Neurologia最新文献_第2页

Test cognitivos breves como herramienta de decisión en Atención Primaria. Estudio poblacional y de validación 作为初级保健决策工具的简短认知测试。人口研究和验证

IF 2.9 4区医学 Q2 CLINICAL NEUROLOGY

Neurologia

Pub Date : 2024-11-01 DOI: 10.1016/j.nrl.2022.05.006

M. Tainta , A. Iriondo , M. Ecay-Torres , A. Estanga , M. de Arriba , M. Barandiaran , M. Clerigue , M. Garcia-Sebastian , J. Villanua , A. Izagirre , J. Saldias , A. Aramburu , J. Taboada , J. Múgica , A. Barandiaran , A. Arrospide , J. Mar , P. Martinez-Lage

Introduction and objectives

Brief cognitive tests (BCT) are used in primary care (PC) for the detection of cognitive impairment (CI). Still, there are little data on their diagnostic utility (DU) in a community setting. This work evaluates the DU at the population level of Fototest, T@M, AD8 questionnaire and MMSE. It provides new cut-off points (CoP) validated in a CI early detection program.

Material and methods

In the population and validation samples, the evaluation was carried out in 2 phases, a first of screening and administration of BCT and a second of clinical diagnosis, blinded to the results of the BCT, applying the current NIA-AA criteria. The DU of BCT in the population sample was evaluated with the area under the ROC curve (aROC). Youden index and the CoP with the best specificity that ensured a sensitivity of 80% were used to decide on the most appropriate CoP. The sensitivity, specificity, and predictive values for these CoP were calculated in the validation sample.

Results

260 participants (23.1% with CI) from the population sample and 177 (42.4% with CI) from the validation sample were included. The Fototest has the best UD at the population level (aROC 0.851), which improves with the combination of Fototest and AD8 (aROC 0.875). The proposed CoP are AD8≥1, Fototest≤35, T@M≤40, and MMSE≤26.

Conclusion

BCT are helpful in detecting CI in PC. This work supports the use of more demanding PoC.

简介和目的简短认知测试（BCT）被用于初级保健（PC）中认知障碍（CI）的检测。然而，有关其在社区环境中的诊断效用（DU）的数据仍然很少。这项研究评估了 Fototest、T@M、AD8 问卷和 MMSE 在人群中的诊断效用。材料和方法在人群和验证样本中，评估分两个阶段进行，第一阶段是筛查和实施 BCT，第二阶段是临床诊断，在对 BCT 结果保密的情况下，采用现行的 NIA-AA 标准。用 ROC 曲线下面积（aROC）评估了人群样本中 BCT 的 DU 值。尤登指数和特异性最好且能确保灵敏度达到 80% 的 CoP 被用来决定最合适的 CoP。在验证样本中计算了这些CoP的灵敏度、特异性和预测值。结果 260名参与者（23.1%，含CI）来自人群样本，177名参与者（42.4%，含CI）来自验证样本。在群体水平上，Fototest 的 UD 最佳（aROC 0.851），而 Fototest 和 AD8 的组合（aROC 0.875）则提高了 UD。建议的 CoP 为 AD8≥1、Fototest≤35、T@M≤40 和 MMSE≤26。这项工作支持使用要求更高的 PoC。

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引用次数: 0

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

IF 2.9 4区医学 Q2 CLINICAL NEUROLOGY

Neurologia

Pub Date : 2024-10-01 DOI: 10.1016/j.nrl.2023.02.002

引用次数: 0

Post-COVID-19 memory complaints: Prevalence and associated factors COVID-19 后的记忆不适：流行率和相关因素。

IF 2.9 4区医学 Q2 CLINICAL NEUROLOGY

Neurologia

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

Introduction

Memory complaints resulting from COVID-19 may have a significant impact on the survivors’ quality of life. Unfortunately, there is insufficient information available on memory loss and its relationship to COVID-19. Therefore, the purpose of this research was to determine the prevalence of memory complaints in post-COVID-19 patients and to find potential contributing factors.

Method

A cross-sectional survey was conducted on 401 individuals who had previously been diagnosed with COVID-19 at four COVID testing centers situated across Bangladesh. The MAC-Q questionnaire was used to evaluate memory. A binary logistic regression model was fit to study the variables related to memory complaints, with a p-value of <0.05 deemed statistically significant.

