Cerebellum最新文献

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Efficacy and Safety of Repetitive Transcranial Magnetic Stimulation in Spinocerebellar Ataxia Type 3: a Systematic Review and Meta‑analysis of Randomized Controlled Trials. 反复经颅磁刺激治疗脊髓小脑性共济失调3型的疗效和安全性:随机对照试验的系统评价和Meta分析
IF 2.7 3区 医学
Cerebellum Pub Date : 2024-08-01 Epub Date: 2023-11-17 DOI: 10.1007/s12311-023-01628-z
Mengqiu Qiu, Rui Wang, Yusha Shen, Zhenggang Hu, Yanbin Zhang
{"title":"Efficacy and Safety of Repetitive Transcranial Magnetic Stimulation in Spinocerebellar Ataxia Type 3: a Systematic Review and Meta‑analysis of Randomized Controlled Trials.","authors":"Mengqiu Qiu, Rui Wang, Yusha Shen, Zhenggang Hu, Yanbin Zhang","doi":"10.1007/s12311-023-01628-z","DOIUrl":"10.1007/s12311-023-01628-z","url":null,"abstract":"<p><p>Therapeutic alternatives for spinocerebellar ataxia type 3 (SCA3) are limited. Repetitive transcranial magnetic stimulation (rTMS) as a potential intervention has drawn heightened interest because of its ease of implementation, cost-effectiveness, and safety profile. We conducted a systematic review and meta-analysis to evaluate the efficacy of rTMS in the treatment of SCA3. We systematically searched databases-PubMed, Embase, the Cochrane Library, and Springer-for randomized controlled trials (RCTs) investigating the use of rTMS in the treatment of SCA3. Major efficacy outcomes were assessed, including International Cooperative Ataxia Rating Scale (ICARS) scores, Scale for the Assessment and Rating of Ataxia (SARA) scores, and ICARS subscale scores. Six randomized controlled trials involving 175 patients were included in the analysis. The meta-analysis results indicated statistically significant increases in ICARS (mean difference (MD) =  - 3.88, 95% confidence interval (CI) =  - 7.46 to - 0.30; p = 0.03) and SARA (MD of - 1.59, 95% CI - 2.99 to - 0.19; p = 0.03) scores. No significant heterogeneity was observed across all outcomes (I<sup>2</sup> = 0%). Dynamic function within the ICARS scale markedly improved with rTMS (MD =  - 2.19, 95% CI =  - 3.82 to - 0.55; p = 0.009). The majority of the included studies exhibited a low risk of bias, and no severe adverse reactions were noted. Our meta-analysis, consisting of six randomized controlled trials with 175 participants, suggests that rTMS exhibits efficacy in alleviating both ataxic symptoms and certain aspects of motor function in SCA3.</p>","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136400168","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
Amyotrophic Lateral Sclerosis with SOD1 Mutation Presenting with Progressive Cerebellar Ataxia. 肌萎缩性侧索硬化伴SOD1突变,表现为进行性小脑共济失调。
IF 2.7 3区 医学
Cerebellum Pub Date : 2024-08-01 Epub Date: 2023-12-05 DOI: 10.1007/s12311-023-01643-0
Marta Sequeira, Filipe Godinho, João Lourenço
{"title":"Amyotrophic Lateral Sclerosis with SOD1 Mutation Presenting with Progressive Cerebellar Ataxia.","authors":"Marta Sequeira, Filipe Godinho, João Lourenço","doi":"10.1007/s12311-023-01643-0","DOIUrl":"10.1007/s12311-023-01643-0","url":null,"abstract":"<p><p>Amyotrophic lateral sclerosis is a fatal neurodegenerative disorder that affects upper and lower motor neurons. SOD1 mutations are the second most commonly found in familial and sporadic cases. We describe a patient with a homozygous pathogenic mutation in SOD1 gene that presented with a progressive cerebellar ataxia and ultimately developed a complex phenotype of cerebellar ataxia and motor neuron disease. The linkage between the cerebellum and ALS is shortly discussed.</p>","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138483375","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
Temporal Relationship between Impairment of Cerebellar Motor Learning and Deterioration of Ataxia in Patients with Cerebellar Degeneration. 小脑变性患者小脑运动学习能力受损与共济失调恶化之间的时间关系
IF 2.7 3区 医学
Cerebellum Pub Date : 2024-08-01 Epub Date: 2023-04-28 DOI: 10.1007/s12311-023-01545-1
Takeru Honda, Ken Matsumura, Yuji Hashimoto, Takanori Yokota, Hidehiro Mizusawa, Soichi Nagao, Kinya Ishikawa
