Cerebellum最新文献_第9页

The Neuroimmune System and the Cerebellum. 神经免疫系统和小脑。

IF 2.7 3区医学

Cerebellum Pub Date : 2024-12-01 Epub Date: 2023-11-10 DOI: 10.1007/s12311-023-01624-3

Donna L Gruol

{"title":"The Neuroimmune System and the Cerebellum.","authors":"Donna L Gruol","doi":"10.1007/s12311-023-01624-3","DOIUrl":"10.1007/s12311-023-01624-3","url":null,"abstract":"The recognition that there is an innate immune system of the brain, referred to as the neuroimmune system, that preforms many functions comparable to that of the peripheral immune system is a relatively new concept and much is yet to be learned. The main cellular components of the neuroimmune system are the glial cells of the brain, primarily microglia and astrocytes. These cell types preform many functions through secretion of signaling factors initially known as immune factors but referred to as neuroimmune factors when produced by cells of the brain. The immune functions of glial cells play critical roles in the healthy brain to maintain homeostasis that is essential for normal brain function, to establish cytoarchitecture of the brain during development, and, in pathological conditions, to minimize the detrimental effects of disease and injury and promote repair of brain structure and function. However, dysregulation of this system can occur resulting in actions that exacerbate or perpetuate the detrimental effects of disease or injury. The neuroimmune system extends throughout all brain regions, but attention to the cerebellar system has lagged that of other brain regions and information is limited on this topic. This article is meant to provide a brief introduction to the cellular and molecular components of the brain immune system, its functions, and what is known about its role in the cerebellum. The majority of this information comes from studies of animal models and pathological conditions, where upregulation of the system facilitates investigation of its actions.","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":" ","pages":"2511-2537"},"PeriodicalIF":2.7,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11585519/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72211768","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

Cerebellar Roles in Motor and Social Functions and Implications for ASD. 小脑在运动和社交功能中的作用及其对 ASD 的影响。

IF 2.7 3区医学

Cerebellum Pub Date : 2024-12-01 Epub Date: 2024-07-17 DOI: 10.1007/s12311-024-01720-y

Azhagu Madhavan Sivalingam, Arjun Pandian

{"title":"Cerebellar Roles in Motor and Social Functions and Implications for ASD.","authors":"Azhagu Madhavan Sivalingam, Arjun Pandian","doi":"10.1007/s12311-024-01720-y","DOIUrl":"10.1007/s12311-024-01720-y","url":null,"abstract":"The cerebellum, traditionally linked to voluntary motor coordination, is now recognized for its role in nonmotor functions, including cognitive and social behaviors. This expanded understanding is vital for identifying neurodevelopmental disorders such as autism spectrum disorder (ASD), where cerebellar abnormalities are common. Recent research has identified specific cerebellar circuits contributing to these diverse functions, revealing interconnected pathways that regulate both motor and social behaviors. The cerebellum communicates extensively with the cerebral cortex, thalamus, and limbic structures through converging and diverging pathways, integrating sensory and motor information to fine-tune outputs and influence higher-order functions. Mouse models have been instrumental in dissecting cerebellar functions, with studies using genetic and neuroanatomical techniques to manipulate specific circuits and observe behavioral outcomes. Disruptions in cerebellar pathways can lead to motor deficits and social impairments, mirroring human neurodevelopmental disorders. This review explores the anatomical and functional organization of cerebellar pathways in mice, their role in behavior, and the implications of cerebellar dysfunction in disorders such as ASD. Understanding these pathways enhances knowledge of cerebellar contributions to behavior and informs therapeutic strategies for cerebellar and neurodevelopmental disorders, emphasizing the integral role of the cerebellum in motor and social functions.","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":" ","pages":"2564-2574"},"PeriodicalIF":2.7,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141629244","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

Cerebellar Contributions to Traumatic Autobiographical Memory in People with Post-Traumatic Stress Disorder. 小脑对创伤后应激障碍患者创伤自传体记忆的贡献

IF 2.7 3区医学

Cerebellum Pub Date : 2024-12-01 Epub Date: 2024-08-24 DOI: 10.1007/s12311-024-01731-9

