Neurologia最新文献_第5页

Ischemic encephalopathic debut of CADASIL, a case report: It is better to be safe than sorry CADASIL缺血性脑病首发病例报告：安全胜于遗憾

Neurologia Pub Date : 2024-10-01 DOI: 10.1016/j.nrleng.2024.09.005

C. González-Mingot , A. Gil-Sánchez , R. Begué-Gómez , R. López-Ortega , B.R. Luis

引用次数: 0

Cerebral venous thrombosis in children an 18-year review of a Portuguese hospital 葡萄牙一家医院 18 年来的儿童脑静脉血栓病回顾

Neurologia Pub Date : 2024-10-01 DOI: 10.1016/j.nrleng.2024.09.002

J. Tenente , S. Lopes , P. Bem , M. Vila-Real , D. Ferreira , A.F. Geraldo , F. Santos

{"title":"Cerebral venous thrombosis in children an 18-year review of a Portuguese hospital","authors":"J. Tenente , S. Lopes , P. Bem , M. Vila-Real , D. Ferreira , A.F. Geraldo , F. Santos","doi":"10.1016/j.nrleng.2024.09.002","DOIUrl":"10.1016/j.nrleng.2024.09.002","url":null,"abstract":"<div><h3>Introduction</h3><div>Cerebral venous thrombosis (CVT) is an uncommon and clinically heterogeneous cerebrovascular particularly in children, only a few published case series focused in the pediatric population.</div></div><div><h3>Patients and methods</h3><div>Retrospective single-center observational and analytical study of consecutive pediatric patients admitted in a level II Portuguese hospital with a confirmed diagnosis of CVT, from 2003 to 2021. Clinical presentation, neuroimaging findings, prothrombotic factors, treatment strategies, outcome and recanalization were documented.</div></div><div><h3>Results</h3><div>Twelve children were included (58% female). Mean age was 7.3 years. The most frequent symptoms were vomiting, headache and behavioral alterations. Infection was the triggering factor in 50% of the cases. The diagnosis of CVT was made based on imaging evidence of thrombosis through magnetic imaging resonance (MRI) with venography and/or computed tomography (CT) with venography. In 67% of cases there were multiples sinuses involved; the transverse sinus was the most affected, followed by the sigmoid sinus. In 83% of cases anticoagulant therapy was initiated with low molecular weight heparin (LMWH) and associated prothrombotic factors were investigated, with no major prothrombotic factors identified. No deaths occurred, but 30% had long-term neurological sequelae. One patient recurred 18 years later.</div></div><div><h3>Conclusion</h3><div>The results of this study are consistent with data from other published studies. MRI is the preferred imaging method for diagnosis in children by avoiding ionizing radiation and allowing identification of subjacent causes. Anticoagulation with LMWH is recommended and important to reduce mortality and sequelae. Infectious diseases are the most common trigger for CVT and can also be the cause for high morbidity and poor outcomes.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"39 8","pages":"Pages 658-665"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142425825","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Psychometric properties of the Spanish version of the Frontal Assessment Battery (FAB-E) and normative values in a representative adult population sample 西班牙文版前额叶评估测验（FAB-E）的心理测量特性和具有代表性的成人样本的标准值。

