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Transcranial sonography in carriers of Gaucher disease. 戈谢病携带者的经颅超声检查。
Pub Date : 2018-07-06
Fatemeh Omrani, Shahla Ansari-Damavandi, Babak Zamani, Zahra Omrani, Nahid Mohammadzade, Sadra Rohani, Mohammad Rohani

Background: Glucocerebrosidase (GBA) mutation is the most common genetic risk factor in Parkinson's disease (PD). Transcranial sonography (TCS) shows increased substantia nigra (SN) echogenicity in both idiopathic and genetic forms of PD. The goal of this study was to compare maximal area of SN hyperechogenicity (aSNmax) and diameter of third ventricle (DTV) between GBA mutation carriers and healthy controls. Methods: Twenty-six carriers of GBA mutation and twenty-six healthy controls underwent TCS. The aSNmax and the DTV were measured. Mini-mental status examination (MMSE) and demographic data of the subjects were recorded, too. Results: Mean aSNmax in GBA mutation carriers was significantly higher (0.31 ± 0.06 cm2) than controls (0.16 ± 0.04 cm2). Moreover, DTV was significantly higher in GBA mutation carriers group (3.98 ± 0.90 vs 3.29 ± 0.56 cm). Conclusion: Increased SN echogenicity and increased third ventricle diameter in GBA mutation carriers may be caused by alterations in iron metabolism with reference to their genetic status.

背景:糖脑苷酶(GBA)突变是帕金森病(PD)最常见的遗传危险因素。经颅超声(TCS)显示特发性和遗传性PD的黑质(SN)回声增强。本研究的目的是比较GBA突变携带者和健康对照组的最大SN高回声面积(aSNmax)和第三脑室直径(DTV)。方法:对26例GBA突变携带者和26例健康对照进行TCS。测量了aSNmax和DTV。同时记录受试者的简易精神状态检查(MMSE)和人口统计数据。结果:GBA突变携带者的平均aSNmax(0.31±0.06 cm2)显著高于对照组(0.16±0.04 cm2)。此外,GBA突变携带者组的DTV(3.98±0.90 cm vs 3.29±0.56 cm)显著高于前者。结论:GBA突变携带者SN回声增强、第三脑室直径增大可能与铁代谢改变有关,并与基因状况有关。
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引用次数: 0
Pegylated versus non-pegylated interferon beta 1a in patients with relapsing-remitting multiple sclerosis: A cost-effectiveness analysis 聚乙二醇化与非聚乙二醇化干扰素β 1a在复发缓解型多发性硬化症患者中的应用:成本-效果分析
Pub Date : 2018-07-06 DOI: 10.18502/IJNL.V17I3.371
Amir Hashemi-Meshkini, Hedieh-Sadat Zekri, Hasan Karimi-Yazdi, P. Zaboli, M. Sahraian, S. Nikfar
Background: Pegylated (PEG) interferon beta 1a has been approved by the United States Food and Drug Administration (USFDA) as an alternative to interferon beta 1a for multiple sclerosis (MS). Due to its higher price, this study aimed to evaluate the cost-effectiveness of PEG-interferon beta 1-a compared with interferon beta 1a from an Iranian payer perspective. Methods: A Markov model was designed according to health states based on Expanded Disability Status Scale (EDSS) and one-month cycles over a 10-year time horizon. Direct medical and non-medical costs were included from a payer perspective. Results: The incremental cost-effectiveness ratio (ICER) was estimated around 11111 US dollars (USD) per quality-adjusted life-year (QALY) gained for the PEG-interferon versus interferon regimen [with currency rate of 29,000 Iranian Rial (IRR) to 1 USD in 2016]. Conclusion: Considering the cost-effectiveness threshold in Iran [three times of gross domestic product (GDP) per capita or 15,945 USD], PEG-interferon beta 1-a could be considered as a cost effective treatment for Iranian patients with MS.