{"title":"Temporal Relationship between Impairment of Cerebellar Motor Learning and Deterioration of Ataxia in Patients with Cerebellar Degeneration.","authors":"Takeru Honda, Ken Matsumura, Yuji Hashimoto, Takanori Yokota, Hidehiro Mizusawa, Soichi Nagao, Kinya Ishikawa","doi":"10.1007/s12311-023-01545-1","DOIUrl":"10.1007/s12311-023-01545-1","url":null,"abstract":"<p><p>Ataxia and impaired motor learning are both fundamental features in diseases affecting the cerebellum. However, it remains unclarified whether motor learning is impaired only when ataxia clearly manifests, nor it is known whether the progression of ataxia, the speed of which often varies among patients with the same disease, can be monitored by examining motor learning. We evaluated motor learning and ataxia at intervals of several months in 40 patients with degenerative conditions [i.e., multiple system atrophy (MSA), Machado-Joseph disease (MJD)/spinocerebellar ataxia type 3 (SCA3), SCA6, and SCA31]. Motor learning was quantified as the adaptability index (AI) in the prism adaptation task and ataxia was scored using the Scale for the Assessment and Rating of Ataxia (SARA). We found that AI decreased most markedly in both MSA-C and MSA-P, moderately in MJD, and mildly in SCA6 and SCA31. Overall, the AI decrease occurred more rapidly than the SARA score increase. Interestingly, AIs remained normal in purely parkinsonian MSA-P patients (n = 4), but they dropped into the ataxia range when these patients started to show ataxia. The decrease in AI during follow-up (dAI/dt) was significant in patients with SARA scores < 10.5 compared with patients with SARA scores ≥ 10.5, indicating that AI is particularly useful for diagnosing the earlier phase of cerebellar degeneration. We conclude that AI is a useful marker for progressions of cerebellar diseases, and that evaluating the motor learning of patients can be particularly valuable for detecting cerebellar impairment, which is often masked by parkinsonisms and other signs.</p>","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11269492/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9362417","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 Gait and Balance Outcomes for Ataxia Trials: Consensus Recommendations by the Ataxia Global Initiative Working Group on Digital-Motor Biomarkers. 共济失调试验的定量步态和平衡结果:由共济失调全球倡议工作组关于数字运动生物标志物的共识建议。
IF 2.7 3区 医学
Cerebellum Pub Date : 2024-08-01 Epub Date: 2023-11-13 DOI: 10.1007/s12311-023-01625-2
Winfried Ilg, Sarah Milne, Tanja Schmitz-Hübsch, Lisa Alcock, Lukas Beichert, Enrico Bertini, Norlinah Mohamed Ibrahim, Helen Dawes, Christopher M Gomez, Hasmet Hanagasi, Kirsi M Kinnunen, Martina Minnerop, Andrea H Németh, Jane Newman, Yi Shiau Ng, Clara Rentz, Bedia Samanci, Vrutangkumar V Shah, Susanna Summa, Gessica Vasco, James McNames, Fay B Horak
{"title":"Quantitative Gait and Balance Outcomes for Ataxia Trials: Consensus Recommendations by the Ataxia Global Initiative Working Group on Digital-Motor Biomarkers.","authors":"Winfried Ilg, Sarah Milne, Tanja Schmitz-Hübsch, Lisa Alcock, Lukas Beichert, Enrico Bertini, Norlinah Mohamed Ibrahim, Helen Dawes, Christopher M Gomez, Hasmet Hanagasi, Kirsi M Kinnunen, Martina Minnerop, Andrea H Németh, Jane Newman, Yi Shiau Ng, Clara Rentz, Bedia Samanci, Vrutangkumar V Shah, Susanna Summa, Gessica Vasco, James McNames, Fay B Horak","doi":"10.1007/s12311-023-01625-2","DOIUrl":"10.1007/s12311-023-01625-2","url":null,"abstract":"<p><p>With disease-modifying drugs on the horizon for degenerative ataxias, ecologically valid, finely granulated, digital health measures are highly warranted to augment clinical and patient-reported outcome measures. Gait and balance disturbances most often present as the first signs of degenerative cerebellar ataxia and are the most reported disabling features in disease progression. Thus, digital gait and balance measures constitute promising and relevant performance outcomes for clinical trials.This narrative review with embedded consensus will describe evidence for the sensitivity of digital gait and balance measures for evaluating ataxia severity and progression, propose a consensus protocol for establishing gait and balance metrics in natural history studies and clinical trials, and discuss relevant issues for their use as performance outcomes.</p>","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11269489/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89720406","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
Exploring the Therapeutic Effects and Mechanisms of Transcranial Alternating Current Stimulation on Improving Walking Ability in Stroke Patients via Modulating Cerebellar Gamma Frequency Band-a Narrative Review. 探讨经颅交流电刺激通过调节小脑伽马频段改善脑卒中患者行走能力的疗效及机制。
IF 2.7 3区 医学
Cerebellum Pub Date : 2024-08-01 Epub Date: 2023-11-14 DOI: 10.1007/s12311-023-01632-3
Tingyi Feng, Lichao Zhang, Yuwei Wu, Lin Tang, Xixi Chen, Yuanli Li, Chunlei Shan
