Acta neurologica Belgica最新文献_第9页

Giant cell vasculitis as a mimicker of cerebral amyloid angiopathy related inflammatory disease 巨细胞血管炎是脑淀粉样变性血管病相关炎症的模仿者。

IF 2 4区医学

Acta neurologica Belgica Pub Date : 2023-09-13 DOI: 10.1007/s13760-023-02376-x

Robin Stinissen, Martin Wyckmans, Anne Sieben, Tatjana Reynders, Caroline M. J. Loos

引用次数: 0

Corticospinal tract: a new hope for the treatment of post-stroke spasticity 皮质脊髓束：治疗中风后痉挛的新希望。

IF 2 4区医学

Acta neurologica Belgica Pub Date : 2023-09-13 DOI: 10.1007/s13760-023-02377-w

Linxing Huang, Lizhen Yi, Huiyuan Huang, Sheng Zhan, Ruixue Chen, Zenghui Yue

{"title":"Corticospinal tract: a new hope for the treatment of post-stroke spasticity","authors":"Linxing Huang, Lizhen Yi, Huiyuan Huang, Sheng Zhan, Ruixue Chen, Zenghui Yue","doi":"10.1007/s13760-023-02377-w","DOIUrl":"10.1007/s13760-023-02377-w","url":null,"abstract":"<div><p>Stroke is the third leading cause of death and disability worldwide. Post-stroke spasticity (PSS) is the most common complication of stroke but represents only one of the many manifestations of upper motor neuron syndrome. As an upper motor neuron, the corticospinal tract (CST) is the only direct descending motor pathway that innervates the spinal motor neurons and is closely related to the recovery of limb function in patients with PSS. Therefore, promoting axonal remodeling in the CST may help identify new therapeutic strategies for PSS. In this review, we outline the pathological mechanisms of PSS, specifically their relationship with CST, and therapeutic strategies for axonal regeneration of the CST after stroke. We found it to be closely associated with astroglial scarring produced by astrocyte activation and its secretion of neurotrophic factors, mainly after the onset of cerebral ischemia. We hope that this review offers insight into the relationship between CST and PSS and provides a basis for further studies.</p></div>","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":"124 1","pages":"25 - 36"},"PeriodicalIF":2.0,"publicationDate":"2023-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10874326/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10233331","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"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

Spectrum of various CNS inflammatory demyelination diseases following COVID-19 vaccinations 接种 COVID-19 疫苗后各种中枢神经系统炎症性脱髓鞘疾病的分布。

IF 2 4区医学

Acta neurologica Belgica Pub Date : 2023-09-05 DOI: 10.1007/s13760-023-02373-0

Manish Salunkhe, Kamlesh Tayade, Megha Priyadarshi, Vinay Goel, Isha Gulati, Ajay Garg, Rohit Bhatia, M. V. Padma Srivastava

{"title":"Spectrum of various CNS inflammatory demyelination diseases following COVID-19 vaccinations","authors":"Manish Salunkhe, Kamlesh Tayade, Megha Priyadarshi, Vinay Goel, Isha Gulati, Ajay Garg, Rohit Bhatia, M. V. Padma Srivastava","doi":"10.1007/s13760-023-02373-0","DOIUrl":"10.1007/s13760-023-02373-0","url":null,"abstract":"<div><h3>Background and purpose</h3><p>Although rare, neurological adverse events have been reported post-COVID-19 vaccination. This study reports 16 patients diagnosed with CNS inflammatory demyelinating diseases (CNS-IDD) within 6 weeks of COVID-19 vaccine administration.</p><h3>Methodology</h3><p>A prospective observational study was conducted from June 2021 to May 2022. All patients were diagnosed according to the latest international guidelines with CNS-IDD within 6 weeks of COVID-19 vaccine exposure. Data regarding the demographic profile, clinical features, type of COVID-19 vaccination, radiological findings and occurrence of symptoms were noted and further analysed using descriptive statistics.</p><h3>Results</h3><p>We reported 16 cases (median age 40 years) of CNS demyelination: fourteen occurred in temporal association with ChAdOx1-S vaccine and two in association with BBV152 vaccine. Median time duration of presenting symptoms after vaccination was 19 days (3–40 days). The most common presentation was myelitis (7/16 patients), followed by optic neuritis (6/16 patients). Demyelination events were reported after first and second dose in thirteen and five patients respectively, although two patients reported such events after both vaccine dosages. Myelin oligodendrocyte glycoprotein (MOG) IgG antibodies were positive in eight patients. Tumefactive demyelination was seen in four patients. Management included high-dose methylprednisolone, PLEX, IVIG or a combination of those, with a favourable outcome in the majority of cases.</p><h3>Conclusion</h3><p>Although a rare event, awareness regarding potential demyelinating episodes post-COVID-19 vaccination can help in early diagnosis. The presence of increased MOG-IgG antibodies with temporal association in post-COVID vaccine patients raises a possibility of an immunogenic phenomenon leading to demyelinating disorders.</p></div>","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":"124 1","pages":"193 - 203"},"PeriodicalIF":2.0,"publicationDate":"2023-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10527729","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 novel DCTN1 mutation causing perry syndrome leads to abnormal splicing of mRNA: genetic and functional analyses 导致佩里综合征的新型 DCTN1 突变导致 mRNA 剪接异常：遗传和功能分析。

