Acta neurologica Belgica最新文献_第8页

Association between peripheral blood immunological status and intrathecal inflammatory markers differentiate multiple sclerosis clinical phenotypes. 外周血免疫状态与鞘内炎症标志物之间的关系可区分多发性硬化症的临床表型。

IF 2 4区医学

Acta neurologica Belgica Pub Date : 2024-08-03 DOI: 10.1007/s13760-024-02597-8

Ana Turčić, Josip Knežević, Ljiljana Zaninović, Mario Habek, Magdalena Krbot Skorić, Antonija Babić, Željka Vogrinc

{"title":"Association between peripheral blood immunological status and intrathecal inflammatory markers differentiate multiple sclerosis clinical phenotypes.","authors":"Ana Turčić, Josip Knežević, Ljiljana Zaninović, Mario Habek, Magdalena Krbot Skorić, Antonija Babić, Željka Vogrinc","doi":"10.1007/s13760-024-02597-8","DOIUrl":"https://doi.org/10.1007/s13760-024-02597-8","url":null,"abstract":"Background: The difference in the clinical course, response to therapy, and distribution of CNS inflammation in primary-progressive (PPMS) and relapsing-remitting multiple sclerosis (RRMS) suggests differences in the underlying immunological characteristics of the disease. We aimed to investigate differences in immunological profiles in relation to intrathecal inflammation in different MS forms.Methods: The peripheral blood (PB) proportions of CD4 + and CD8 + T-cells and CD19 + B-cells were retrospectively compared with the markers of intrathecal immunoglobulin G (IgG) synthesis at diagnosis: IgG index, percentage of intrathecal IgG synthesis (IF IgG), the number of oligoclonal bands (OCB), depending on the blood-brain barrier (BBB) function, and antibody specific index to neurotrophic viruses (MRZH reaction).Results: Thirty-six controls, 71 RRMS and 25 PPMS were enrolled. PPMS had higher percentage of CD4 + T-cells compared to RRMS (P = 0.043) and controls (P = 0.003). The percentage of CD8 + T-cells and CD19 + B-cells, and respective absolute cell counts did not differ according to the MS phenotype. In RRMS with the dysfunctional BBB, the IgG index (r = 0.642, P = 0.012) correlated significantly with the CD19 + B-cells while the CD4 + T-cells inversely correlated with IF IgG (r=-0.574, P = 0.039). Interestingly, in PPMS the number of OCB was positively associated with CD4+ (r = 0.603, P = 0.015) and negatively associated with CD8 + T-cells (r=-0.554, P = 0.033), while IF IgG negatively correlated with CD8 + T-cells (r=-0.689, P = 0.003), but only in the preserved BBB function.Conclusions: The PB CD4 + T-cells and B-cells were associated with the intrathecal inflammation in RRMS with BBB dysfunction while CD8 + T-cells were involved in PPMS with CNS-compartmentalized inflammation.","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-08-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141878206","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Pediatric case of a particularly large clear cell meningioma - a case report. 小儿特大透明细胞脑膜瘤--病例报告。

IF 2 4区医学

Acta neurologica Belgica Pub Date : 2024-08-03 DOI: 10.1007/s13760-024-02618-6

Markéta Češpivová, Martin Lammens, Dana Dumitriu, Herbert Rooijakkers, Lina Daoud

引用次数: 0

Sjögren syndrome and neuromyelitis optica spectrum disorder associated with early relapse after initial paucity of symptoms. 斯约格伦综合征和神经脊髓炎视网膜频谱障碍与最初症状轻微后早期复发有关。

IF 2 4区医学

Acta neurologica Belgica Pub Date : 2024-08-01 DOI: 10.1007/s13760-024-02614-w

G Magro, O Di Benedetto, G Fazio, M Trimboli

引用次数: 0

"If you cannot measure it, you cannot improve it". Outcome measures in Duchenne Muscular Dystrophy: current and future perspectives. 杜兴氏肌肉萎缩症的结果测量。