Chrysanthi Blithikioti, Or Duek, Charles Gordon, John H Krystal, Ifat Levy, Ilan Harpaz-Rotem, Daniela Schiller, Ofer Perl

{"title":"Cerebellar Contributions to Traumatic Autobiographical Memory in People with Post-Traumatic Stress Disorder.","authors":"Chrysanthi Blithikioti, Or Duek, Charles Gordon, John H Krystal, Ifat Levy, Ilan Harpaz-Rotem, Daniela Schiller, Ofer Perl","doi":"10.1007/s12311-024-01731-9","DOIUrl":"10.1007/s12311-024-01731-9","url":null,"abstract":"Post-traumatic stress disorder (PTSD) is a debilitating mental health condition characterized by recurrent re-experiencing of traumatic events. Despite increasing evidence suggesting that the cerebellum is involved in PTSD pathophysiology, it remains unclear whether this involvement is related to symptoms directly resulting from previous trauma exposure, such as involuntary re-experiencing of the traumatic events, or reflects a broader cerebellar engagement in negative affective states. In this study, we investigated the specific role of the cerebellum in PTSD by employing a script reactivation paradigm with personalized traumatic and sad autobiographical memories in 28 individuals diagnosed with chronic PTSD. Functional magnetic resonance imaging (fMRI) data were collected while participants listened to their own autobiographical narratives recounted by a third person. Activation in the right cerebellar lobule VI was uniquely associated with traumatic autobiographical recall and was parametrically modulated by the severity of re-experiencing symptoms. In contrast, cerebellar Crus II showed increased activation during both traumatic and sad autobiographical recall, suggesting a broader involvement in processing negative emotions. Our findings highlight the unique contribution of the right cerebellar lobule VI in the processing of traumatic autobiographical memories, potentially through its engagement in low-level representation of sensory and emotional aspects of traumatic events.","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":" ","pages":"2332-2340"},"PeriodicalIF":2.7,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142047449","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

COVID-19-Associated Cerebellar Ataxia: A Case Series. COVID-19相关性小脑共济失调：病例系列。

IF 2.7 3区医学

Cerebellum Pub Date : 2024-12-01 Epub Date: 2024-07-10 DOI: 10.1007/s12311-024-01712-y

Mange Liu, Siyuan Fan, Chang Geng, Haitao Ren, Sixian Chen, Manqing Xie, Ruixue Cui, Hongzhi Guan

{"title":"COVID-19-Associated Cerebellar Ataxia: A Case Series.","authors":"Mange Liu, Siyuan Fan, Chang Geng, Haitao Ren, Sixian Chen, Manqing Xie, Ruixue Cui, Hongzhi Guan","doi":"10.1007/s12311-024-01712-y","DOIUrl":"10.1007/s12311-024-01712-y","url":null,"abstract":"COVID-19-associated cerebellar ataxia has rarely been reported and its clinical characteristics remain understudied. This study aims to report patients with COVID-19-associated cerebellar ataxia from our institution. COVID-19-associated cerebellar ataxia was diagnosed based on the prodromal COVID-19 infection and the exclusion of other causes. This study provides a summary of the patients' clinical presentations, neuroimaging features, and the results of anti-cerebellar antibody examinations. Our study included 11 patients and 4 were male. The median onset age was 38 years. Five patients also demonstrated signs of encephalopathy. Brain magnetic resonance imaging (MRI) was either unremarkable (n = 6) or showed bilateral cerebellar lesions (n = 5), which were typically transient, although brain atrophy could be observed later in the disease course. Anti-Homer-3 and anti-Yo antibodies were each detected in one patient, respectively. All patients received immunotherapy and nine improved. Compared with the late-onset group, individuals who exhibited ataxia earlier following COVID-19 onset (interval<5 days) were significantly younger [median age 18 (15.5-31) vs. 53.5 (44-64.8) years, p = 0.009] and more likely to present with encephalopathy (5/5 vs. 0/6, p = 0.002).They also experienced more severe symptoms [median modified Rankin scale (mRS) score at zenith 5 (5-5) vs. 2 (1.75-2.75), p = 0.017] and had a less favorable prognosis [median mRS score at the last follow-up 4 (2-5) vs. 1 (0-1.25), p = 0.009]. COVID-19-associated cerebellar ataxia can appear with encephalopathy. Brain MRI may show transient bilateral cerebellar lesions and brain atrophy later. Patients who exhibited ataxia earlier following COVID-19 were younger, had more severe symptoms and poorer outcomes.","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":" ","pages":"2237-2243"},"PeriodicalIF":2.7,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141565052","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