IF 2 4区医学

Acta neurologica Belgica Pub Date : 2023-09-05 DOI: 10.1007/s13760-023-02368-x

Wotu Tian, Li Yao, Guochao Shi, Ranran Dai, Li Cao

引用次数: 0

The utility of heart-type free fatty-acid binding-protein (HFABP) levels for differentiating acute ischemic stroke from stroke mimics 心脏型游离脂肪酸结合蛋白（HFABP）水平在区分急性缺血性中风和中风模拟物方面的作用。

IF 2 4区医学

Acta neurologica Belgica Pub Date : 2023-09-04 DOI: 10.1007/s13760-023-02372-1

Emir Unal, Haldun Akoglu, Erkman Sanri, Sinan Karacabey, Melis Efeoglu Sacak, Ozge Onur, Arzu Denizbasi

{"title":"The utility of heart-type free fatty-acid binding-protein (HFABP) levels for differentiating acute ischemic stroke from stroke mimics","authors":"Emir Unal, Haldun Akoglu, Erkman Sanri, Sinan Karacabey, Melis Efeoglu Sacak, Ozge Onur, Arzu Denizbasi","doi":"10.1007/s13760-023-02372-1","DOIUrl":"10.1007/s13760-023-02372-1","url":null,"abstract":"<div><h3>Background</h3><p>Heart-type fatty acid-binding protein (HFABP) is found in the myocardium, brain, and some organs and is rapidly released from damaged cells into the circulation in case of ischemia.</p><h3>Aims</h3><p>We aimed to determine the diagnostic utility of HFABP levels in patients suggesting acute ischemic stroke (AIS).</p><h3>Methods</h3><p>This study was a prospective, single-center, observational diagnostic accuracy study with a nested cohort design. The estimated sample size was 126 patients, with a 1:1 case and control ratio. We included all consecutive patients with a lateralizing symptom (motor or sensory) or finding suggesting AIS (139 patients) who presented to ED within 24 h of their symptom onset and collected plasma at admission to the ED. After further evaluations, 111 patients (79.8%) were diagnosed with AIS and 28 with other neurological diseases (stroke-mimics).</p><h3>Findings</h3><p>In our study, the median HFABP levels of the cases and controls were 2.6 μg/ml and 2.2 μg/ml, respectively, without any statistically significant difference (<i>p</i> = 0.120). The diagnostic accuracy of HFABP for AIS was also insignificant at 0.60 (95% CI 0.51–0.68; <i>p</i> = 0.119).</p><h3>Discussion</h3><p>Plasma HFABP level is not a marker that can differentiate AIS from other neurological pathologies in patients presenting to the ED, with findings suggesting AIS.</p></div>","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":"124 1","pages":"183 - 191"},"PeriodicalIF":2.0,"publicationDate":"2023-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10144582","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

Genetic, serological and clinical evaluation of childhood myasthenia syndromes- single center subgroup analysis experience in Turkey 儿童肌无力综合征的遗传、血清学和临床评价——土耳其单中心亚组分析经验

IF 2.7 4区医学

Acta neurologica Belgica Pub Date : 2023-09-01 DOI: 10.1007/s13760-023-02370-3

Özlem Özsoy, Tayfun Cinleti, Çağatay Günay, Gamze Sarıkaya Uzan, Özlem Giray Bozkaya, Ahmet Okay Çağlayan, Semra Hız Kurul, Uluç Yiş

