Cerebellum最新文献

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Potentiation of Motor Adaptation Via Cerebellar tACS: Characterization of the Stimulation Frequency. 小脑tACS对运动适应的潜能:刺激频率的特征
IF 2.7 3区 医学
Cerebellum Pub Date : 2024-10-22 DOI: 10.1007/s12311-024-01748-0
Paulo Figueroa-Taiba, Joel Álvarez-Ruf, Paulette Ulloa, Trinidad Bruna-Melo, Liam Espinoza-Maraboli, Pablo Ignacio Burgos, Juan J Mariman
{"title":"Potentiation of Motor Adaptation Via Cerebellar tACS: Characterization of the Stimulation Frequency.","authors":"Paulo Figueroa-Taiba, Joel Álvarez-Ruf, Paulette Ulloa, Trinidad Bruna-Melo, Liam Espinoza-Maraboli, Pablo Ignacio Burgos, Juan J Mariman","doi":"10.1007/s12311-024-01748-0","DOIUrl":"https://doi.org/10.1007/s12311-024-01748-0","url":null,"abstract":"<p><p>Motor adaptation is critical to update motor tasks in new or modified environmental conditions. While the cerebellum supports error-based adaptations, its neural implementation is partially known. By controlling the frequency of cerebellar transcranial alternating current stimulation (c-tACS), we can test the influence of neural oscillation from the cerebellum for motor adaptation. Two independent experiments were conducted. In Experiment 1, 16 participants received four c-tACS protocols (45 Hz, 50 Hz, 55 Hz, and sham) on four different days while they practiced a visuomotor adaptation task (30 degrees CCW) with variable intensity (within-subject design). In Experiment 2, 45 participants separated into three groups received the effect of 45 Hz, 55 Hz c-tACS, and sham, respectively (between-subject design), performing the same visuomotor task with a fixed intensity (0.9 mA). In Experiment 1, 45 Hz and 50 Hz of c-tACS accelerated motor adaptation when participants performed the task only for the first time, independent of the time interval between sessions or the stimulation intensity. The effect of active c-tACS was ratified in Experiment 2, where 45 Hz c-tACS benefits motor adaptation during the complete practice period. Reaction time, velocity, or duration of reaching are not affected by c-tACS. Cerebellar alternating current stimulation is an effective strategy to potentiate visuomotor adaptations. Frequency-dependent effects on the gamma band, especially for 45 Hz c-tACS, ratify the oscillatory profile of cerebellar processes behind the motor adaptation. This can be exploited in future interventions to enhance motor learning.</p>","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142479875","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Correction: Silica Nanoparticles from Melon Seed Husk Abrogated Binary Metal(loid) Mediated Cerebellar Dysfunction by Attenuation of Oxido-inflammatory Response and Upregulation of Neurotrophic Factors in Male Albino Rats. 更正:瓜子壳中的二氧化硅纳米颗粒通过减轻氧化-炎症反应和上调白化雄性大鼠的神经营养因子来缓解二元金属(loid)介导的小脑功能障碍
IF 2.7 3区 医学
Cerebellum Pub Date : 2024-10-18 DOI: 10.1007/s12311-024-01752-4
Chidinma P Anyachor, Chinna N Orish, Anthonet N Ezejiofor, Ana Cirovic, Aleksandar Cirovic, Baridoo Donatus Dooka, Kenneth M Ezealisiji, Xavier Siwe Noundou, Orish E Orisakwe
{"title":"Correction: Silica Nanoparticles from Melon Seed Husk Abrogated Binary Metal(loid) Mediated Cerebellar Dysfunction by Attenuation of Oxido-inflammatory Response and Upregulation of Neurotrophic Factors in Male Albino Rats.","authors":"Chidinma P Anyachor, Chinna N Orish, Anthonet N Ezejiofor, Ana Cirovic, Aleksandar Cirovic, Baridoo Donatus Dooka, Kenneth M Ezealisiji, Xavier Siwe Noundou, Orish E Orisakwe","doi":"10.1007/s12311-024-01752-4","DOIUrl":"https://doi.org/10.1007/s12311-024-01752-4","url":null,"abstract":"","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142479874","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Impact of SARS-CoV-2 Infection on Essential Tremor: A Retrospective Clinical and Kinematic Analysis. SARS-CoV-2 感染对基本性震颤的影响:回顾性临床和运动学分析
IF 2.7 3区 医学
Cerebellum Pub Date : 2024-10-09 DOI: 10.1007/s12311-024-01751-5
Davide Costa, Sofia Grandolfo, Daniele Birreci, Luca Angelini, Massimiliano Passaretti, Antonio Cannavacciuolo, Adriana Martini, Martina De Riggi, Giulia Paparella, Alfonso Fasano, Matteo Bologna