Phenotypic Spectrum and Natural History of Gillespie Syndrome. An Updated Literature Review with 2 New Cases. 吉莱斯皮综合征的表型谱和自然史。最新文献综述及两个新病例。

IF 2.7 3区医学

Cerebellum Pub Date : 2024-12-01 Epub Date: 2024-08-23 DOI: 10.1007/s12311-024-01733-7

Claudia Ciaccio, Matilde Taddei, Chiara Pantaleoni, Marina Grisoli, Daniela Di Bella, Stefania Magri, Franco Taroni, Stefano D'Arrigo

{"title":"Phenotypic Spectrum and Natural History of Gillespie Syndrome. An Updated Literature Review with 2 New Cases.","authors":"Claudia Ciaccio, Matilde Taddei, Chiara Pantaleoni, Marina Grisoli, Daniela Di Bella, Stefania Magri, Franco Taroni, Stefano D'Arrigo","doi":"10.1007/s12311-024-01733-7","DOIUrl":"10.1007/s12311-024-01733-7","url":null,"abstract":"Background: Gillespie syndrome is a rare disorder caused by pathogenic variants in ITPR1 gene and characterized by the typical association of cerebellar ataxia, bilateral aniridia and intellectual disability. Since its first description in 1965, less than 100 patients have been reported and only 30 with a molecular confirmation.Methods: We present two additional cases, both carrying a loss-of-function variant in the Gly2539 amino acid residue. We describe the clinical evolution of the patients, one of whom is now 17 years old, and discuss the updated phenotypic spectrum of the disorder.Results: The study gives an overview on the condition, allowing to confirm important data, such as an overall positive evolution of development (with some patient not presenting intellectual disability), a clinical stability of the neurological signs (regardless of a possible progression of cerebellar atrophy) and ocular aspects, and a low prevalence of general health comorbidities.Discussion: Data about development and the observation of middle-aged patients lend support to the view that Gillespie is to be considered a non-progressive cerebellar ataxia, making this concept a key point for both clinicians and therapists, and for the families.","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":" ","pages":"2655-2670"},"PeriodicalIF":2.7,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11585489/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142037638","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

De Novo GRID2 Variant as a Cause of Ataxia with Oculomotor Apraxia and Alpha-Fetoprotein Elevation. 导致共济失调伴眼运动障碍和甲胎蛋白升高的新GRID2变异体

IF 2.7 3区医学

Cerebellum Pub Date : 2024-12-01 Epub Date: 2024-09-23 DOI: 10.1007/s12311-024-01743-5

Jacopo Sartorelli, Lorena Travaglini, Vito Luigi Colona, Carlo Casali, Francesca Cumbo, Adele D'Amico, Daniela Longo, Antonio Novelli, Gessica Vasco, Enrico Bertini, Francesco Nicita