{"title":"Genetic, serological and clinical evaluation of childhood myasthenia syndromes- single center subgroup analysis experience in Turkey","authors":"Özlem Özsoy, Tayfun Cinleti, Çağatay Günay, Gamze Sarıkaya Uzan, Özlem Giray Bozkaya, Ahmet Okay Çağlayan, Semra Hız Kurul, Uluç Yiş","doi":"10.1007/s13760-023-02370-3","DOIUrl":"10.1007/s13760-023-02370-3","url":null,"abstract":"<div><h3>Background</h3><p>Congenital myasthenic syndrome is a disease that occurs due to several types such as mutations in different pre-synaptic, synaptic, post-synaptic proteins and, glycosylation defects associated with congenital myopathy. Juvenile myasthenia gravis is an autoimmune condition usually caused by antibodies targeting the acetylcholine receptor.</p><h3>Aims</h3><p>Our objective is to conduct an analysis on the subgroup traits exhibited by patients who have been diagnosed with congenital myasthenic syndrome and juvenile myasthenia gravis, with a focus on their long-term monitoring and management.</p><h3>Methods</h3><p>This study was conducted on children diagnosed with myasthenia gravis, who were under the care of Dokuz Eylul University’s Department of Pediatric Neurology for a period of ten years.</p><h3>Results</h3><p>A total of 22 (12 congenital myasthenic syndrome, 10 juvenile myasthenia gravis) patients were identified. Defects in the acetylcholine receptor (6/12) were the most common type in the congenital myasthenic syndrome group. Basal-lamina-related defects (5/12) were the second most prevalent. One patient had a <i>GFPT1</i> gene mutation (1/12). Patients with ocular myasthenia gravis (<i>n</i> = 6) exhibited milder symptoms. In the generalized myasthenia gravis group (<i>n</i> = 4), specifically in postpubertal girls, a more severe clinical progression was observed, leading to the implementation of more aggressive treatment strategies.</p><h3>Conclusion</h3><p>This study highlights that clinical recognition of congenital myasthenic syndrome and knowledge of related genes will aid the rapid diagnosis and treatment of these rare neuromuscular disorders. Findings in the juvenile myasthenia gravis group demonstrate the impact of pubertal development and the need for timely and appropriate active therapy, including thymectomy, to improve prognosis.</p></div>","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":"123 6","pages":"2325 - 2335"},"PeriodicalIF":2.7,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10503054","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

Long-term efficacy and safety of leflunomide combined with low-dose prednisone in treatment of myasthenia gravis: a retrospective study 来氟米特联合小剂量泼尼松治疗重症肌无力的长期疗效和安全性：一项回顾性研究。

IF 2 4区医学

Acta neurologica Belgica Pub Date : 2023-09-01 DOI: 10.1007/s13760-023-02367-y

Li Qiu, Pei Chen, Changyi Ou, Juan Deng, Zhidong Huang, Zhongqiang Lin, Qian Ma, Xin Huang, Lu Yu, Hao Ran, Weibin Liu

{"title":"Long-term efficacy and safety of leflunomide combined with low-dose prednisone in treatment of myasthenia gravis: a retrospective study","authors":"Li Qiu, Pei Chen, Changyi Ou, Juan Deng, Zhidong Huang, Zhongqiang Lin, Qian Ma, Xin Huang, Lu Yu, Hao Ran, Weibin Liu","doi":"10.1007/s13760-023-02367-y","DOIUrl":"10.1007/s13760-023-02367-y","url":null,"abstract":"<div><h3>Background</h3><p>Leflunomide and low-dose prednisone (0.25 mg/kg/day) (LEF + Pred) rapidly improved the clinical symptoms of myasthenia gravis (MG) patients. Here, we aimed to analyze the long-term efficacy and safety of LEF + Pred in MG patients.</p><h3>Methods</h3><p>This retrospective cohort study enrolled MG patients treated with LEF + Pred in our center between 2012 and 2020. We reviewed all the MG patients continuously treated with LEF + Pred for more than 1 year. MG activities of daily living (MG-ADL) profile score and quantitative MG scale (QMG) score in each clinical follow-up visits were collected for the efficacy analysis. The laboratory testing results of MG patients, the relevant chief complain and physical examination results in each follow-up visits were collected for the safety evaluation.</p><h3>Results</h3><p>In total, 103 patients were examined. Effective treatment was achieved in 58.3% of patients after 1 month and in 88.4% after 12 months. Overall, 63 patients (61.2%) exhibited only minimal manifestations after 12 months of treatment. The average MG-ADL score decreased from 6.0 to 1.0, while the average QMG score decreased from 10.0 to 4.0. The decrease in MG-ADL and QMG scores of patients with generalized MG was more pronounced than those of the ocular MG patients. Patients with MG who had a thymectomy had a smaller decrease in MG-ADL and QMG scores than those who did not have a thymectomy. Sixteen adverse effects associated with LEF + Pred were observed; none was severe.</p><h3>Conclusions</h3><p>Long-term LEF + Pred therapy could considerably improve clinical symptoms in MG patients while being well tolerated with just few side effects.</p></div>","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":"124 1","pages":"175 - 182"},"PeriodicalIF":2.0,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10503051","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