{"title":"Impact of SARS-CoV-2 Infection on Essential Tremor: A Retrospective Clinical and Kinematic Analysis.","authors":"Davide Costa, Sofia Grandolfo, Daniele Birreci, Luca Angelini, Massimiliano Passaretti, Antonio Cannavacciuolo, Adriana Martini, Martina De Riggi, Giulia Paparella, Alfonso Fasano, Matteo Bologna","doi":"10.1007/s12311-024-01751-5","DOIUrl":"https://doi.org/10.1007/s12311-024-01751-5","url":null,"abstract":"<p><p>In the past few years, SARS-CoV-2 infection has substantially impacted public health. Alongside respiratory symptoms, some individuals have reported new neurological manifestations or a worsening of pre-existing neurological conditions. We previously documented two cases of essential tremor (ET) who experienced a deterioration in tremor following SARS-CoV-2 infection. However, the effects of SARS-CoV-2 on ET remain largely unexplored. This study aims to evaluate the impact of SARS-CoV-2 infection on a relatively broad sample of ET patients by retrospectively comparing their clinical and kinematic data collected before and after the exposure to SARS-CoV-2. We surveyed to evaluate the impact of SARS-CoV-2 infection on tremor features in ET. Subsequently, we retrospectively analysed clinical and kinematic data, including accelerometric recordings of postural and kinetic tremor. We included 36 ET patients (14 females with a mean age of 71.1 ± 10.6 years). Among the 25 patients who reported SARS-CoV-2 infection, 11 (44%) noted a subjective worsening of tremor. All patients reporting subjective tremor worsening also exhibited symptoms of long COVID, whereas the prevalence of these symptoms was lower (50%) in those without subjective exacerbation. The retrospective analysis of clinical data revealed a tremor deterioration in infected patients, which was not observed in non-infected patients. Finally, kinematic analysis revealed substantial stability of tremor features in both groups. The study highlighted a potential correlation between the SARS-CoV-2 infection and clinical worsening of ET. Long COVID contributes to a greater impact of tremor on the daily life of ET patients.</p>","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142394963","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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 Impact of Bilateral Cerebellar Transcranial Direct Current Stimulation on Balance Control in Healthy Young Adults. 双侧小脑经颅直流电刺激对健康年轻人平衡控制的影响
IF 2.7 3区 医学
Cerebellum Pub Date : 2024-10-09 DOI: 10.1007/s12311-024-01749-z
Xin Huang, Zhiqin Xu, Lingxiang Zhou, Ke Dong, Qingqing Liu, Jiating Li, Di Lei, Hanjun Liu, Xi Chen
{"title":"The Impact of Bilateral Cerebellar Transcranial Direct Current Stimulation on Balance Control in Healthy Young Adults.","authors":"Xin Huang, Zhiqin Xu, Lingxiang Zhou, Ke Dong, Qingqing Liu, Jiating Li, Di Lei, Hanjun Liu, Xi Chen","doi":"10.1007/s12311-024-01749-z","DOIUrl":"https://doi.org/10.1007/s12311-024-01749-z","url":null,"abstract":"<p><p>Cerebellar transcranial direct current stimulation (tDCS) has been shown to influence movement functions, but little is known about the specific effects of stimulation polarity on balance control. This study investigated the impact of bilateral cerebellar tDCS on balance functions as a function of stimulation polarity. In this randomized, controlled trial, thirty-nine healthy young adults were assigned to one of three groups: right anodal/left cathodal cerebellar stimulation (AC group), right cathodal/left anodal cerebellar stimulation (CA group), and a control sham group. Each participant underwent a daily 30-minute session of tDCS at 2 mA for one week. Balance function was assessed pre- and post-intervention and the data were analyzed using generalized estimating equations. The CA group exhibited a significant reduction in sway area when standing on the left leg and on both stable and unstable surfaces with eyes open, compared to both the AC and sham groups. However, there were no significant differences among the groups in terms of sway length, anteroposterior velocity, or mediolateral velocity. Our results indicate the polarity-dependent effects of bilateral cerebellar tDCS on balance functions, with enhanced stability observed only following cathodal tDCS over the right cerebellum paired with anodal tDCS over the left cerebellum. This polarity-specific modulation may have implications for developing cerebellar neuromodulation interventions for movement disorders.</p>","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142394977","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Differential Effects of Cerebellar Transcranial Direct Current Stimulation with Gait Training on Functional Mobility, Balance, and Ataxia Symptoms. 小脑经颅直流电刺激与步态训练对功能活动度、平衡和共济失调症状的不同影响