IF 2 4区医学

Acta neurologica Belgica Pub Date : 2024-07-31 DOI: 10.1007/s13760-024-02600-2

Silvia Benemei, Francesca Gatto, Luca Boni, Marika Pane

{"title":"\"If you cannot measure it, you cannot improve it\". Outcome measures in Duchenne Muscular Dystrophy: current and future perspectives.","authors":"Silvia Benemei, Francesca Gatto, Luca Boni, Marika Pane","doi":"10.1007/s13760-024-02600-2","DOIUrl":"10.1007/s13760-024-02600-2","url":null,"abstract":"Duchenne Muscular Dystrophy (DMD) is an X-linked recessive neuromuscular disorder primarily affecting males, caused by mutations in the dystrophin gene. The absence of dystrophin protein leads to progressive skeletal muscle degeneration. Recent advances in the therapeutic landscape underscore the need to identify appropriate outcome measures to assess treatment efficacy in ambulant and non-ambulant DMD patients, across clinical and research settings. This is essential for accurately evaluating new treatments and attributing therapeutic benefits.It is crucial to establish a robust correlation between outcome scores and disease progression patterns. This task is challenging since functional test performance may be influenced by different patient's characteristics, including the physiological evolution of the neurodevelopment together with the disease progression. While widely used DMD outcomes such as the North Star Ambulatory Assessment, the 6-Minute Walking Test, the 4 stairs climbed, and the Performance of the Upper Limb exhibit reliability and validity, their clinical significance is influenced by the wide phenotype and progression variability of the disease.We present and discuss the features (relevance, quantifiability, validity, objectivity, reliability, sensitivity, specificity, precision) of available DMD outcome measures, including new potential measures that may be provided by digital tools and artificial intelligence.","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141854513","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Hydrops regression after vestibular denervation - longitudinal magnetic resonance study in patients with severe Meniere's disease treated with vestibular neurectomy. 前庭神经支配术后水肿消退--对接受前庭神经切除术治疗的重度梅尼埃病患者进行的纵向磁共振研究。

IF 2 4区医学

Acta neurologica Belgica Pub Date : 2024-07-30 DOI: 10.1007/s13760-024-02605-x

Agnieszka Jasińska-Nowacka, Magdalena Lachowska, Emilia Wnuk, Kazimierz Niemczyk

{"title":"Hydrops regression after vestibular denervation - longitudinal magnetic resonance study in patients with severe Meniere's disease treated with vestibular neurectomy.","authors":"Agnieszka Jasińska-Nowacka, Magdalena Lachowska, Emilia Wnuk, Kazimierz Niemczyk","doi":"10.1007/s13760-024-02605-x","DOIUrl":"https://doi.org/10.1007/s13760-024-02605-x","url":null,"abstract":"Objectives: The aim was to evaluate endolymphatic hydrops in patients with severe Ménière's disease (MD) before and after vestibular neurectomy to verify if vestibular denervation results in hydrops regression.Methods: Magnetic resonance imaging was performed after intravenous gadolinium injection in twenty patients with unilateral definite MD before and after the vestibular neurectomy. Clinical symptoms and audiovestibular tests were evaluated. Follow-up intervals ranged from 18 to 35 months after the surgery.Results: Endolymphatic hydrops were visualized in all patients in the preoperative scans. After the vestibular neurectomy, all patients presented a complete resolution of vertigo episodes. Regression of the endolymphatic hydrops was observed in 35% and 15% of cases analyzing cochlea and vestibule, respectively. In 71.43% of patients with utricular herniation into the lateral semicircular canal, withdrawal of the hernia was visualized. Asymmetrical contrast enhancement in the cochlea regressed in 17.64% of cases. Analyzing all the parameters collectively, in 60% of patients, partial regression of at least one of the radiological signs was confirmed in the follow-up examination. No progression of the endolymphatic hydrops was visualized after the surgery in either the cochlea or the vestibule.Conclusions: Vestibular neurectomy is an effective treatment, eliminating vertigo attacks and improving the quality of life in patients with MD. Magnetic resonance imaging of the inner ear allows visualization of changes in endolymphatic hydrops degree after treatment. Regression of the endolymphatic hydrops after vestibular neurectomy suggests that vestibular denervation may effectively halt the progression of the endolymphatic space dilatation and result in hydrops regression.","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141791638","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