Depression, anxiety, stress, and suicide risk in patients with functional seizures vs. those with epilepsy 功能性癫痫发作患者与癫痫患者的抑郁、焦虑、压力和自杀风险。

IF 2 4区医学

Acta neurologica Belgica Pub Date : 2023-08-29 DOI: 10.1007/s13760-023-02365-0

Ali A. Asadi-Pooya, Mohsen Farazdaghi, Hanieh Asadi-Pooya, Khatereh Fazelian

{"title":"Depression, anxiety, stress, and suicide risk in patients with functional seizures vs. those with epilepsy","authors":"Ali A. Asadi-Pooya, Mohsen Farazdaghi, Hanieh Asadi-Pooya, Khatereh Fazelian","doi":"10.1007/s13760-023-02365-0","DOIUrl":"10.1007/s13760-023-02365-0","url":null,"abstract":"<div><h3>Background</h3><p>We aimed to investigate the rates of positive screening for depression, anxiety, stress, and suicide risk in adults with seizures [i.e., well-matched groups of patients with focal epilepsy <i>vs.</i> idiopathic generalized epilepsy (IGE) <i>vs.</i> functional seizures (FS)].</p><h3>Methods</h3><p>This was a cross sectional study. Patients, 19–55 years of age, with a diagnosis of IGE, focal epilepsy or FS were investigated at the outpatient epilepsy clinic at Shiraz University of Medical Sciences, Shiraz, Iran, from September 2022 until January 2023 and during their follow-up visits. We used the validated Farsi version of DASS-21 (Depression-Anxiety-Stress Scale) to investigate and screen for depression, anxiety, and stress in these patients. We also used the Beck Scale for Suicide Ideation (BSSI).</p><h3>Results</h3><p>Forty patients with focal epilepsy, 40 persons with IGE, and 40 individuals with FS were included. Depression and anxiety were more prevalent among patients with FS compared with those with epilepsy. The rate of stress among patients with FS was not significantly different compared with that in patients with epilepsy. The suicide risks were not significantly different between the groups either.</p><h3>Conclusion</h3><p>Patients with FS are at high risk for psychiatric comorbidities that is comparable or even worse than that in patients with epilepsy. Specific validated scales to screen for psychiatric comorbidities and suicide risk should be integral components of the evaluation and treatment of all patients with seizures.</p></div>","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":"124 1","pages":"169 - 173"},"PeriodicalIF":2.0,"publicationDate":"2023-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10103244","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

Human herpesvirus meningitis type 7 combined with neuromyelitis optica spectrum disorders: a case report 人类疱疹病毒 7 型脑膜炎合并神经脊髓炎谱系障碍：病例报告。

IF 2 4区医学

Acta neurologica Belgica Pub Date : 2023-08-28 DOI: 10.1007/s13760-023-02364-1

Wensheng Zhang, Weifang Xing, Yunqiang Huang, JinZhao He, Li Ling

引用次数: 0

A DLB patient with complicated CAA interruptions and intermediate alleles of 43 CAG/CAA repeats in TBP 一名患有复杂 CAA 中断和 TBP 中 43 个 CAG/CAA 重复序列中间等位基因的 DLB 患者。

IF 2 4区医学

Acta neurologica Belgica Pub Date : 2023-08-26 DOI: 10.1007/s13760-023-02351-6

Zhiru Lin, Jiaxiang Li, Wei Luo

引用次数: 0