背景:聚乙二醇化(PEG)干扰素β 1a已被美国食品和药物管理局(USFDA)批准作为干扰素β 1a治疗多发性硬化症(MS)的替代药物。由于其价格较高,本研究旨在从伊朗付款人的角度评估peg -干扰素β 1-a与干扰素β 1a的成本效益。方法:以扩展残疾状态量表(EDSS)为基础,以10年为周期,根据健康状况设计马尔可夫模型。从付款人的角度包括直接医疗和非医疗费用。结果:与干扰素方案相比,peg -干扰素方案每质量调整生命年(QALY)的增量成本-效果比(ICER)估计约为11111美元(USD)[2016年货币汇率为29,000伊朗里亚尔(IRR)兑换1美元]。结论:考虑到伊朗的成本-效果阈值[人均国内生产总值(GDP)的3倍或15,945美元],peg -干扰素β 1-a可被认为是伊朗多发性硬化症患者的成本-效果治疗方法。
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引用次数: 4
Cognitive function, depression, and quality of life in patients with ruptured cerebral aneurysms. 脑动脉瘤破裂患者的认知功能、抑郁和生活质量。
Pub Date : 2018-07-06
Samira Zabyhian, Seyed Javad Mousavi-Bayegi, Humain Baharvahdat, Farhad Faridhosseini, Payam Sasannejad, Maryam Salehi, Maryam Boroumand, Zahra Hatefipour

Background: Neuropsychiatric dysfunction is one of the most common complications after aneurysmal subarachnoid hemorrhage (aSAH). The aim of this study was to evaluate cognitive function, depression, and quality of life (QOL) in patients with aSAH. Methods: In this study, we prospectively enrolled patients with SAH due to rupture of anterior circulation aneurysms who referred to Ghaem hospital, Mashhad, Iran, and who had good function outcome [modified Rankin scale (mRS) > 2]. They underwent microsurgery or endovascular treatment. Cognitive function, depression, and QOL were evaluated 6 months after surgery with standard psychiatric examinations, including Mini-Mental State Examination (MMSE) for cognitive function, Hospital Anxiety and Depression Scale (HADS) for depression, and 36-Item Short Form Health Survey (SF-36) for QOL. Risk factors for cognitive dysfunction were assessed. Results: Fifty-three patients were entered the study. The mean of age was 50.9 ± 13.6 years. QOL and its components were affected in most patients. Fifty-five percent of patients suffered from depression. Cognitive impairment was found in 57% of patients. Older patients experienced more cognitive impairment (P < 0.001). Conclusion: Neuropsychological sequels are common in patients with aSAH, even if they classified as good functional outcome (mRS > 2). These complications could be found with appropriate neuropsychological evaluation of these patients to be managed as soon as possible.

背景:神经精神功能障碍是动脉瘤性蛛网膜下腔出血(aSAH)后最常见的并发症之一。本研究的目的是评估aSAH患者的认知功能、抑郁和生活质量(QOL)。方法:在本研究中,我们前瞻性地招募了到伊朗Mashhad Ghaem医院就诊并具有良好功能预后的前循环动脉瘤破裂SAH患者[改良Rankin量表(mRS) > 2]。他们接受了显微手术或血管内治疗。术后6个月采用标准精神病学检查评估认知功能、抑郁和生活质量,包括认知功能的简易精神状态检查(MMSE)、抑郁的医院焦虑抑郁量表(HADS)和生活质量的36项简短健康调查(SF-36)。评估认知功能障碍的危险因素。结果:53例患者进入研究。平均年龄50.9±13.6岁。大多数患者的生活质量及其各组成部分均受到影响。55%的患者患有抑郁症。57%的患者存在认知障碍。老年患者有更多的认知障碍(P < 0.001)。结论:神经心理学后遗症在aSAH患者中很常见,即使他们被归类为良好的功能结局(mRS > 2)。这些并发症可以通过对这些患者进行适当的神经心理学评估来发现并尽快处理。
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引用次数: 0
Pegylated versus non-pegylated interferon beta 1a in patients with relapsing-remitting multiple sclerosis: A cost-effectiveness analysis. 聚乙二醇化与非聚乙二醇化干扰素β 1a在复发缓解型多发性硬化症患者中的应用:成本-效果分析
Pub Date : 2018-07-06
Amir Hashemi-Meshkini, Hedieh Sadat Zekri, Hasan Karimi-Yazdi, Pardis Zaboli, Mohammad Ali Sahraian, Shekoufeh Nikfar

Background: Pegylated (PEG) interferon beta 1a has been approved by the United States Food and Drug Administration (USFDA) as an alternative to interferon beta 1a for multiple sclerosis (MS). Due to its higher price, this study aimed to evaluate the cost-effectiveness of PEG-interferon beta 1-a compared with interferon beta 1a from an Iranian payer perspective. Methods: A Markov model was designed according to health states based on Expanded Disability Status Scale (EDSS) and one-month cycles over a 10-year time horizon. Direct medical and non-medical costs were included from a payer perspective. Results: The incremental cost-effectiveness ratio (ICER) was estimated around 11111 US dollars (USD) per quality-adjusted life-year (QALY) gained for the PEG-interferon versus interferon regimen [with currency rate of 29,000 Iranian Rial (IRR) to 1 USD in 2016]. Conclusion: Considering the cost-effectiveness threshold in Iran [three times of gross domestic product (GDP) per capita or 15,945 USD], PEG-interferon beta 1-a could be considered as a cost effective treatment for Iranian patients with MS.