A case of mixed hereditary gelsolin amyloidosis and hydroxychloroquine induced myopathy. 一例混合型遗传性凝胶淀粉样变性病和羟氯喹诱发的肌病。

IF 2 4区医学

Acta neurologica Belgica Pub Date : 2024-07-29 DOI: 10.1007/s13760-024-02607-9

Gloria Mak, Mark Tarnopolsky, Jian-Qiang Lu

引用次数: 0

Association between levels of sex hormones and risk of multiple sclerosis: a mendelian randomization study. 性激素水平与多发性硬化症风险之间的关系：一项亡羊补牢式随机研究。

IF 2 4区医学

Acta neurologica Belgica Pub Date : 2024-07-29 DOI: 10.1007/s13760-024-02613-x

Chaofan Geng, Yi Tang

{"title":"Association between levels of sex hormones and risk of multiple sclerosis: a mendelian randomization study.","authors":"Chaofan Geng, Yi Tang","doi":"10.1007/s13760-024-02613-x","DOIUrl":"https://doi.org/10.1007/s13760-024-02613-x","url":null,"abstract":"Background: This research aimed to examine the causal connections between multiple sclerosis (MS) and a range of sex hormone-related traits, such as bioavailable testosterone (BT), sex hormone-binding globulin (SHBG), testosterone, and estradiol (E2).Methods: A bidirectional two-sample Mendelian randomization (MR) analysis using summary statistics from genome-wide association studies (GWAS) was conducted to investigate the relationship between sex hormone-related traits and MS. Moreover, the Inverse-variance weighted (IVW) method was employed as the primary analysis approach.Results: The MR analysis, using the IVW method, found a significant correlation between genetically determined SHBG levels and MS (OR = 1.634, 95% CI: 1.029-2.599, p = 0.038). Similarly, the reverse MR analysis suggested a causal link between MS and SHBG (OR = 1.005, 95% CI: 1.001-1.009, P = 0.003). However, no association was observed between MS risk and E2, testosterone, or BT levels.Conclusion: Our MR analysis demonstrated that genetically predicted higher SHBG may be positively correlated with the risk of MS. Moreover, the role of SHBG in MS could be further investigated.","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141787036","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

The relationship between work difficulties and physical disability, cognitive and social cognitive impairment and subcortical gray matter atrophy in persons with multiple sclerosis. 多发性硬化症患者的工作困难与身体残疾、认知和社会认知障碍以及皮层下灰质萎缩之间的关系。