Neurologia Pub Date : 2024-10-01 DOI: 10.1016/j.nrleng.2022.09.004

{"title":"Psychometric properties of the Spanish version of the Frontal Assessment Battery (FAB-E) and normative values in a representative adult population sample","authors":"","doi":"10.1016/j.nrleng.2022.09.004","DOIUrl":"10.1016/j.nrleng.2022.09.004","url":null,"abstract":"<div><h3>Background</h3><div>The Frontal Assessment Battery is a short bedside test used to assess executive functions (EF). The aims of the present study were, first, to evaluate the psychometric proprieties of the Spanish version of the FAB (FAB-E) in a representative sample, and second, to establish cut-off points for impairment in executive function according to age and education level.</div></div><div><h3>Methods</h3><div>A sample of 798 healthy Spanish adult subjects aged 19 to 91 participated in this study. Neuropsychological assessment of participants was conducted using the FAB-E, Mini-Mental State Examination (MMSE) and Trail Making Test (TMT). We examined internal consistency, intraclass correlation, test-retest reliability, and concurrent and divergent validity. In addition, we established a cut-off point for detecting executive function impairment based on the 5th percentile by age group and education level.</div></div><div><h3>Results</h3><div>The analysis of the psychometric properties of the FAB-E showed good internal consistency (Cronbach’s α = 0.60), intraclass correlation (0.72), test-retest reliability (0.70) and concurrent and divergent validity between the TMT (<em>r</em> = −0.523), MMSE (<em>r</em> = 0.426) and the FAB-E. The cut-off points for each age group were 16 points for the ≤ 29 group, 15 points for the 30-39 group, 14 points for the 40-49 and 50-59 groups, 12 points for the 60-69 group, and 10 points for the ≥ 70 age group.</div></div><div><h3>Conclusions</h3><div>The psychometric analysis showed that the FAB-E has good validity and reliability. Thus, FAB-E may be a helpful tool to evaluate EF in a healthy Spanish population. In addition, this study provides information on reference data that will be very valuable for clinicians and researchers.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"39 8","pages":"Pages 694-700"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33497663","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Kernohan-Woltman notch phenomenon: an exceptional neurological picture? 克诺汉-沃尔特曼缺口现象：一种特殊的神经症状？

Neurologia Pub Date : 2024-10-01 DOI: 10.1016/j.nrleng.2022.09.010

{"title":"Kernohan-Woltman notch phenomenon: an exceptional neurological picture?","authors":"","doi":"10.1016/j.nrleng.2022.09.010","DOIUrl":"10.1016/j.nrleng.2022.09.010","url":null,"abstract":"<div><h3>Introduction</h3><div>Ipsilateral hemiparesis (IH) can be defined as a paradoxical dysfunction of the first motor neuron involving the extremities on the opposite side to that expected, given the location of the triggering intracranial pathology. Compression of the corticospinal tract (CSt) along its course through the contralateral cerebral peduncle against the free edge of the tentorium, known as the Kernohan-Woltman notch phenomenon (KWNP), represents the main cause of IH.</div></div><div><h3>Methods</h3><div>This retrospective study analyses a series of 12 patients diagnosed with IH secondary to KWNP treated at our institution, including a descriptive study of epidemiological, clinical, radiological, neurophysiological, and prognostic variables.</div></div><div><h3>Results</h3><div>In 75% of the cases, symptoms had an acute or subacute onset. Initial imaging studies showed signs of significant mass effect in half of the patients, whereas magnetic resonance imaging (MRI) identified a structural lesion in the contralateral cerebral peduncle in two thirds of them. Impairment of the motor evoked potentials (MEP) was verified in 4 patients. During follow-up 7 patients experienced improvement in motor activity, and near half of the cases were classified in the first three categories of the modified Rankin scale.</div></div><div><h3>Conclusions</h3><div>In contrast to prior historical series, most of our patients developed a KWNP secondary to a traumatic mechanism. MRI represents the optimal method to identify both the classic cerebral peduncle notch and the underlying structural lesion of the CSt. The use of MEP can help to establish the diagnosis, especially in those cases lacking definite radiological findings.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"39 8","pages":"Pages 683-693"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40711319","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Design and validation of a questionnaire for monitoring neurological dysphagia and respiratory deterioration in patients with amyotrophic lateral sclerosis (DEREDELA) 设计并验证用于监测肌萎缩性脊髓侧索硬化症患者神经性吞咽困难和呼吸恶化的调查问卷（DEREDELA）

Neurologia Pub Date : 2024-10-01 DOI: 10.1016/j.nrleng.2024.09.003

S. Bermudo Fuenmayor , P.J. Serrano Castro , P. Quiroga Subirana , S. López Palmero , M.M. Requena Mullor , T. Parrón Carreño