IF 2.7 3区 医学
Cerebellum Pub Date : 2024-10-05 DOI: 10.1007/s12311-024-01750-6
Rodrigo Brito, João Victor Fabrício, Aurine Araujo, Mariana Sacchi, Adriana Baltar, Fernanda Albuquerque Lima, Ana Cecília Ribeiro, Bárbara Sousa, Camilla Santos, Clarice Tanaka, Kátia Monte-Silva
{"title":"Differential Effects of Cerebellar Transcranial Direct Current Stimulation with Gait Training on Functional Mobility, Balance, and Ataxia Symptoms.","authors":"Rodrigo Brito, João Victor Fabrício, Aurine Araujo, Mariana Sacchi, Adriana Baltar, Fernanda Albuquerque Lima, Ana Cecília Ribeiro, Bárbara Sousa, Camilla Santos, Clarice Tanaka, Kátia Monte-Silva","doi":"10.1007/s12311-024-01750-6","DOIUrl":"https://doi.org/10.1007/s12311-024-01750-6","url":null,"abstract":"<p><p>Cerebellar transcranial direct current stimulation (ctDCS) has emerged as a promising, non-invasive, and safe neuromodulatory intervention capable of reducing ataxia symptoms and restoring cerebellum-motor connectivity. However, previous studies have only applied ctDCS in isolation, without association with specific training. This study aimed to assess the effect of ctDCS combined with gait training on functional mobility, balance, and symptoms and severity of ataxia. A randomized, triple-blind, sham-controlled, bi-center clinical trial was conducted with forty-four adults with cerebellar ataxia. Volunteers were randomized to receive five daily sessions of either real ctDCS (n = 11; 2 mA for 25 min) or sham ctDCS (n = 11) during gait training. Functional mobility, balance, and symptoms and severity of ataxia were assessed using the Time Up and Go test, the MiniBESTest, and the Scale for the Assessment and Rating of Ataxia (SARA), respectively, before and after the interventions. Both groups showed improvement in functional mobility, but there was no significant difference between the ctDCS and sham groups. However, the ctDCS group demonstrated significant improvements in cerebellar ataxia severity as reflected by SARA scores, particularly in tests of stance, sitting, speech disturbance, nose-finger test, and heel-shin slide test. Notably, no improvements were observed in balance. This study indicates that while ctDCS combined with gait training may improve specific symptoms of cerebellar ataxia, it does not significantly enhance overall functional mobility compared to sham treatment.</p>","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142378544","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Differences in the Impact of Intensive Rehabilitation on Hereditary Ataxias and the Cerebellar Subtype of Multiple System Atrophy. 强化康复对遗传性共济失调和多系统萎缩症小脑亚型的影响存在差异。
IF 2.7 3区 医学
Cerebellum Pub Date : 2024-10-04 DOI: 10.1007/s12311-024-01744-4
Kyota Bando, Yuki Kondo, Yuta Miyazaki, Takatoshi Hara, Yuji Takahashi
{"title":"Differences in the Impact of Intensive Rehabilitation on Hereditary Ataxias and the Cerebellar Subtype of Multiple System Atrophy.","authors":"Kyota Bando, Yuki Kondo, Yuta Miyazaki, Takatoshi Hara, Yuji Takahashi","doi":"10.1007/s12311-024-01744-4","DOIUrl":"https://doi.org/10.1007/s12311-024-01744-4","url":null,"abstract":"<p><p>Multiple system atrophy-cerebellar type (MSA-C) exhibits faster disease progression than does hereditary spinocerebellar degeneration (hSCD). In this study, we aimed to investigate the differences in the treatment effects and sustainability of intensive rehabilitation between patients with hSCD and those with MSA-C. Forty-nine patients (hSCD = 30, MSA-C = 19) underwent a 2- or 4-week intensive rehabilitation program. Balance function was evaluated using the scale for the assessment and rating of ataxia (SARA) and the balance evaluation systems test (BESTest) at pre-intervention, post-intervention, and 6-month follow-up. Notably, both groups demonstrated beneficial effects from the rehabilitation intervention. However, differences were observed in the magnitude and duration of these effects. In the hSCD group, the SARA scores at follow-up remained similar to those at baseline, indicating sustained benefits. However, the MSA-C group showed some deterioration in SARA scores compared with baseline scores but maintained improvements on the BESTest, demonstrating partial sustainability. Differences, mainly in sustainability, were observed between the hSCD and MSA-C groups. This may be due to varying rates of symptom progression. The findings of this study are significant when considering the frequency of follow-ups based on disease type.</p>","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142373471","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Psychometric Validation of the Modified Functional Scale for the Assessment and Rating of Ataxia (f-SARA) in Patients With Spinocerebellar Ataxia. 脊髓小脑共济失调患者共济失调评估和评级修正功能量表(f-SARA)的心理计量学验证。
