exon inclusion, in PS. Therefore, we sought to identify the degree of TDP‐43 dysfunction in two regions of PS brains.MethodsWe evaluated the levels of insoluble pTDP‐43 and TDP‐43‐regulated cryptic RNAs and protein in the caudate nucleus and substantia nigra of 7 PS cases, 12 cases of frontotemporal lobar degeneration (FTLD) with TDP‐43 pathology, and 11 cognitively healthy controls without TDP‐43 pathology.ResultsInsoluble pTDP‐43 protein levels were detected in PS brains to a similar extent in the caudate nucleus and substantia nigra but lower than those in FTLD brains. The caudate nucleus of PS showed accumulation of eight TDP‐43‐regulated cryptic RNAs (<jats:italic>ACTL6B</jats:italic>, <jats:italic>CAMK2B</jats:italic>, <jats:italic>STMN2</jats:italic>, <jats:italic>UNC13A</jats:italic>, <jats:italic>KCNQ2</jats:italic>, <jats:italic>ATG4B</jats:italic>, <jats:italic>GPSM2</jats:italic>, and <jats:italic>HDGFL2</jats:italic>) and cryptic protein (HDGFL2) characteristic of FTLD. Conversely, only one cryptic target, <jats:italic>UNC13A</jats:italic>, reached significance in the substantia nigra despite similar pTDP‐43 levels.ConclusionWe detected TDP‐43 cryptic RNAs and protein in PS caudate nucleus. Given the importance of cryptic exon biology in the development of biomarkers, and the identification of novel targets for therapeutic intervention, it is imperative we understand the consequences of TDP‐43 dysfunction across different brain regions and determine the targets that are specific and common to TDP‐43 proteinopathies. © 2025 The Author(s). <jats:italic>Movement Disorders</jats:italic> published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.","PeriodicalId":213,"journal":{"name":"Movement Disorders","volume":"1 1","pages":""},"PeriodicalIF":8.6,"publicationDate":"2025-01-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142940146","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Effect of a Wrist Harmonic Liquid Tremor Absorber-Damping on the Upper Extremity Essential Tremor.
IF 7.4 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2025-01-10 DOI: 10.1002/mds.30102
Sultan Tarlacı, Furkan Doğan

Background: Essential tremor (ET) is a common type of tremor. Previous research has shown that wearable orthoses and biomechanical loading methods can suppress tremors.

Objective: This study aims to investigate the effect of a harmonic liquid dampener on upper extremity ET.

Methods: The study involved 9 women and 5 men from a neurology outpatient clinic. A 285-g liquid-based harmonic dampening wristband was used. Tremors were recorded from the wrist along the x-y-z axes using a vibrometer, and the Bain-Findley Rating Scale (BFRS) was employed for assessment.

Results: BFRS scores significantly improved after the wristband was used (mean difference 35.64, P = 0.001, Cohen's d effect size =2.979). The tremor amplitude decreased significantly along the x-axis (Cohen's d: 1.35, P = 0.001), y-axis (Cohen's d: 3.232, P = 0.001), and z-axis (Cohen's d: 3.321, P = 0.001).

Conclusion: The liquid-filled harmonic dampening wristband shows promise as a new, effective treatment option for ET patients. © 2025 International Parkinson and Movement Disorder Society.

{"title":"Effect of a Wrist Harmonic Liquid Tremor Absorber-Damping on the Upper Extremity Essential Tremor.","authors":"Sultan Tarlacı, Furkan Doğan","doi":"10.1002/mds.30102","DOIUrl":"https://doi.org/10.1002/mds.30102","url":null,"abstract":"<p><strong>Background: </strong>Essential tremor (ET) is a common type of tremor. Previous research has shown that wearable orthoses and biomechanical loading methods can suppress tremors.</p><p><strong>Objective: </strong>This study aims to investigate the effect of a harmonic liquid dampener on upper extremity ET.</p><p><strong>Methods: </strong>The study involved 9 women and 5 men from a neurology outpatient clinic. A 285-g liquid-based harmonic dampening wristband was used. Tremors were recorded from the wrist along the x-y-z axes using a vibrometer, and the Bain-Findley Rating Scale (BFRS) was employed for assessment.</p><p><strong>Results: </strong>BFRS scores significantly improved after the wristband was used (mean difference 35.64, P = 0.001, Cohen's d effect size =2.979). The tremor amplitude decreased significantly along the x-axis (Cohen's d: 1.35, P = 0.001), y-axis (Cohen's d: 3.232, P = 0.001), and z-axis (Cohen's d: 3.321, P = 0.001).</p><p><strong>Conclusion: </strong>The liquid-filled harmonic dampening wristband shows promise as a new, effective treatment option for ET patients. © 2025 International Parkinson and Movement Disorder Society.</p>","PeriodicalId":213,"journal":{"name":"Movement Disorders","volume":" ","pages":""},"PeriodicalIF":7.4,"publicationDate":"2025-01-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142941883","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Prospective Multicenter Evaluation of the MDS "Suggestive of PSP" Diagnostic Criteria.
IF 7.4 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2025-01-10 DOI: 10.1002/mds.30112
Andrea Quattrone, Nicolai Franzmeier, Johannes Levin, Gabor C Petzold, Annika Spottke, Frederic Brosseron, Björn Falkenburger, Johannes Prudlo, Thomas Gasser, Günter U Höglinger

Background: The recent Movement Disorders Society (MDS)-progressive supranuclear palsy (PSP) diagnostic criteria conceptualized three clinical diagnostic certainty levels: "suggestive of PSP" for sensitive early diagnosis based on subtle clinical signs, "possible PSP" balancing sensitivity and specificity, and "probable PSP" highly specific for PSP pathology.