Result

Memory complaints was prevalent in 19.2% of the post-COVID patients. Individual predictor analysis revealed that among the treatment modalities, steroids and antibiotics were associated with impaired memory. Multiple logistic regression showed that individuals who recovered from COVID-19 within six to twelve months were more likely to have memory deficits. Even though age, sex, oxygen demand, and hospitalization were not linked with memory complaints, rural residents exhibited more significant memory complaints than urban residents.

Conclusion

Nearly one-fifth of the COVID-19 patients suffer from various degrees of memory complaints within one year. However, no association was found between COVID-19 severity to memory complaints.

简介COVID-19 导致的记忆障碍可能会对幸存者的生活质量产生重大影响。遗憾的是，有关记忆力衰退及其与 COVID-19 关系的信息尚不充分。因此，本研究旨在确定 COVID-19 患者记忆力衰退的普遍程度，并找出潜在的诱因：方法：在孟加拉国的四个 COVID 测试中心对 401 名曾被诊断为 COVID-19 的患者进行了横断面调查。采用 MAC-Q 问卷评估记忆力。通过二元逻辑回归模型来研究与记忆力不适有关的变量，结果的 p 值为：19.2%的后 COVID 患者普遍存在记忆障碍。个体预测分析显示，在治疗方式中，类固醇和抗生素与记忆力受损有关。多元逻辑回归显示，在 6 至 12 个月内从 COVID-19 康复的患者更有可能出现记忆障碍。尽管年龄、性别、需氧量和住院时间与记忆障碍无关，但农村居民比城市居民表现出更明显的记忆障碍：结论：近五分之一的 COVID-19 患者在一年内出现不同程度的记忆障碍。结论：近五分之一的 COVID-19 患者在一年内出现不同程度的记忆障碍，但 COVID-19 的严重程度与记忆障碍之间并无关联。

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引用次数: 0

Unilateral MRIgFUS thalamotomy: Long-term follow-up in fragile X-associated tremor/ataxia syndrome 单侧 MRIgFUS 丘脑切开术：脆性X相关震颤/共济失调综合征的长期随访

IF 2.9 4区医学 Q2 CLINICAL NEUROLOGY

Neurologia

Pub Date : 2024-10-01 DOI: 10.1016/j.nrl.2023.11.002

引用次数: 0

Cerebral venous thrombosis in children an 18-year review of a Portuguese hospital 葡萄牙一家医院对儿童脑静脉血栓形成18年的回顾

IF 2.9 4区医学 Q2 CLINICAL NEUROLOGY

Neurologia

Pub Date : 2024-10-01 DOI: 10.1016/j.nrl.2022.04.006

Introduction

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.

Patients and methods

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.

Results

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.

Conclusion

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.

导言脑静脉血栓（CVT）是一种不常见的临床异质性脑血管病，尤其是在儿童中，只有少数发表的系列病例集中在儿科人群中。患者和方法对葡萄牙一家二级医院 2003 年至 2021 年连续收治的确诊为脑静脉血栓的儿科患者进行回顾性单中心观察和分析研究。研究记录了临床表现、神经影像学检查结果、血栓形成诱因、治疗策略、疗效和再通情况。平均年龄为 7.3 岁。最常见的症状是呕吐、头痛和行为改变。在50%的病例中，感染是诱发因素。CVT的诊断依据是通过磁共振成像（MRI）和静脉造影术和/或计算机断层扫描（CT）和静脉造影术获得的血栓形成影像学证据。67%的病例涉及多个静脉窦；横窦受影响最大，其次是乙状窦。83%的病例开始使用低分子量肝素（LMWH）进行抗凝治疗，并对相关的促血栓形成因素进行了调查，但未发现主要的促血栓形成因素。无死亡病例，但30%的患者有长期神经系统后遗症。结论本研究结果与其他已发表的研究数据一致。核磁共振成像可避免电离辐射，并能识别亚邻近病因，是诊断儿童疾病的首选成像方法。建议使用 LMWH 抗凝，这对降低死亡率和减少后遗症非常重要。感染性疾病是 CVT 最常见的诱因，也是导致高发病率和不良后果的原因。

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引用次数: 0

Incidencia y prevalencia de la esclerosis múltiple en España. Una revisión sistemática 西班牙多发性硬化症的发病率和患病率。系统回顾

IF 2.9 4区医学 Q2 CLINICAL NEUROLOGY

Neurologia

Pub Date : 2024-10-01 DOI: 10.1016/j.nrl.2022.02.006

Introduction

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.