IF 2.7 3区 医学
Cerebellum Pub Date : 2024-10-01 Epub Date: 2024-06-12 DOI: 10.1007/s12311-024-01707-9
Michele Potashman, Evan Popoff, Lauren Powell, Ainsley Mackenzie, Melissa Wolfe Beiner, Vlad Coric, Jeremy Schmahmann, Gilbert L'Italien
{"title":"Psychometric Validation of the Modified Functional Scale for the Assessment and Rating of Ataxia (f-SARA) in Patients With Spinocerebellar Ataxia.","authors":"Michele Potashman, Evan Popoff, Lauren Powell, Ainsley Mackenzie, Melissa Wolfe Beiner, Vlad Coric, Jeremy Schmahmann, Gilbert L'Italien","doi":"10.1007/s12311-024-01707-9","DOIUrl":"10.1007/s12311-024-01707-9","url":null,"abstract":"<p><p>This study aimed to generate evidence to support psychometric validity of the modified functional Scale for the Assessment and Rating of Ataxia (f-SARA) among patients with spinocerebellar ataxia (SCA). Psychometric measurement properties and minimal change thresholds of the f-SARA were evaluated using data from a cohort of SCA subjects (recruited at Massachusetts General Hospital [MGH]; n = 33) and data from a phase 3 trial of troriluzole in adults with SCA (NCT03701399 [Study 206]; n = 217), including a subset of patients with the SCA3 genotype (n = 89). f-SARA item ceiling effects were absent within the MGH cohort, while floor effects were present. Excellent internal consistency reliability was demonstrated (α<sub>total</sub> = 0.90; α<sub>items-removed</sub> = 0.86-0.90), and item-to-total correlations were strong (r = 0.82-0.91, per item). High test-retest reliability was demonstrated with intraclass correlation coefficients of 0.91 (total) and 0.73-0.92 (items). Convergent and divergent validity was supported, with strong correlations observed between the f-SARA and similarly constructed scales (FARS-FUNC, BARS, PROM-ADL, and FARS-ADL; all p < 0.001) and weaker correlations observed among measures of differing constructs. Mean item and total scores increased with disease severity (by FARS-FUNC quartile; p < 0.001). A 1-point threshold for meaningful changes was supported as 0.5 × SD = 0.89, SEM = 1.12, and mean changes from baseline for patients classified as \"improved,\" \"no change,\" or \"deteriorated\" were -0.68, 0.02, and 0.58, respectively. Similar trends were observed in Study 206 all-SCA and SCA3 cohorts. The measurement properties of the f-SARA provide evidence of its psychometric validity, responsiveness, and suitability as a clinical outcome measure in patients with SCA, including those with SCA3.</p>","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11489232/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141307263","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Development and Validation of SCACOMS, a Composite Scale for Assessing Disease Progression and Treatment Effects in Spinocerebellar Ataxia. 脊髓小脑共济失调疾病进展和治疗效果综合评估量表 SCACOMS 的开发与验证
IF 2.7 3区 医学
Cerebellum Pub Date : 2024-10-01 Epub Date: 2024-05-07 DOI: 10.1007/s12311-024-01697-8
Gilbert L'Italien, Evan Popoff, Basia Rogula, Lauren Powell, Michele Potashman, Sam Dickson, Patrick O'Keefe, Melissa Beiner, Vlad Coric, Susan Perlman, Jeremy D Schmahmann, Suzanne Hendrix
{"title":"Development and Validation of SCACOMS, a Composite Scale for Assessing Disease Progression and Treatment Effects in Spinocerebellar Ataxia.","authors":"Gilbert L'Italien, Evan Popoff, Basia Rogula, Lauren Powell, Michele Potashman, Sam Dickson, Patrick O'Keefe, Melissa Beiner, Vlad Coric, Susan Perlman, Jeremy D Schmahmann, Suzanne Hendrix","doi":"10.1007/s12311-024-01697-8","DOIUrl":"10.1007/s12311-024-01697-8","url":null,"abstract":"<p><p>Spinocerebellar ataxias (SCA) are rare inherited neurodegenerative disorders characterized by a progressive impairment of gait, balance, limb coordination, and speech. There is currently no composite scale that includes multiple aspects of the SCA experience to assess