{"title":"De Novo GRID2 Variant as a Cause of Ataxia with Oculomotor Apraxia and Alpha-Fetoprotein Elevation.","authors":"Jacopo Sartorelli, Lorena Travaglini, Vito Luigi Colona, Carlo Casali, Francesca Cumbo, Adele D'Amico, Daniela Longo, Antonio Novelli, Gessica Vasco, Enrico Bertini, Francesco Nicita","doi":"10.1007/s12311-024-01743-5","DOIUrl":"10.1007/s12311-024-01743-5","url":null,"abstract":"Bi-allelic pathogenic variants in GRID2 have been initially associated to an autosomal recessive form of spinocerebellar ataxia, namely SCAR18. Subsequently, few monoallelic cases have been described. Here we present a new subject harboring a novel de novo heterozygous GRID2 missense variant presenting with progressive ataxia together with cerebellar atrophy and, for the first time, alpha-fetoprotein (AFP) elevation. We retrospectively collected data of the patient followed at our clinic. Genetic analysis was performed through clinical exome sequencing with an in-house in-silico ataxia-related genes panel. Variant effect prediction was performed through in silico modeling. The patient had normal psychomotor development except for mild fine and gross motor impairment. In adolescence, he started presenting dysarthria and progressive ataxia. Blood tests showed significant AFP elevation. Brain MRI showed cerebellar atrophy mainly involving the vermis. The novel de novo heterozygous GRID2 (c.1954C>A; p.Leu652Ile) missense variant was disclosed. This variant is located within a highly conserved site with low tolerance to variation and it is predicted to cause protein structure destabilization. GRID2 expression appears to be influenced by other genes related with ataxia and AFP elevation, like ATM and APTX, suggesting a possible shared mechanism. This additional patient increases the scarce literature and genotypic spectrum of the GRID2-related ataxia and evidences a fairly homogeneous phenotype of ataxia with oculomotor abnormalities for the autosomal-dominant form. Alfa-fetoprotein elevation is a novel finding in this condition and this data must be confirmed in larger case-series to definitively state that GRID2-related ataxia can be included among ataxias with AFP increase.","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":" ","pages":"2408-2413"},"PeriodicalIF":2.7,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142300080","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

Caffeine Consumption and Interaction with ADORA2A, CYP1A2 and NOS1 Variants Do Not Influence Age at Onset of Machado-Joseph Disease. 咖啡因摄入量及与 ADORA2A、CYP1A2 和 NOS1 变异的相互作用不会影响马查多-约瑟夫病的发病年龄。

IF 2.7 3区医学

Cerebellum Pub Date : 2024-12-01 Epub Date: 2024-07-06 DOI: 10.1007/s12311-024-01717-7

Ana Carolina Martins, Jordânia Dos Santos Pinheiro, Luciana Szinwelski, Eduardo Rockenbach Cidade, Danilo Fernando Santin, Laura Damke Proença, Bruna Almeida Araújo, Maria Luiza Saraiva-Pereira, Laura Bannach Jardim

{"title":"Caffeine Consumption and Interaction with ADORA2A, CYP1A2 and NOS1 Variants Do Not Influence Age at Onset of Machado-Joseph Disease.","authors":"Ana Carolina Martins, Jordânia Dos Santos Pinheiro, Luciana Szinwelski, Eduardo Rockenbach Cidade, Danilo Fernando Santin, Laura Damke Proença, Bruna Almeida Araújo, Maria Luiza Saraiva-Pereira, Laura Bannach Jardim","doi":"10.1007/s12311-024-01717-7","DOIUrl":"10.1007/s12311-024-01717-7","url":null,"abstract":"Background: The age at onset (AO) of Machado-Joseph disease (SCA3/MJD), a disorder due to an expanded CAG repeat (CAGexp) in ATXN3, is quite variable and the role of environmental factors is still unknown. Caffeine was associated with protective effects against other neurodegenerative diseases, and against SCA3/MJD in transgenic mouse models. We aimed to evaluate whether caffeine consumption and its interaction with variants of caffeine signaling/metabolization genes impact the AO of this disease.Methods: a questionnaire on caffeine consumption was applied to adult patients and unrelated controls living in Rio Grande do Sul, Brazil. AO and CAGexp were previously determined. SNPs rs5751876 (ADORA2A), rs2298383 (ADORA2A), rs762551 (CYP1A2) and rs478597 (NOS1) were genotyped. AO of subgroups were compared, adjusting the CAGexp to 75 repeats (p < 0.05).Results: 171/179 cases and 98/100 controls consumed caffeine. Cases with high and low caffeine consumption (more or less than 314.5 mg of caffeine/day) had mean (SD) AO of 35.05 (11.44) and 35.43 (10.08) years (p = 0.40). The mean (SD) AO of the subgroups produced by the presence or absence of caffeine-enhancing alleles in ADORA2A (T allele at rs5751876 and rs2298383), CYP1A2 (C allele) and NOS1 (C allele) were all similar (p between 0.069 and 0.516).Discussion: Caffeine consumption was not related to changes in the AO of SCA3/MJD, either alone or in interaction with protective genotypes at ADORA2A, CYP1A2 and NOS1.","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":" ","pages":"2217-2225"},"PeriodicalIF":2.7,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141538881","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 Role of Protein Quantity Control in Polyglutamine Spinocerebellar Ataxias. 多谷氨酰胺脊髓小脑共济失调症中蛋白质数量控制的作用