{"title":"Exploring the Therapeutic Effects and Mechanisms of Transcranial Alternating Current Stimulation on Improving Walking Ability in Stroke Patients via Modulating Cerebellar Gamma Frequency Band-a Narrative Review.","authors":"Tingyi Feng, Lichao Zhang, Yuwei Wu, Lin Tang, Xixi Chen, Yuanli Li, Chunlei Shan","doi":"10.1007/s12311-023-01632-3","DOIUrl":"10.1007/s12311-023-01632-3","url":null,"abstract":"<p><p>The cerebellum plays an important role in maintaining balance, posture control, muscle tone, and lower limb coordination in healthy individuals and stroke patients. At the same time, the relationship between cerebellum and motor learning has been widely concerned in recent years. Due to the relatively intact structure preservation and high plasticity after supratentorial stroke, non-invasive neuromodulation targeting the cerebellum is increasingly used to treat abnormal gait in stroke patients. The gamma frequency of transcranial alternating current stimulation (tACS) is commonly used to improve motor learning. It is an essential endogenous EEG oscillation in the gamma range during the swing phase, and rhythmic movement changes in the gait cycle. However, the effect of cerebellar tACS in the gamma frequency band on balance and walking after stroke remains unknown and requires further investigation.</p>","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11269344/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"92157256","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
P300 Wave Alterations and Cognitive Impairment in Cerebellum Lesions. 小脑损伤的 P300 波变化和认知障碍
IF 2.7 3区 医学
Cerebellum Pub Date : 2024-08-01 Epub Date: 2023-05-26 DOI: 10.1007/s12311-023-01570-0
Sourav Nanda, José Lapeña-Motilva, Amar Kumar Misra, Gautam Guha, Sinjan Ghosh, Akash Manna, Soumit Roy, Julián Benito-León
{"title":"P300 Wave Alterations and Cognitive Impairment in Cerebellum Lesions.","authors":"Sourav Nanda, José Lapeña-Motilva, Amar Kumar Misra, Gautam Guha, Sinjan Ghosh, Akash Manna, Soumit Roy, Julián Benito-León","doi":"10.1007/s12311-023-01570-0","DOIUrl":"10.1007/s12311-023-01570-0","url":null,"abstract":"<p><p>Patients with cognitive deficits have a prolonged latency and reduced amplitude of the P300 wave. However, no study has correlated P300 wave alterations with the cognitive performance of patients with cerebellar lesions. We aimed to determine if the cognitive status of these patients was associated with P300 wave alterations. We recruited 30 patients with cerebellar lesions from the wards of the N.R.S. Medical College, Kolkata, in West Bengal (India). The Kolkata Cognitive Screening Battery tasks and the Frontal Assessment Battery (FAB) were used to assess the cognitive status and the International Cooperative Ataxia Rating Scale (ICARS) for cerebellar signs. We compared the results with the normative data of the Indian population. Patients had P300 wave alterations with a significant increase in latency and a non-significant trend in amplitude. In a multivariate model, P300 wave latency was positively associated with the ICARS kinetic subscale (p = 0.005) and age (p = 0.009), regardless of sex and years of education. In the model that included cognitive variables, P300 wave latency was negatively associated with performance in phonemic fluency (p = 0.035) and construction (p = 0.009). Furthermore, P300 wave amplitude was positively associated with the FAB total score (p < 0.001). In closing, patients with cerebellar lesions had an increase in latency and a decrease in the amplitude of the P300 wave. These P300 wave alterations were also associated with worse cognitive performance and some of the subscales of the ICARS, reinforcing that the cerebellum has motor, cognitive, and affective functions.</p>","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11269376/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9515147","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
Ablation of Projection Glutamatergic Neurons in the Lateral Cerebellar Nuclei Alters Motor Coordination in Vglut2-Cre+ Mice. 消融小脑外侧核的谷氨酸能神经元会改变 Vglut2-Cre+ 小鼠的运动协调能力
IF 2.7 3区 医学
Cerebellum Pub Date : 2024-08-01 Epub Date: 2023-06-08 DOI: 10.1007/s12311-023-01575-9
Azam Asemi-Rad, Farshid Ghiyamihoor, G Giacomo Consalez, Hassan Marzban