IF 2 4区医学

Acta neurologica Belgica Pub Date : 2024-07-24 DOI: 10.1007/s13760-024-02609-7

Taha Aslan, Asiye Tuba Ozdogar, Ozge Sagici, Serkan Ozakbas

{"title":"The relationship between work difficulties and physical disability, cognitive and social cognitive impairment and subcortical gray matter atrophy in persons with multiple sclerosis.","authors":"Taha Aslan, Asiye Tuba Ozdogar, Ozge Sagici, Serkan Ozakbas","doi":"10.1007/s13760-024-02609-7","DOIUrl":"https://doi.org/10.1007/s13760-024-02609-7","url":null,"abstract":"Background: Multiple sclerosis (MS) is commonly associated with work difficulties. This study aimed to examine the relationship between work difficulties and physical disability, cognitive and social cognitive impairment, and subcortical gray matter (scGM) atrophy in pwMS.Methods: Thirty-three employees with MS underwent assessments with Multiple Sclerosis Work Difficulties Questionnaire-23 MSWDQ-23. Physical disability was measured using EDSS, Timed 25-Foot Walk (T25FW), 2-Minute Walking Test (2-MWT), the Nine-Hole Peg test (N-HPT), and 12-item Multiple Sclerosis Walking Scale (MSWS-12). Cognitive functions were evaluated with Brief International Cognitive Assessment in MS (BICAMS), social cognition with Facial Emotion Identification (FEI), Reading the Mind in the Eyes Test (RMET), and Empathy Quotient (EQ). Anxiety and depression were assessed using Hospital Anxiety and Depression Scale (HADS). The association between variables was analysed using Spearman's correlation coefficient. GM volumes were calculated from 3T MRI data using Freesurfer, their potential relationship with work difficulties were evaluated through a linear regression model.Results: MSWDQ-23 was strongly correlated with T25FW and MSWS-12 (p < 0.01), moderately correlated with EDSS, 2MWT, HAD, BICAMS, and EQ (p < 0.05). According to the linear regression model the decrease in volumes of total GM and scGM, bilateral Thalamus, bilateral Hippocampus, left Putamen, and right Caudate related with the severity of work difficulties (R²=0.815, p = 0.25).Conclusion: This study provides valuable insights into the multifaceted nature of work difficulties experienced by pwMS. It suggests that not only physical disability but also other factors, such as mood, cognition, empathy, and cortical and subcortical gray matter atrophy may contribute to work difficulties among pwMS.","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141750771","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Beneficial therapeutic plasma exchange response in the treatment of severe relapses in patients with multiple sclerosis. 治疗多发性硬化症严重复发患者的有益治疗性血浆置换反应。

IF 2 4区医学

Acta neurologica Belgica Pub Date : 2024-07-24 DOI: 10.1007/s13760-024-02606-w

Sarlota Mesaros, Tatjana Pekmezovic, Vanja Martinovic, Jovana Ivanovic, Olivera Tamas, Marija Dinic, Jelena Drulovic

{"title":"Beneficial therapeutic plasma exchange response in the treatment of severe relapses in patients with multiple sclerosis.","authors":"Sarlota Mesaros, Tatjana Pekmezovic, Vanja Martinovic, Jovana Ivanovic, Olivera Tamas, Marija Dinic, Jelena Drulovic","doi":"10.1007/s13760-024-02606-w","DOIUrl":"https://doi.org/10.1007/s13760-024-02606-w","url":null,"abstract":"Purpose: Therapeutic plasma exchange (PLEX) is effective as a second-line treatment of severe relapses of multiple sclerosis (MS) that failed to respond to standard steroid therapy. Our objective was to evaluate the effectiveness of PLEX in the severe MS relapses in a cohort of patients treated at Neurology Clinic, University Clinical Centre of Serbia, Belgrade, from 2007 until 2020.Methods: This retrospective study comprised 107 MS patients with 127 severe relapses treated with PLEX. Majority of our patients suffered from relapsing remitting MS (83.2%), 12.1% had secondary progressive MS and 4.7% had primary progressive MS. Mean age was 39.2 years (range, 19-79 years), female/male ratio 2.3:1. Pulse corticosteroid treatment was used before PLEX in 99.3% of patients. Median EDSS score at nadire during relapse was 6.0 (range 2.0-10.0). After PLEX, 73.8% relapses showed a marked clinical improvement, 7.1% showed mild improvement and in 19.0% there was no improvement. Median EDSS at discharge was 4.0 (6.0 at nadir of relapse vs. 4.0 at discharge; p<0.0001) and it was sustained at the same level, 6 month after PLEX. Multivariate regression analysis showed that higher EDSS at nadir during relapse (OR=0.63, 95% CI 0.41-0.96, p=0.039) and older age (OR=1.07, 95% CI 1.02- 1.12, p=0.010) were significantly associated with poor treatment response after 6- month follow-up. Adverse events occurred in 17.3 of procedures and they were completely resolved.Conclusion: Our study in a large cohort of MS patients confirmed that PLEX is effective.","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141750770","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Endovascular treatment of deep sinus vein thrombosis 72 h after initial cardioembolic stroke. 首次心脑血管栓塞中风 72 小时后的深静脉窦血栓形成的血管内治疗。

IF 2 4区医学

Acta neurologica Belgica Pub Date : 2024-07-24 DOI: 10.1007/s13760-024-02608-8

Thorleif Etgen, Andreas Mangold, Philip Hölter

引用次数: 0