IF 2.7 3区医学

Cerebellum Pub Date : 2024-12-01 Epub Date: 2024-07-25 DOI: 10.1007/s12311-024-01722-w

Hongfeng Zhang, Xin Wang

引用次数: 0

Immune Checkpoint Inhibitor-Related Cerebellar Toxicity: Clinical Features and Comparison with Paraneoplastic Cerebellar Ataxia. 免疫检查点抑制剂相关的小脑毒性：临床特征及与副肿瘤性小脑共济失调的比较。

IF 2.7 3区医学

Cerebellum Pub Date : 2024-12-01 Epub Date: 2024-08-17 DOI: 10.1007/s12311-024-01727-5

Marta Dentoni, Irene Florean, Antonio Farina, Bastien Joubert, Le-Duy Do, Jérôme Honnorat, Valentina Damato, Martina Fabris, Gian Luigi Gigli, Mariarosaria Valente, Alberto Vogrig

{"title":"Immune Checkpoint Inhibitor-Related Cerebellar Toxicity: Clinical Features and Comparison with Paraneoplastic Cerebellar Ataxia.","authors":"Marta Dentoni, Irene Florean, Antonio Farina, Bastien Joubert, Le-Duy Do, Jérôme Honnorat, Valentina Damato, Martina Fabris, Gian Luigi Gigli, Mariarosaria Valente, Alberto Vogrig","doi":"10.1007/s12311-024-01727-5","DOIUrl":"10.1007/s12311-024-01727-5","url":null,"abstract":"Immune checkpoint inhibitors (ICIs) have revolutionized cancer therapy, and the association with immune-related adverse events (irAEs) is well-established. However, cerebellar irAEs are poorly defined and their relationship with paraneoplastic disorders remains unclear. Our aim was (i) to characterize cerebellar irAE; (ii) to compare it with paraneoplastic cerebellar ataxia (PCA). We performed a multicenter, retrospective, cohort study of patients developing new-onset, immune-mediated, isolated/predominant cerebellar dysfunction after ICI administration. In addition, a systematic review following PRISMA guidelines was performed. Cerebellar irAE cases were compared with a consecutive cohort of patients with PCA. Overall, 35 patients were included, of whom 12 were original cases (males: 25/35 (71%), median age: 65 [range: 20-82]). The most frequent tumor was non-small cell lung cancer (12/35, 34%). Anti-PD1 were adopted in 19/35 (54%). Symptoms developed at a median of 11 weeks after ICI onset. Neuronal antibodies were detected in 15/31 patients tested (48%). Cerebrospinal fluid was inflammatory in 25/30 (83%). Magnetic resonance imaging showed cerebellar hyperintensities in 8/35 (23%). Immunotherapy was applied in 33/35 cases (94%), and most patients improved with residual disability (16/35, 46%). When compared with a series of PCA (n = 15), the cerebellar irAE group was significantly more associated with male sex, lung cancer (rather than gynecological/breast cancers), isolated ataxia, and a better outcome. We provide a detailed characterization of cerebellar irAE. Compared to PCA, differences exist in terms of tumor association, clinical features, and outcome. Clinical presentation-antibody-tumor triad in the ICI group only partially reflects the associations described in paraneoplastic disorders.","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":" ","pages":"2308-2323"},"PeriodicalIF":2.7,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11585521/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141996879","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

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 : 2024-12-01 Epub 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":"10.1007/s12311-024-01744-4","url":null,"abstract":"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.","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":" ","pages":"2447-2456"},"PeriodicalIF":2.7,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11585490/pdf/","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":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0