{"title":"Design and validation of a questionnaire for monitoring neurological dysphagia and respiratory deterioration in patients with amyotrophic lateral sclerosis (DEREDELA)","authors":"S. Bermudo Fuenmayor , P.J. Serrano Castro , P. Quiroga Subirana , S. López Palmero , M.M. Requena Mullor , T. Parrón Carreño","doi":"10.1016/j.nrleng.2024.09.003","DOIUrl":"10.1016/j.nrleng.2024.09.003","url":null,"abstract":"<div><h3>Introduction</h3><div>Amyotrophic lateral sclerosis (ALS) is a degenerative disease of unknown origin that affects the motor neurons. It has a rapid, fatal course.</div></div><div><h3>Method</h3><div>For this study, an initial questionnaire of eleven items was developed by experts in the field, who evaluated the suitability and relevance of the items.</div></div><div><h3>Results</h3><div>The questionnaire was then applied to a pilot group of 22 patients diagnosed with ALS. Confirmatory factor analysis, based on estimating maximum likelihood, confirmed the three domains detected in the exploratory factor analysis. The reliability of the scale was tested using Cronbach's <em>α</em> (0.801) and the Kaiser–Meyer–Olkin test (0.770) confirmed the construct validity.</div></div><div><h3>Conclusions</h3><div>The DEREDELA questionnaire is valid, in terms of its content, for monitoring the neurological dysphagia and respiratory deterioration suffered by patients diagnosed with ALS.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"39 8","pages":"Pages 666-674"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142425826","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Cybersickness. A systematic literature review of adverse effects related to virtual reality 网络病。与虚拟现实有关的不良影响的系统文献综述

Neurologia Pub Date : 2024-10-01 DOI: 10.1016/j.nrleng.2022.04.007

L. Simón-Vicente , S. Rodríguez-Cano , V. Delgado-Benito , V. Ausín-Villaverde , E. Cubo Delgado

{"title":"Cybersickness. A systematic literature review of adverse effects related to virtual reality","authors":"L. Simón-Vicente , S. Rodríguez-Cano , V. Delgado-Benito , V. Ausín-Villaverde , E. Cubo Delgado","doi":"10.1016/j.nrleng.2022.04.007","DOIUrl":"10.1016/j.nrleng.2022.04.007","url":null,"abstract":"<div><h3>Background</h3><div>Virtual Reality (VR) uses computer technology to create a simulated environment. VR is a growing technology with promising extensive applications in different areas such as Medicine, entertainment, sports, gaming, and simulation. However, information about VR side effects is still limited. We aimed to identify the most frequent physical side effects caused by VR therapeutic applications.</div></div><div><h3>Methodology</h3><div>All available full-text articles evaluating VR as a therapeutic intervention and side effects using the Simulator Sickness Questionnaire (SSQ) between 2016 and 2021 were consulted across 4 electronic (Entrez Pubmed, Scopus, Science Direct, and Wiley databases). The methodological quality was assessed using the PEDro scale.</div></div><div><h3>Results</h3><div>Ten out of 55 reviewed articles (18%) met inclusion/exclusion criteria, including a sample of 416 patients, mean age of 24.54 (15–52.6)years old. According to the PEDro scale, two articles (20%) were considered good or excellent. Side effects were reported more frequently with head-mounted displays compared to desktop systems, especially disorientation, followed by nausea and oculomotor disturbances.</div></div><div><h3>Conclusions</h3><div>Although VR might have positive effects as a therapeutic tool, VR can also cause side events. As in any other therapeutic intervention, it is important to understand the effectiveness and safety before planning a VR intervention using a well-designed scientific methodology.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"39 8","pages":"Pages 701-709"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142425829","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Analysis of the prognostic value of emergency blood tests in ischaemic stroke 缺血性脑卒中急诊血液化验的预后价值分析。