{"title":"Ablation of Projection Glutamatergic Neurons in the Lateral Cerebellar Nuclei Alters Motor Coordination in Vglut2-Cre+ Mice.","authors":"Azam Asemi-Rad, Farshid Ghiyamihoor, G Giacomo Consalez, Hassan Marzban","doi":"10.1007/s12311-023-01575-9","DOIUrl":"10.1007/s12311-023-01575-9","url":null,"abstract":"<p><p>Cerebellar nuclei (CN) constitute the sole cerebellar output to the rest of the central nervous system and play a central role in cerebellar circuits. Accumulating evidence from both human genetics and animal studies point to a crucial role for CN connectivity in neurological diseases, including several types of ataxia. However, because of the compact and restricted topography and close functional connection between the CN and the cerebellar cortex, identifying cerebellar deficits exclusively linked to CN is challenging. In this study, we have experimentally ablated large projection glutamatergic neurons of the lateral CN and evaluated the impact of this selective manipulation on motor coordination in mice. To this end, through stereotaxic surgery, we injected the lateral CN of Vglut2-Cre+ mice with an adeno-associated virus (AAV) encoding a Cre-dependent diphtheria toxin receptor (DTR), followed by an intraperitoneal injection of diphtheria toxin (DT) to ablate the glutamatergic neurons of the lateral nucleus. Double immunostaining of cerebellar sections with anti-SMI32 and -GFP antibodies revealed GFP expression and provided evidence of SMI32+ neuron degeneration at the site of AAV injection in the lateral nucleus of Vglut2-Cre+ mice. No changes were observed in Vglut2-Cre negative mice. Analysis of motor coordination by rotarod test indicated that the latency to fall was significantly different before and after AAV/DT injection in the Vglut2-Cre+ group. Elapsed time and number of steps in the beam walking test were significantly higher in AAV/DT injected Vglut2-Cre+ AAV/DT mice compared to controls. We demonstrate for the first time that partial degeneration of glutamatergic neurons in the lateral CN is sufficient to induce an ataxic phenotype.</p>","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9596301","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
Efficacy of Cerebellar Transcranial Magnetic Stimulation for Post-stroke Balance and Limb Motor Function Impairments: Meta-analyses of Random Controlled Trials and Resting-State fMRI Studies. 小脑经颅磁刺激对中风后平衡和肢体运动功能障碍的疗效:随机对照试验和静息状态 fMRI 研究的元分析》。
IF 2.7 3区 医学
Cerebellum Pub Date : 2024-08-01 Epub Date: 2024-01-27 DOI: 10.1007/s12311-024-01660-7
Yuheng Zeng, Zujuan Ye, Wanxin Zheng, Jue Wang
{"title":"Efficacy of Cerebellar Transcranial Magnetic Stimulation for Post-stroke Balance and Limb Motor Function Impairments: Meta-analyses of Random Controlled Trials and Resting-State fMRI Studies.","authors":"Yuheng Zeng, Zujuan Ye, Wanxin Zheng, Jue Wang","doi":"10.1007/s12311-024-01660-7","DOIUrl":"10.1007/s12311-024-01660-7","url":null,"abstract":"<p><p>This study aimed to investigate the potential therapeutic effects of cerebellar transcranial magnetic stimulation (TMS) on balance and limb motor impairments in stroke patients. A meta-analysis of randomized controlled trials was conducted to assess the effects of cerebellar TMS on balance and motor impairments in stroke patients. Additionally, an activation likelihood estimation (ALE) meta-analysis was performed on resting-state functional magnetic resonance imaging (fMRI) studies to compare spontaneous neural activity differences between stroke patients and healthy controls using measures including the amplitude of low frequency fluctuation (ALFF), fractional ALFF (fALFF), and regional homogeneity (ReHo). The analysis included 10 cerebellar TMS studies and 18 fMRI studies. Cerebellar TMS treatment demonstrated significant improvements in the Berg Balance Scale score (p < 0.0001) and the Fugl-Meyer Assessment lower extremity score (p < 0.0001) compared to the control group in stroke patients. Additionally, spontaneous neural activity alterations were identified in motor-related regions after stroke, including the precentral gyrus, putamen, thalamus, and paracentral lobule. Cerebellar TMS shows promise as a therapeutic intervention to enhance balance and lower limb motor function in stroke patients. It is easy for clinical application and addresses the limitations of insufficient direct stimulation depth on the leg area of the cortex. However, further research combining neuroimaging outcomes with clinical measurements is necessary to validate these findings.</p>","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139572036","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
Syndrome of Irreversible Lithium-Effectuated Neurotoxicity (SILENT): A Preventable Cerebellar Disorder. 不可逆锂效应神经毒性综合征(SILENT):可预防的小脑疾病。