Methods

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.

Results

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.

Conclusions

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.

引言为了改善医疗资源的分配并研究病因，有必要进一步了解多发性硬化症在西班牙的患病率和发病率及其时间趋势。方法我们对 MedLine 数据库进行了系统检索，并查阅了所收集文章的参考文献目录。我们收集了报告西班牙任何地区多发性硬化症患病率或发病率的研究，没有时间限制。在 70% 的案例中，数据由两名研究人员（FGL 和 EAC）提取；任何差异均以协商一致的方式解决。结果我们确定了 1968 年至 2018 年间发表的 51 篇患病率研究和 33 篇发病率研究。在调整后的分析中，每 10 万人口中的流行病例数每 10 年增加 26.6 例（95% 置信区间 [CI]，21.5-31.8）。根据年份和纬度进行调整后，每 10 万人中的发病人数每 10 年增加 1.34 例（95% 置信区间 [CI]，0.98-1.69）。我们观察到纬度越高，患病率和发病率越高的趋势。结论近几十年来，多发性硬化症在西班牙的患病率有所上升，但许多研究的病例确定似乎并不完整。发病率也有所上升，但这可能是由于最近在发现新病例方面有所改进。

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引用次数: 0

Cybersickness. A systematic literature review of adverse effects related to virtual reality 赛博病。与虚拟现实相关的不良影响的系统文献综述

IF 2.9 4区医学 Q2 CLINICAL NEUROLOGY

Neurologia

Pub Date : 2024-10-01 DOI: 10.1016/j.nrl.2022.04.009

Background

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.

Methodology

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.

Results

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.

Conclusions

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.

背景虚拟现实（VR）利用计算机技术创建模拟环境。VR 是一项不断发展的技术，有望在医学、娱乐、体育、游戏和模拟等不同领域得到广泛应用。然而，有关 VR 副作用的信息仍然有限。我们的目的是找出 VR 治疗应用最常见的身体副作用。研究方法通过 4 个电子数据库（Entrez Pubmed、Scopus、Science Direct 和 Wiley 数据库）查阅了 2016 年至 2021 年间所有使用模拟器疾病问卷（SSQ）评估 VR 作为治疗干预和副作用的全文文章。结果55篇综述文章中有10篇（18%）符合纳入/排除标准，包括416名患者样本，平均年龄为24.54（15-52.6）岁。根据 PEDro 评分标准，两篇文章（20%）被认为是良好或优秀。与桌面系统相比，头戴式显示器的副作用报告更为频繁，尤其是迷失方向，其次是恶心和眼球运动障碍。与其他任何治疗干预一样，在使用精心设计的科学方法规划 VR 干预之前，了解其有效性和安全性非常重要。

{"title":"Cybersickness. A systematic literature review of adverse effects related to virtual reality","authors":"","doi":"10.1016/j.nrl.2022.04.009","DOIUrl":"10.1016/j.nrl.2022.04.009","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":19300,"journal":{"name":"Neurologia","volume":"39 8","pages":"Pages 701-709"},"PeriodicalIF":2.9,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48410244","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

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:儿科姑息治疗单位的病例回顾

IF 2.9 4区医学 Q2 CLINICAL NEUROLOGY

Neurologia

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

Introduction

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.

Materials and methods

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.

Results

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).

Conclusions

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.

简介：在儿科姑息治疗综合病房（PPCIU）接受治疗的患者都患有严重的神经系统疾病，中枢神经系统受到严重损害。运动障碍是一种非常常见的临床问题，如果使用常规技术或药物无法充分控制患者的肌张力，则应考虑使用鞘内巴氯芬疗法（IBT）。材料和方法根据对马德里自治区姑息治疗综合病房在 2012 年 9 月至 2021 年 2 月期间接受 IBT 治疗的患者临床记录的回顾性研究。结果 8 名小儿脑瘫（ICP）患者接受了 IBT 植入术，其粗大运动功能量表（GMFCS）为 IV-V，3 名患者为泛酸激酶缺乏相关神经变性（PKAN），2 名患者为后天性脑损伤，其余 3 名患者分别为戊二酸尿症 I 型（GA-1）和多畸形综合征。我们观察到所有患者在 IBT 后都有一段临床稳定期，我们称之为 "蜜月期"。两名患者分别在植入泵 24.9 个月和 19.6 个月后在蜜月期死亡；其余 14 名患者蜜月期的中位数为 14.4 个月（IQ：8.3-25.8）。在所有这些患者中，我们都发现在植入该装置后，他们的临床症状在一段时间内趋于稳定，肌张力障碍也得到了更好的控制。