disease progression and treatment effects. Applying the method of partial least squares (PLS) regression, we developed the Spinocerebellar Ataxia Composite Scale (SCACOMS) from two SCA natural history datasets (NCT01060371, NCT02440763). PLS regression selected items based on their ability to detect clinical decline, with optimized weights based on the item's degree of progression. Following model validation, SCACOMS was leveraged to examine disease progression and treatment effects in a 48-week SCA clinical trial cohort (NCT03701399). Items from the Clinical Global Impression-Global Improvement Scale (CGI-I), the Friedreich Ataxia Rating Scale (FARS) - functional stage, and the Modified Functional Scale for the Assessment and Rating of Ataxia (f-SARA) were objectively selected with weightings based on their sensitivity to clinical decline. The resulting SCACOMS exhibited improved sensitivity to disease progression and greater treatment effects (compared to the original scales from which they were derived) in a 48-week clinical trial of a novel therapeutic agent. The trial analyses also provided a SCACOMS-derived estimate of the temporal delay in SCA disease progression. SCACOMS is a useful composite measure, effectively capturing disease progression and highlighting treatment effects in patients with SCA. SCACOMS will be a powerful tool in future studies given its sensitivity to clinical decline and ability to detect a meaningful clinical impact of disease-modifying treatments.</p>","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11489241/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140861127","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Quantitative Evaluation of Stance as a Sensitive Biomarker of Postural Ataxia Development in Preclinical SCA1 Mutation Carriers. 将姿态定量评估作为临床前 SCA1 突变携带者姿势共济失调发展的敏感生物标志物
IF 2.7 3区 医学
Cerebellum Pub Date : 2024-10-01 Epub Date: 2024-03-16 DOI: 10.1007/s12311-024-01679-w
Anna Sobanska, Leszek Czerwosz, Anna Sulek, Rafal Rola, Iwona Stepniak, Maria Rakowicz
{"title":"Quantitative Evaluation of Stance as a Sensitive Biomarker of Postural Ataxia Development in Preclinical SCA1 Mutation Carriers.","authors":"Anna Sobanska, Leszek Czerwosz, Anna Sulek, Rafal Rola, Iwona Stepniak, Maria Rakowicz","doi":"10.1007/s12311-024-01679-w","DOIUrl":"10.1007/s12311-024-01679-w","url":null,"abstract":"<p><p>The aim of this study was to determine the time between the first detection of postural control impairments and the evident manifestation of ataxia in preclinical SCA1 individuals. Twenty five preclinical SCA1 mutation carriers: 13 with estimated disease onset ≤ 6 years (SCA1 +) aged 27.8 ± 8.1 years; 12 with expected disease onset > 6 years (SCA1-) aged 26.6 ± 3.1 years and 26 age and sex matched healthy controls (HCs) underwent static posturography during 5 years of observation. The movements of the centre of feet pressure (COP) during quiet standing with eyes open (EO) and closed (EC) were quantified by calculating the mean radius (R), developed surface area (A) and mean COP movement velocity (V). Ataxia was evaluated by use of the Scale for Assessment and Rating of Ataxia (SARA).SCA1 + exhibited significantly worse quality of stance with EC vs. SCA1- (p < 0.05 for V) and HCs (p < 0.001) even 5 to 6 years before estimated disease onset. There were no statistically significant differences between SCA1- and HCs. A slow increase in Cohen's d effect size was observed for V<sub>EO</sub> up to the clinical manifestation of ataxia. V<sub>EO</sub> and A<sub>EC</sub> recorded in preclinical SCA1 individuals correlated slightly but statistically significantly with SARA (r = 0.47).The study confirms that static posturography detects COP sway changes in SCA1 preclinical gene carriers even 5 to 6 years before estimated disease onset. The quantitative evaluation of stance in preclinical SCA is a sensitive biomarker for the monitoring of the disease progression and may be useful in clinical trials.</p>","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140141062","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Gap Junctions May Have A Computational Function In The Cerebellum: A Hypothesis. 小脑中的间隙连接可能具有计算功能:一个假设
IF 2.7 3区 医学
Cerebellum Pub Date : 2024-10-01 Epub Date: 2024-03-18 DOI: 10.1007/s12311-024-01680-3
Mike Gilbert, Anders Rasmussen
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