Objective: The aim of this study was to prospectively validate the criteria against long-term clinical follow-up and characterize the diagnostic certainty increase over time.

Methods: Patients with "possible PSP" or "suggestive of PSP" diagnosis and clinical follow-up were recruited in two German multicenter longitudinal observational studies (ProPSP and DescribePSP). The cumulative percentage of patients longitudinally increasing diagnostic certainty was assessed over up to 2.5 years of follow-up. The sample size per arm required to detect 30% attenuated rate in diagnostic certainty increase in trials was estimated over multiple time intervals.

Results: Of 254 patients with available longitudinal data, 61 patients had low diagnostic certainty at baseline (48 suggestive of PSP, 13 possible PSP) and multiple clinical visits (median: 3, range: 2-4). The cumulative percentage of patients increasing diagnostic certainty progressed with follow-up duration (30.4% at 6 months, 51.7% at 1 year, 80.4% at 2.5 years). The sample size required to detect 30% reduction in diagnostic certainty increase rate within 1 year was 163, slightly smaller than that required using the PSP rating scale.

Conclusions: Most "suggestive of PSP" patients increased diagnostic certainty upon longitudinal follow-up, providing the first prospective multicenter validation of MDS-PSP diagnostic criteria. Our data support the design of trials tailored for these early-stage patients, suggesting the PSP rating scale and the diagnostic certainty increase rate as potential endpoint measures. © 2025 The Author(s). Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

{"title":"Prospective Multicenter Evaluation of the MDS \"Suggestive of PSP\" Diagnostic Criteria.","authors":"Andrea Quattrone, Nicolai Franzmeier, Johannes Levin, Gabor C Petzold, Annika Spottke, Frederic Brosseron, Björn Falkenburger, Johannes Prudlo, Thomas Gasser, Günter U Höglinger","doi":"10.1002/mds.30112","DOIUrl":"https://doi.org/10.1002/mds.30112","url":null,"abstract":"<p><strong>Background: </strong>The recent Movement Disorders Society (MDS)-progressive supranuclear palsy (PSP) diagnostic criteria conceptualized three clinical diagnostic certainty levels: \"suggestive of PSP\" for sensitive early diagnosis based on subtle clinical signs, \"possible PSP\" balancing sensitivity and specificity, and \"probable PSP\" highly specific for PSP pathology.</p><p><strong>Objective: </strong>The aim of this study was to prospectively validate the criteria against long-term clinical follow-up and characterize the diagnostic certainty increase over time.</p><p><strong>Methods: </strong>Patients with \"possible PSP\" or \"suggestive of PSP\" diagnosis and clinical follow-up were recruited in two German multicenter longitudinal observational studies (ProPSP and DescribePSP). The cumulative percentage of patients longitudinally increasing diagnostic certainty was assessed over up to 2.5 years of follow-up. The sample size per arm required to detect 30% attenuated rate in diagnostic certainty increase in trials was estimated over multiple time intervals.</p><p><strong>Results: </strong>Of 254 patients with available longitudinal data, 61 patients had low diagnostic certainty at baseline (48 suggestive of PSP, 13 possible PSP) and multiple clinical visits (median: 3, range: 2-4). The cumulative percentage of patients increasing diagnostic certainty progressed with follow-up duration (30.4% at 6 months, 51.7% at 1 year, 80.4% at 2.5 years). The sample size required to detect 30% reduction in diagnostic certainty increase rate within 1 year was 163, slightly smaller than that required using the PSP rating scale.</p><p><strong>Conclusions: </strong>Most \"suggestive of PSP\" patients increased diagnostic certainty upon longitudinal follow-up, providing the first prospective multicenter validation of MDS-PSP diagnostic criteria. Our data support the design of trials tailored for these early-stage patients, suggesting the PSP rating scale and the diagnostic certainty increase rate as potential endpoint measures. © 2025 The Author(s). Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.</p>","PeriodicalId":213,"journal":{"name":"Movement Disorders","volume":" ","pages":""},"PeriodicalIF":7.4,"publicationDate":"2025-01-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142963251","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Two Families with ANO10-Related Spinocerebellar Ataxia with Novel Exon Deletions: A First Report from India.
IF 7.4 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2025-01-08 DOI: 10.1002/mds.30107
Vikram V Holla, Nitish Kamble, Reghunathan Sindhu Harishma, Gautham Arunachal, Ravi Yadav, Pramod Kumar Pal
{"title":"Two Families with ANO10-Related Spinocerebellar Ataxia with Novel Exon Deletions: A First Report from India.","authors":"Vikram V Holla, Nitish Kamble, Reghunathan Sindhu Harishma, Gautham Arunachal, Ravi Yadav, Pramod Kumar Pal","doi":"10.1002/mds.30107","DOIUrl":"https://doi.org/10.1002/mds.30107","url":null,"abstract":"","PeriodicalId":213,"journal":{"name":"Movement Disorders","volume":" ","pages":""},"PeriodicalIF":7.4,"publicationDate":"2025-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142941897","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Rigidity in Parkinson's Disease: The Objective Effect of Levodopa.
IF 7.4 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2025-01-08 DOI: 10.1002/mds.30114
Marco Falletti, Francesco Asci, Alessandro Zampogna, Martina Patera, Giulia Pinola, Diego Centonze, Mark Hallett, John Rothwell, Antonio Suppa