背景:聚乙二醇化(PEG)干扰素β 1a已被美国食品和药物管理局(USFDA)批准作为干扰素β 1a治疗多发性硬化症(MS)的替代药物。由于其价格较高,本研究旨在从伊朗付款人的角度评估peg -干扰素β 1-a与干扰素β 1a的成本效益。方法:以扩展残疾状态量表(EDSS)为基础,以10年为周期,根据健康状况设计马尔可夫模型。从付款人的角度包括直接医疗和非医疗费用。结果:与干扰素方案相比,peg -干扰素方案每质量调整生命年(QALY)的增量成本-效果比(ICER)估计约为11111美元(USD)[2016年货币汇率为29,000伊朗里亚尔(IRR)兑换1美元]。结论:考虑到伊朗的成本-效果阈值[人均国内生产总值(GDP)的3倍或15,945美元],peg -干扰素β 1-a可被认为是伊朗多发性硬化症患者的成本-效果治疗方法。
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引用次数: 0
Cognitive function, depression, and quality of life in patients with ruptured cerebral aneurysms 脑动脉瘤破裂患者的认知功能、抑郁和生活质量
Pub Date : 2018-07-06 DOI: 10.18502/IJNL.V17I3.368
Samira Zabyhian, Seyed Javad Mousavi-Bayegi, H. Baharvahdat, F. Faridhosseini, P. Sasannejad, M. Salehi, M. Boroumand, Zahra Hatefipour
Background: Neuropsychiatric dysfunction is one of the most common complications after aneurysmal subarachnoid hemorrhage (aSAH). The aim of this study was to evaluate cognitive function, depression, and quality of life (QOL) in patients with aSAH. Methods: In this study, we prospectively enrolled patients with SAH due to rupture of anterior circulation aneurysms who referred to Ghaem hospital, Mashhad, Iran, and who had good function outcome [modified Rankin scale (mRS) > 2]. They underwent microsurgery or endovascular treatment. Cognitive function, depression, and QOL were evaluated 6 months after surgery with standard psychiatric examinations, including Mini-Mental State Examination (MMSE) for cognitive function, Hospital Anxiety and Depression Scale (HADS) for depression, and 36-Item Short Form Health Survey (SF-36) for QOL. Risk factors for cognitive dysfunction were assessed. Results: Fifty-three patients were entered the study. The mean of age was 50.9 ± 13.6 years. QOL and its components were affected in most patients. Fifty-five percent of patients suffered from depression. Cognitive impairment was found in 57% of patients. Older patients experienced more cognitive impairment (P < 0.001). Conclusion: Neuropsychological sequels are common in patients with aSAH, even if they classified as good functional outcome (mRS > 2). These complications could be found with appropriate neuropsychological evaluation of these patients to be managed as soon as possible.