IF 2.7 3区 医学
Cerebellum Pub Date : 2024-08-01 Epub Date: 2024-02-07 DOI: 10.1007/s12311-024-01668-z
Sarah Marmol, Nestor Beltre, Jason Margolesky
{"title":"Syndrome of Irreversible Lithium-Effectuated Neurotoxicity (SILENT): A Preventable Cerebellar Disorder.","authors":"Sarah Marmol, Nestor Beltre, Jason Margolesky","doi":"10.1007/s12311-024-01668-z","DOIUrl":"10.1007/s12311-024-01668-z","url":null,"abstract":"<p><p>We report a case study of a 60-year-old man with bipolar disorder on stable lithium treatment who developed severe toxicity while admitted to ICU with sepsis and multiorgan failure. Despite unchanged lithium administration, his serum levels escalated due to renal dysfunction, resulting in lithium toxicity. After regaining consciousness, he exhibited a cerebellar syndrome marked by ataxia, tremor, and scanning speech. MRI revealed cerebellar atrophy. Following discontinuation of lithium and hemodialysis, the patient's symptoms remained static. The patient was diagnosed with syndrome of irreversible lithium-effectuated neurotoxicity (SILENT), a chronic cerebellar disorder characterized by persistent ataxia, nystagmus, and gait abnormalities extending beyond two months post-lithium exposure. The disorder has a predilection for cerebellar and basal ganglia dysfunction. MRI findings include cerebellar gliosis and atrophy and leptomeningeal enhancement. This case report highlights that SILENT is both preventable and permanent, urging heightened awareness among clinicians to facilitate early detection and intervention. Patients on lithium with compromised renal function or fever necessitate vigilant lithium level monitoring, dose adjustment, or cessation, to forestall enduring morbidity. This case emphasizes the significance of recognizing and managing SILENT, particularly in critical care settings, to mitigate long-term cerebellar impairment and optimize patient outcomes.</p>","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139698828","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 Use of the Voice Trainer App for Vocal Control in People with a Degenerative Ataxia: A Pilot Intervention Study. 变性共济失调患者使用语音训练器应用程序控制发声:试点干预研究
IF 2.7 3区 医学
Cerebellum Pub Date : 2024-08-01 Epub Date: 2024-01-29 DOI: 10.1007/s12311-024-01662-5
S Knuijt, J Nonnekes, B P C van de Warrenburg, M Nijkamp, S Scholten, B J M de Swart, J G Kalf
{"title":"The Use of the Voice Trainer App for Vocal Control in People with a Degenerative Ataxia: A Pilot Intervention Study.","authors":"S Knuijt, J Nonnekes, B P C van de Warrenburg, M Nijkamp, S Scholten, B J M de Swart, J G Kalf","doi":"10.1007/s12311-024-01662-5","DOIUrl":"10.1007/s12311-024-01662-5","url":null,"abstract":"<p><p>Dysarthria is disabling in persons with degenerative ataxia. There is limited evidence for speech therapy interventions. In this pilot study, we used the Voice trainer app, which was originally developed for patients with Parkinson's disease, as a feedback tool for vocal control. We hypothesized that patients with ataxic dysarthria would benefit from the Voice trainer app to better control their loudness and pitch, resulting in a lower speaking rate and better intelligibility. This intervention study consisted of five therapy sessions of 30 min within 3 weeks using the principles of the Pitch Limiting Voice Treatment. Patients received real-time visual feedback on loudness and pitch during the exercises. Besides, they were encouraged to practice at home or to use the Voice trainer in daily life. We used observer-rated and patient-rated outcome measures. The primary outcome measure was intelligibility, as measured by the Dutch sentence intelligibility test. Twenty-one out of 25 included patients with degenerative ataxia completed the therapy. We found no statistically significant improvements in intelligibility (p = .56). However, after the intervention, patients were speaking slower (p = .03) and the pause durations were longer (p < .001). The patients were satisfied about using the app. At the group level, we found no evidence for an effect of the Voice trainer app on intelligibility in degenerative ataxia. Because of the heterogeneity of ataxic dysarthria, a more tailor-made rather than generic intervention seems warranted.</p>","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11269506/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139571998","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
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