Neurologia Pub Date : 2024-10-01 DOI: 10.1016/j.nrleng.2022.03.007

{"title":"Analysis of the prognostic value of emergency blood tests in ischaemic stroke","authors":"","doi":"10.1016/j.nrleng.2022.03.007","DOIUrl":"10.1016/j.nrleng.2022.03.007","url":null,"abstract":"<div><h3>Objectives</h3><div>This study aims to evaluate the prognostic value of emergency blood test results in patients with acute ischaemic stroke.</div></div><div><h3>Methods</h3><div>We evaluated 592 prospectively patients with neuroimaging-confirmed ischaemic stroke admitted to our stroke unit between 2015 and 2018. We gathered emergency blood test results and calculated the neutrophil-to-lymphocyte ratio and the neutrophil-to-platelet ratio (neutrophils × 1.000/platelets). The association between blood test results and functional prognosis (as measured with the modified Rankin Scale) and such complications as haemorrhagic transformation was evaluated by logistic regression analysis. The additional predictive value of blood test parameters was assessed with receiver operating characteristic curves and the net reclassification index.</div></div><div><h3>Results</h3><div>An neutrophil-to-lymphocyte ratio ≥ 3 at admission was associated with a two-fold increase in the risk of functional dependence at 3 months (OR: 2.24; 95% CI: 1.35-3.71) and haemorrhagic transformation (OR: 2.11; 95% CI: 1.09-4.05), while an neutrophil-to-lymphocyte ratio ≥ 3.86 resulted in an increase of 2.4 times in the risk of mortality at 3 months (OR: 2.41; 95% CI: 1.37-4.26) after adjusting for the traditional predictors of poor outcomes. Patients with neutrophil-to-platelet ratio ≥ 32 presented 3 times more risk of haemorrhagic transformation (OR: 3.17; 95% CI: 1.70-5.92) and mortality at 3 months (OR: 3.07; 95% CI: 1.69-5.57). Adding these laboratory parameters to standard clinical-radiological models significantly improved discrimination and prognostic accuracy.</div></div><div><h3>Conclusions</h3><div>Basic blood test parameters provide important prognostic information for stroke patients and should therefore be analysed in combination with standard clinical and radiological parameters to optimise ischaemic stroke management.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"39 8","pages":"Pages 617-628"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40696646","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Intrathecal baclofen therapy as treatment for spasticity and dystonia: Review of cases in a pediatric palliative care unit 鞘内巴氯芬治疗痉挛和肌张力障碍：儿科姑息治疗病房病例回顾

Neurologia Pub Date : 2024-10-01 DOI: 10.1016/j.nrleng.2022.04.008

V. Puertas M , I. de Noriega Echevarria , I. Errasti V , M.A. Perez M , R.M. Alba

{"title":"Intrathecal baclofen therapy as treatment for spasticity and dystonia: Review of cases in a pediatric palliative care unit","authors":"V. Puertas M , I. de Noriega Echevarria , I. Errasti V , M.A. Perez M , R.M. Alba","doi":"10.1016/j.nrleng.2022.04.008","DOIUrl":"10.1016/j.nrleng.2022.04.008","url":null,"abstract":"<div><h3>Introduction</h3><div>Patients managed in the Pediatric Palliative Care Integral Unit (PPCIU) have serious neurological conditions that involve significant damage at central nervous system level. The movement disorder is a very common clinical problem and for the patients where an adequate control of muscle tone is not achieved with usual techniques or drugs, intrathecal baclofen therapy (IBT) should be considered.</div></div><div><h3>Materials and methods</h3><div>Descriptive retrospective study based on the review of clinical records of patients who received IBT being followed by the PPCIU of Madrid Autonomous Region in the timeframe between September 2012 and February 2021.</div></div><div><h3>Results</h3><div>IBT was implanted in 8 patients affected by infantile cerebral palsy (ICP) with a Gross Motor Function Scale (GMFCS) IV–V, 3 patients was a Pantothenate kinase deficit-associated neurodegeneration (PKAN), 2 had Acquired Brain Damage, and the remaining 3 had, respectively, 2 glutaric aciduria type I (GA-1), and poly-malformative syndrome. In all patients we observed a period of clinical stability after IBT, we call this period “honeymoon”. Two patients died while in the honeymoon period, at 24.9 and 19.6 months from implantation of the pump; the median of duration of the honeymoon period in the remaining 14 was 14.4 months (IQ: 8.3–25.8).</div></div><div><h3>Conclusions</h3><div>IBT was not only used in patients with non-progressive diseases, but also in the group of patients with neurodegenerative or progressive diseases. In all of them, after implantation of the device, we have objectified a period of clinical stability and a better control of muscle tone disorders.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"39 8","pages":"Pages 675-682"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142425827","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Incidence and prevalence of multiple sclerosis in Spain: a systematic review 西班牙多发性硬化症的发病率和流行率：系统综述。

