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Bullous pemphigoid as an injection site reaction of glatiramer acetate 醋酸格拉替默注射部位反应的大疱性类天疱疮
Pub Date : 2019-10-07 DOI: 10.18502/ijnl.v18i4.2193
S. Baghbanian, M. Ghasemi, Somayeh Sheidaei, Z. Hajheydari
1 Department of Neurology, School of Medicine, Mazandaran University of Medical Sciences, Sari, Iran 2 Department of Pathology, School of Medicine, Mazandaran University of Medical Sciences, Sari, Iran 3 Immunogenetic Research Center, Mazandaran University of Medical Sciences, Sari, Iran 4 Department of Dermatology, School of Medicine, Mazandaran University of Medical Sciences, Sari, Iran 5 Invasive Fungi Research Center, Bualicina Hospital, Mazandaran University of Medical Sciences, Sari, Iran
1伊朗萨里Mazandaran医科大学医学院神经内科2伊朗萨里Mazandaran医科大学医学院病理学系3伊朗萨里Mazandaran医科大学免疫遗传学研究中心4伊朗萨里Mazandaran医科大学医学院皮肤病学学系5伊朗萨里Mazandaran医科大学Bualicina医院侵袭性真菌研究中心
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引用次数: 1
Fitness to drive in seizure and epilepsy: A protocol for Iranian clinicians 癫痫发作和癫痫的健康驱动:伊朗临床医生的协议
Pub Date : 2019-10-07 DOI: 10.18502/ijnl.v18i4.2187
N. Tabrizi
Driving restriction is a well-known undesirable consequence of epilepsy and causes significant problems regarding independence and employment for epileptic patients. Many countries all over the world have provided comprehensive protocols in this regard with the aim of providing the possibility of less restricted, but safe driving for epileptic patients and also providing the opportunity for uniform decision-making for clinicians. However, the available fitness to drive protocol in Iran still lacks sufficient details and clinicians might encounter serious problems in terms of the driving issue in epileptic patients. In order to provide a uniform protocol containing adequate practical data, a systematic review of literature addressing guidelines about driving and epilepsy and driving laws of different countries for epileptic patients was performed and, after consideration of cultural issues, a practical protocol for Iranian neurologists was suggested.
限制驾驶是众所周知的癫痫的不良后果,对癫痫患者的独立和就业造成重大问题。世界上许多国家都在这方面提供了全面的方案,目的是为癫痫患者提供较少限制但安全驾驶的可能性,并为临床医生提供统一决策的机会。然而,伊朗现有的驾驶适应性方案仍然缺乏足够的细节,临床医生可能会在癫痫患者的驾驶问题上遇到严重问题。为了提供一个包含足够实际数据的统一方案,对有关驾驶和癫痫的指南以及不同国家针对癫痫患者的驾驶法律的文献进行了系统的审查,并在考虑文化问题后,建议为伊朗神经科医生提供一个实用的方案。
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引用次数: 1
Amyotrophic onset in GCH1 dopa-responsive dystonia GCH1多巴反应性肌张力障碍的肌萎缩性发作
Pub Date : 2019-10-07 DOI: 10.18502/ijnl.v18i4.2191
S. Habibi, A. Albanese, A. Elia, Paria Arfa-fatollahkhani, N. Hashemi
Dopa-responsive dystonia (DRD) belongs to combined dystonia syndrome (dystonia-plus syndrome)1 which encompasses non-degenerative and neurometabolic disorders characterized by combination of dystonia as the prominent sign, with another movement manifestation. Parkinsonism and myoclonus are the main disturbances accompany dystonia in the combined dystonia syndrome. Dystonia with parkinsonism includes DRD [DYT5, tyrosine hydroxylase (TH), and sepiapterin reductase (SPR)], dopamine agonist-responsive dystonia, rapid-onset dystonia parkinsonism (DYT12), and early-onset dystonia with parkinsonism (DYT16). However, dystonia combined with myoclonus is just classified as myoclonus dystonia (DYT11).2 DRD can be inherited in either autosomal dominant or autosomal recessive patterns. The autosomal dominant inheritance results in the typical phenotype of DRD, known as DYT5 or Segawa disease, which is caused by heterozygous mutations of guanosine triphosphate (GTP) cyclohydrolase I gene (GCH1). Mutations of the TH and SPR genes are responsible for autosomal recessive types of DRD.3 DYT5 is represented as progressive lower limbs dystonia with childhood-onset at the common age of 2-5 years. It shows diurnal fluctuations, which are aggravated toward the evening and alleviated by sleeping. Excellent and sustained response to the low dose of levodopa is the marked feature of DYT5 disease. Additional parkinsonism and spasticity may present later in life.4 Moreover, hemiatrophy of the brain, body, or both has been reported in patients with DRD, associated with a biochemical lesion located in basal ganglia.5 However, focal atrophy and muscle weakness rarely accompanies DRD. Interestingly, we aimed to introduce weakness and focal muscle atrophy as the onset manifestations of DRD in an elderly man misdiagnosed for about 70 years.