背景:神经精神功能障碍是动脉瘤性蛛网膜下腔出血(aSAH)后最常见的并发症之一。本研究的目的是评估aSAH患者的认知功能、抑郁和生活质量(QOL)。方法:在本研究中,我们前瞻性地招募了到伊朗Mashhad Ghaem医院就诊并具有良好功能预后的前循环动脉瘤破裂所致SAH患者[改良Rankin量表(mRS) >2]。他们接受了显微手术或血管内治疗。术后6个月采用标准精神病学检查评估认知功能、抑郁和生活质量,包括认知功能的简易精神状态检查(MMSE)、抑郁的医院焦虑抑郁量表(HADS)和生活质量的36项简短健康调查(SF-36)。评估认知功能障碍的危险因素。结果:53例患者进入研究。平均年龄50.9±13.6岁。大多数患者的生活质量及其各组成部分均受到影响。55%的患者患有抑郁症。57%的患者存在认知障碍。老年患者有更多的认知障碍(P < 0.001)。结论:神经心理学后遗症在aSAH患者中很常见,即使他们被归类为良好的功能结局(mRS bbbb2)。这些并发症可以通过对这些患者进行适当的神经心理学评估来发现并尽快处理。
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引用次数: 6
Validation of the Persian version of dysphagia in multiple sclerosis questionnaire for the assessment of dysphagia in multiple sclerosis 波斯版多发性硬化症吞咽困难问卷评估多发性痴呆症吞咽困难的有效性
Pub Date : 2018-07-06 DOI: 10.18502/IJNL.V17I3.365
Zahra Jafari, M. Shahbodaghi, Saman Maroufizadeh, A. Moghadasi
Background: About one third of patients with multiple sclerosis (MS) experience dysphagia. Dysphagia can cause complications such as malnutrition, lung infections, aspiration pneumonia and dehydration, thus it is very helpful to diagnose dysphagia as soon as possible. The aim in this study was to translate dysphagia in multiple sclerosis (DYMUS) questionnaire into Farsi and and validate it. Methods: Forward-backward method was used to translate original English version of DYMUS into farsi, and then the questionnaire (one for each patient) was filled in through asking questions from 200 patients with MS by a speech and language pathologist. Confirmatory factor analysis (CFA) was used to examine the construct validity. Internal consistency and test-retest reliability of the DYMUS were evaluated using Cronbach’s alpha and the intraclass correlation coefficient (ICC), respectively. Results: CFA showed that a two-factor model of DYMUS including “dysphagia for solid” and “dysphagia for liquid” fitted the data well [relative chi-square [χ2/degree of freedom (df) = 1.55, CFI = 0.967, Tucker-Lewis index (TLI) = 0.957, root mean square error of approximation (RMSEA) = 0.053, and weighted root mean square residual (WRMR) = 0.874]. The Cronbach’s alpha for total score and liquid and solid subscales were 0.776, 0.557, and 0.725, respectively. The DYMUS showed adequate test-retest reliability for the total and subscales (ICC: 0.880-0.956). Moreover, this study has shown a significant relationship between DYMUS score and Expanded Disability Status Scale (EDSS) score, disease duration, type of MS, and self-reported dysphagia. Conclusion: The Persian version of DYMUS is a reliable and valid tool to screen dysphagia among patients with MS.
背景:大约三分之一的多发性硬化症患者有吞咽困难。吞咽困难可引起营养不良、肺部感染、吸入性肺炎和脱水等并发症,因此尽早诊断吞咽困难非常有帮助。本研究的目的是将多发性硬化症吞咽困难(DYMUS)问卷翻译成波斯语并进行验证。方法:采用前向-后向法将DYMUS的英文原版翻译成波斯文,然后由言语和语言病理学家对200名MS患者进行提问,填写问卷(每位患者一份)。验证性因素分析(CFA)用于检验结构的有效性。DYMUS的内部一致性和重测可靠性分别使用Cronbachα和组内相关系数(ICC)进行评估。结果:CFA显示,包括“固体吞咽困难”和“液体吞咽困难”的DYMUS双因素模型与数据拟合良好[相对卡方[χ2/自由度(df)=1.55,CFI=0.967,Tucker Lewis指数(TLI)=0.957,近似均方根误差(RMSEA)=0.053,加权均方根残差(WRMR)=0.874]液体和固体分量表分别为0.776、0.557和0.725。DYMUS在总分和分量表中显示出足够的重测可靠性(ICC:0.880-0.956)。此外,本研究显示DYMUS评分与扩展残疾状态量表(EDSS)评分、疾病持续时间、MS类型和自我报告的吞咽困难之间存在显著关系。结论:波斯版DYMUS是筛查MS患者吞咽困难的可靠有效工具。