Neurologia Pub Date : 2024-10-01 DOI: 10.1016/j.nrleng.2022.02.004

{"title":"Incidence and prevalence of multiple sclerosis in Spain: a systematic review","authors":"","doi":"10.1016/j.nrleng.2022.02.004","DOIUrl":"10.1016/j.nrleng.2022.02.004","url":null,"abstract":"<div><h3>Introduction</h3><div>Greater understanding of the prevalence and incidence of multiple sclerosis in Spain and their temporal trends is necessary to improve the allocation of healthcare resources and to study aetiological factors.</div></div><div><h3>Methods</h3><div>We performed a systematic search of the MedLine database and reviewed the reference lists of the articles gathered. We collected studies reporting prevalence or incidence rates of multiple sclerosis in any geographical location in Spain, with no time limits. In 70% of cases, data were extracted by 2 researchers (FGL and EAC); any discrepancies were resolved by consensus.</div></div><div><h3>Results</h3><div>We identified 51 prevalence and 33 incidence studies published between 1968 and 2018. In the adjusted analysis, the number of prevalent cases per 100 000 population increased by 26.6 (95% confidence interval [CI], 21.5–31.8) every 10 years. After adjusting for year and latitude, the number of incident cases per 100 000 population increased by 1.34 (95% CI, 0.98–1.69) every 10 years. We observed a trend toward higher prevalence and incidence rates at higher latitudes.</div></div><div><h3>Conclusions</h3><div>The prevalence of multiple sclerosis in Spain has increased in recent decades, although case ascertainment appears to be incomplete in many studies. Incidence rates have also increased, but this may be due to recent improvements in the detection of new cases.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"39 8","pages":"Pages 639-650"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10726866","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Functional outcomes of patients with NORSE and FIRES treated with immunotherapy: A systematic review 接受免疫疗法治疗的 NORSE 和 FIRES 患者的功能疗效：系统综述。

Neurologia Pub Date : 2024-10-01 DOI: 10.1016/j.nrleng.2022.03.004

{"title":"Functional outcomes of patients with NORSE and FIRES treated with immunotherapy: A systematic review","authors":"","doi":"10.1016/j.nrleng.2022.03.004","DOIUrl":"10.1016/j.nrleng.2022.03.004","url":null,"abstract":"<div><h3>Objective</h3><div>To determine the frequency of good functional outcomes in patients with NORSE and FIRES treated with immunotherapy.</div></div><div><h3>Methods</h3><div>We performed a systematic search of the MedLine and EMBASE databases to gather studies including at least 5 patients with NORSE or FIRES and at least one patient treated with immunotherapy, and reporting functional outcomes. Good functional outcome was defined as a modified Rankin Scale (mRS) score ≤ 2 (or an equivalent measure) at the last available follow-up assessment. Only patients with known functional outcomes were included in the analysis.</div></div><div><h3>Results</h3><div>We analyzed 16 studies including a total of 161 patients with NORSE. Six studies were carried out only with FIRES patients (n = 64). Of the 161 patients with NORSE, 141 (87.5%) received immunotherapy. Outcome data were available for 135, 56 of whom (41.4%) achieved good functional outcomes. Twenty-four of the 58 patients with FIRES treated with immunotherapy and for whom outcome data were available achieved good functional outcomes (41.3%). Mortality rates in patients with NORSE and FIRES treated with immunotherapy were 20/121 (16.5%) and 6/58 (10.3%), respectively. By type of immunotherapy, good functional outcomes were achieved in 36/89 patients receiving glucocorticoids (40.4%), 27/71 patients receiving IV immunoglobulins (38%), 11/37 patients treated with plasma exchange (29.7%), 5/17 patients receiving rituximab (29.4%), and 2/13 patients receiving cyclophosphamide (15.3%).</div></div><div><h3>Conclusion</h3><div>Despite the lack of randomised clinical trials, immunotherapy is frequently prescribed to patients with NORSE and FIRES. However, rates of functional dependence and mortality remain high in these patients. Second-line therapies achieved lower rates of good outcomes, probably because they were administered to patients with more severe, refractory disease.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"39 8","pages":"Pages 629-638"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33482198","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0