Background: Quantitative evidence of levodopa-induced beneficial effects on parkinsonian rigidity in Parkinson's disease (PD) is lacking. Recent research has demonstrated the velocity-dependent nature of objective rigidity in PD and revealed its neural underpinning.

Objective: The present study aimed to examine the effect of levodopa on objective rigidity in PD.

Methods: Eighteen patients with PD underwent clinical and instrumental evaluations of muscle tone in the OFF and ON states. The clinical assessments focused on rigidity in the most affected upper limb. The biomechanical components of objective rigidity were assessed using robot-assisted wrist extensions at seven angular velocities (5-280°/s). Surface electromyography of the flexor carpi radialis muscle enabled the concurrent evaluation of short- and long-latency stretch reflexes (SLR and LLR).

Results: Levodopa improved the clinical scores of rigidity. Biomechanical measurements showed that levodopa reduced the total and neural components of force but had no effect on viscoelastic components. Levodopa reduced the velocity dependence of the LLRs but did not affect the SLRs. Finally, we found significant clinical-instrumental correlations between levodopa-induced changes and biomechanical and neurophysiological measures of objective rigidity in PD.

Conclusions: Levodopa improved objective rigidity in PD by decreasing its biomechanical neural component as well as the size of LLRs. The beneficial effect of levodopa on biomechanical and neurophysiological features of objective rigidity was related to the specific angular velocity of wrist extensions; that is, the higher the angular velocity, the greater the beneficial impact of levodopa on objective rigidity. These findings allowed the description of a new pathophysiological model of rigidity in PD. © 2025 The Author(s). Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

{"title":"Rigidity in Parkinson's Disease: The Objective Effect of Levodopa.","authors":"Marco Falletti, Francesco Asci, Alessandro Zampogna, Martina Patera, Giulia Pinola, Diego Centonze, Mark Hallett, John Rothwell, Antonio Suppa","doi":"10.1002/mds.30114","DOIUrl":"https://doi.org/10.1002/mds.30114","url":null,"abstract":"<p><strong>Background: </strong>Quantitative evidence of levodopa-induced beneficial effects on parkinsonian rigidity in Parkinson's disease (PD) is lacking. Recent research has demonstrated the velocity-dependent nature of objective rigidity in PD and revealed its neural underpinning.</p><p><strong>Objective: </strong>The present study aimed to examine the effect of levodopa on objective rigidity in PD.</p><p><strong>Methods: </strong>Eighteen patients with PD underwent clinical and instrumental evaluations of muscle tone in the OFF and ON states. The clinical assessments focused on rigidity in the most affected upper limb. The biomechanical components of objective rigidity were assessed using robot-assisted wrist extensions at seven angular velocities (5-280°/s). Surface electromyography of the flexor carpi radialis muscle enabled the concurrent evaluation of short- and long-latency stretch reflexes (SLR and LLR).</p><p><strong>Results: </strong>Levodopa improved the clinical scores of rigidity. Biomechanical measurements showed that levodopa reduced the total and neural components of force but had no effect on viscoelastic components. Levodopa reduced the velocity dependence of the LLRs but did not affect the SLRs. Finally, we found significant clinical-instrumental correlations between levodopa-induced changes and biomechanical and neurophysiological measures of objective rigidity in PD.</p><p><strong>Conclusions: </strong>Levodopa improved objective rigidity in PD by decreasing its biomechanical neural component as well as the size of LLRs. The beneficial effect of levodopa on biomechanical and neurophysiological features of objective rigidity was related to the specific angular velocity of wrist extensions; that is, the higher the angular velocity, the greater the beneficial impact of levodopa on objective rigidity. These findings allowed the description of a new pathophysiological model of rigidity in PD. © 2025 The Author(s). Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.</p>","PeriodicalId":213,"journal":{"name":"Movement Disorders","volume":" ","pages":""},"PeriodicalIF":7.4,"publicationDate":"2025-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142941888","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The Lows of High Reward: Choking Under Pressure.
IF 7.4 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2025-01-08 DOI: 10.1002/mds.30092
Anna Sadnicka, Mark J Edwards
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Movement Disorders
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