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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）

IF 2.9 4区医学 Q2 CLINICAL NEUROLOGY

Neurologia

Pub Date : 2024-10-01 DOI: 10.1016/j.nrl.2022.03.010

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

Introduction

Amyotrophic lateral sclerosis (ALS) is a degenerative disease of unknown origin that affects the motor neurons. It has a rapid, fatal course.

Method

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.

Results

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 α (0.801) and the Kaiser–Meyer–Olkin test (0.770) confirmed the construct validity.

Conclusions

The DEREDELA questionnaire is valid, in terms of its content, for monitoring the neurological dysphagia and respiratory deterioration suffered by patients diagnosed with ALS.

导言肌萎缩性脊髓侧索硬化症（ALS）是一种原因不明的退行性疾病，影响运动神经元。本研究由该领域的专家编制了一份包含 11 个项目的初始问卷，并由他们对这些项目的适用性和相关性进行了评估。基于最大似然估计的确认性因子分析证实了探索性因子分析中发现的三个领域。使用 Cronbach's α（0.801）检验了量表的可靠性，Kaiser-Meyer-Olkin 检验（0.770）证实了量表的建构效度。结论 DEREDELA 问卷的内容对于监测 ALS 患者的神经吞咽困难和呼吸衰竭是有效的。

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引用次数: 0

Pronóstico funcional de pacientes con NORSE y FIRES tratados con inmunoterapia: revisión sistemática 免疫疗法治疗Norse和Fires患者的功能预后：系统评价

IF 2.9 4区医学 Q2 CLINICAL NEUROLOGY

Neurologia

Pub Date : 2024-10-01 DOI: 10.1016/j.nrl.2022.03.005

Objective

To determine the frequency of good functional outcomes in patients with NORSE and FIRES treated with immunotherapy.

Methods

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.

Results

We analysed 16 studies, including a total of 161 patients with NORSE (10 studies) and 64 patients with FIRES (6 studies). 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%).

Conclusion

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.

方法我们对 MedLine 和 EMBASE 数据库进行了系统检索，收集了至少有 5 名 NORSE 或 FIRES 患者、至少有一名患者接受了免疫疗法并报告了功能结果的研究。良好功能结果的定义是：在最后一次随访评估时，改良Rankin量表（mRS）评分≤2（或同等指标）。结果我们分析了 16 项研究，其中包括 161 名 NORSE 患者（10 项研究）和 64 名 FIRES 患者（6 项研究）。在 161 名 NORSE 患者中，141 人（87.5%）接受了免疫疗法。135名患者获得了疗效数据，其中56人（41.4%）获得了良好的功能性疗效。在 58 名接受免疫疗法且有结果数据的 FIRES 患者中，有 24 人（41.3%）获得了良好的功能性结果。接受免疫疗法治疗的 NORSE 和 FIRES 患者的死亡率分别为 20/121 例（16.5%）和 6/58 例（10.3%）。根据免疫疗法的类型，接受糖皮质激素治疗的 36/89 例患者（40.4%）、接受静脉注射免疫球蛋白治疗的 27/71 例患者（38%）、接受血浆置换治疗的 11/37 例患者（29.7%）、接受利妥昔单抗治疗的 5/17 例患者（29.4%）和接受环磷酰胺治疗的 2/13 例患者（15.3%）均获得了良好的功能预后。结论尽管缺乏随机临床试验，但免疫疗法经常被用于 NORSE 和 FIRES 患者，但这些患者的功能依赖率和死亡率仍然很高。二线疗法取得良好疗效的比例较低，这可能是因为这些疗法用于治疗病情更严重的难治性患者。

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引用次数: 0