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引用次数: 1
Oxidative stress-related biomarkers in Parkinson's disease: A systematic review and meta-analysis. 帕金森病的氧化应激相关生物标志物:系统综述和荟萃分析
Pub Date : 2018-07-06
Zeba Khan, Sharique Athar Ali

Parkinson's disease (PD) is a neurodegenerative disease characterized with the loss of dopamine-producing neurons in a mid-brain. This loss is believed to be associated with number of environmental and genetic factors. Oxidative stress is found to be one of the factors responsible for the initiation and progression of PD. However, studies are still continued to confirm the connection and mechanism associated with oxidative stress and PD. This systematic review and meta-analysis aimed to assess the association between oxidative stress markers and PD, and explore factors that may elucidate the contradictions in these results. As per Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guideline systematic literature search was carried out. Meta-analysis was carried out on pooled standardized mean differences with 95% confidence interval (CI) of patients with PD and controls using random effect model in comprehensive meta-analysis statistical software. Total 17 studies were included into which 25 oxidative stress markers were analyzed. The results revealed that oxidative stress markers [nitrate and nitric oxide (NO)] and antioxidant markers [total antioxidant status (TAS) and thiols] were not statistically different between the PD and control group (P > 0.05). In case of oxidative stress markers, levels of malondialdehyde (MDA), 8-Oxo-2'-deoxyguanosine (8-oxo-dG), and lipid hydro-peroxide (LPO) were found to be high in patients with PD as compared to controls with P < 0.05, whereas lower levels of antioxidant activity of superoxide dismutase (SOD), glucose 6 phosphate dehydrogenase (G6PD), catalase (CAT), and glutathione peroxidase (GPx) were noticed in the PD group as compared to controls (P < 0.05 for all). From the results, it is concluded that patients with PD have high oxidative stress and lower antioxidant activity, and these studied biomarkers would be used as potential diagnostic tool to measure oxidative stress in patients with PD.

帕金森病(PD)是一种神经退行性疾病,其特征是中脑中产生多巴胺的神经元的丧失。这种损失被认为与许多环境和遗传因素有关。氧化应激是PD发生和发展的重要因素之一。然而,氧化应激与PD之间的联系及相关机制仍在研究中不断得到证实。本系统综述和荟萃分析旨在评估氧化应激标志物与PD之间的关系,并探讨可能解释这些结果矛盾的因素。按照系统评价和荟萃分析的首选报告项目(PRISMA)指南进行系统文献检索。采用综合meta分析统计软件中的随机效应模型,对PD患者与对照组的95%置信区间(CI)合并标准化均值差进行meta分析。共纳入17项研究,分析了25项氧化应激标志物。结果显示,PD组与对照组的氧化应激指标[硝酸盐和一氧化氮(NO)]和抗氧化指标[总抗氧化状态(TAS)和硫醇]差异均无统计学意义(P > 0.05)。在氧化应激标志物方面,PD组丙二醛(MDA)、8-氧-2′-脱氧鸟苷(8-氧- dg)和脂质过氧化氢(LPO)水平均高于对照组(P < 0.05),而PD组超氧化物歧化酶(SOD)、葡萄糖6磷酸脱氢酶(G6PD)、过氧化氢酶(CAT)和谷胱甘肽过氧化物酶(GPx)的抗氧化活性均低于对照组(P < 0.05)。由此可见,PD患者具有较高的氧化应激和较低的抗氧化活性,这些生物标志物可作为PD患者氧化应激的潜在诊断工具。
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引用次数: 0
Neurobrucellosis with intramedullary spinal cord involvement: A case report 神经布鲁氏菌病伴髓内脊髓受累1例
Pub Date : 2018-07-06 DOI: 10.18502/IJNL.V17I3.376
Vida Mohammadzadeh, Samira Akbarieh, Seyed Abdolreza Ghoreishi, Manizheh Jozpanahi
article's abstract is no available.
没有文章的摘要。
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引用次数: 1
The protective role of TBX21-1514T>C polymorphism in susceptibility to multiple sclerosis TBX21-1514T>C多态性在多发性硬化易感性中的保护作用
Pub Date : 2018-07-06 DOI: 10.18502/IJNL.V17I3.367
F. Akbarian, M. Ataei, Z. Salehi, M. Nabavi, M. Sanati
Background: As a T-cell mediated disease, multiple sclerosis (MS) pathogenesis might be associated with the immune system and its involved genes. TBX21, which encodes T-bet transcription factor, is a critical regulator of the commitment to the Th1 lineage and Interferon gamma (IFNγ) production. Investigation of the association of -1514T > C polymorphism located upstream of TBX21 gene with MS susceptibility is reasonable due to its demonstrated significant association with some other immune-mediated diseases. Methods: We analyzed the genotype frequencies of -1514T > C polymorphism between 248 Iranian patients with MS and 163 matched healthy controls. By applying polymerase chain reaction-single strand conformation polymorphism (PCR-SSCP)- technique, the single-strand conformation patterns of the amplicons were compared and sequenced. Results: Strong association between the wild -1514T allele and MS susceptibility was found with the allelic frequency of 99.6% in patients vs. 95.1% in controls (P = 0.002), and the CC genotype frequency of the TBX21 polymorphism (-1514T > C) reported potential protective effect against the disease (P = 0.014). Conclusion: The TBX21-1514T > C polymorphism confers possible protective effect on MS in Iranian population. Further comprehensive studies in different settings are required to clarify the exact role of TBX21 gene in MS disease.
背景:多发性硬化症作为一种T细胞介导的疾病,其发病机制可能与免疫系统及其相关基因有关。TBX21编码T-bet转录因子,是Th1谱系和干扰素γ(IFNγ)产生的关键调节因子。位于TBX21基因上游的-1514T>C多态性与MS易感性的相关性研究是合理的,因为它与其他一些免疫介导的疾病有显著相关性。方法:我们分析了248例伊朗多发性硬化症患者和163例健康对照者-1514T>C多态性的基因型频率。应用聚合酶链式反应-单链构象多态性(PCR-SSCP)技术对扩增子的单链构象模式进行了比较和测序。结果:发现野生-1514T等位基因与MS易感性之间有很强的相关性,患者的等位基因频率为99.6%,TBX21多态性的CC基因型频率(-1514T>C)报告了对该疾病的潜在保护作用(P=0.014)。结论:TBX21-1514T>C多态性可能对伊朗人群的MS具有保护作用。需要在不同环境中进行进一步的综合研究,以阐明TBX21基因在多发性硬化症中的确切作用。
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引用次数: 1
Induction or aggravation of other immune-mediated disorders by disease-modifying therapy in treatment of multiple sclerosis 在多发性硬化症的治疗中,疾病修饰疗法诱导或加重其他免疫介导的疾病
Pub Date : 2018-07-06 DOI: 10.18502/IJNL.V17I3.372
S. Baghbanian, M. Sahraian
Interferon beta (IFN-β) and glatiramer acetate (GA) are the primary therapeutic immunomodulatory agents that interfere with relapsing-remitting multiple sclerosis (RRMS), and the most commonly-used drugs as well. Induction or aggravation of other immune-mediated diseases has been reported following INF-β administration. We have reviewed the reported cases to notify the treating physicians about these rare adverse events. Although co-morbid autoimmune disorders have been reported in patients with MS, the pro-inflammatory role of disease-modifying drugs, especially INF-β, could affect and enhance this co-occurrence. Clinical or laboratory autoimmunity histories suggest the use of GA over INF-β as the treatment of choice.
干扰素-β(IFN-β)和醋酸格拉默(GA)是干扰复发-缓解型多发性硬化症(RRMS)的主要治疗性免疫调节剂,也是最常用的药物。据报道,INF-β给药后会诱发或加重其他免疫介导的疾病。我们对报告的病例进行了审查,以告知治疗医生这些罕见的不良事件。尽管多发性硬化症患者存在共病性自身免疫性疾病的报道,但疾病修饰药物,尤其是INF-β的促炎作用可能会影响并增强这种共病性。临床或实验室自身免疫史表明,使用GA而不是INF-β作为治疗的选择。
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引用次数: 2
期刊
Iranian Journal of Neurology
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