多巴反应性肌张力障碍(DRD)属于联合肌张力障碍综合征(肌张力障碍加综合征)1,包括非退行性和神经代谢性疾病,其特征是肌张力障碍作为突出症状,与另一种运动表现相结合。帕金森综合征和肌阵挛是合并肌张力障碍综合征中伴随肌张力障碍的主要障碍。肌张力障碍伴帕金森综合征包括DRD[DYT5、酪氨酸羟化酶(TH)和海泡肽还原酶(SPR)]、多巴胺激动剂反应性肌张力障碍、速发型肌张力障碍性帕金森综合征(DYT12)和早发性肌张力症伴帕金森综合症(DYT16)。然而,肌张力障碍合并肌阵挛仅被归类为肌阵挛肌张力障碍(DYT11)。2 DRD可以常染色体显性或常染色体隐性遗传。常染色体显性遗传导致DRD的典型表型,称为DYT5或Segawa病,由三磷酸鸟苷(GTP)环水解酶I基因(GCH1)的杂合突变引起。TH和SPR基因的突变是常染色体隐性遗传型DRD的原因。3 DYT5表现为进行性下肢肌张力障碍,儿童期常见于2-5岁。它显示出昼夜波动,这种波动在晚上加剧,并通过睡眠缓解。对低剂量左旋多巴的良好和持续反应是DYT5疾病的显著特征。在以后的生活中可能会出现额外的帕金森综合征和痉挛。4此外,据报道,DRD患者大脑、身体或两者都出现半萎缩,与基底神经节的生化损伤有关。5然而,DRD很少伴有局灶性萎缩和肌无力。有趣的是,我们的目的是将虚弱和局灶性肌肉萎缩作为一名被误诊约70年的老年人DRD的发病表现。
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引用次数: 1
Posterior reversible encephalopathy syndrome with spinal cord involvement as the first presentation of lupus nephritis 后路可逆性脑病综合征伴脊髓受累为狼疮肾炎的首发表现
Pub Date : 2019-10-07 DOI: 10.18502/ijnl.v18i4.2190
A. Okhovat, S. Abdi, F. Fatehi
A 23-year-old woman was admitted to the emergency department with the history of headache, serial seizures, and decreased the level of consciousness from a week before. At admission, blood pressure was 230/170 mmHg, and creatinine level was 7.6 mg/dl. Initial brain and cervical magnetic resonance imaging (MRI) revealed hyperintense lesions on fluid-attenuated inversion recovery (FLAIR) in bilateral occipital lobes and a longitudinally extensive lesion in the spinal cord (Figure 1, A-C). In the laboratory investigations, the level of anti-double stranded DNA was 45 IU/ml (normal < 10 IU/ml) and anti-nuclear antibody titer was high (> 1/160). Moreover, in renal biopsy, lupus nephritis was reported. Two weeks later, after hypertension treatment, the hyperintense signals wholly disappeared (Figure 1, D-F). Figure 1. Axial fluid-attenuated inversion recovery (FLAIR) brain magnetic resonance imaging (MRI) indicating hypersignal lesions in parieto-occipital areas in favor of posterior reversible encephalopathy syndrome (PRES) at admission (A, B); sagittal T2 cervical MRI demonstrating a longitudinally extensive lesion in the spinal cord at admission (C); two weeks later, the hyperintense signals had completely disappeared on the brain and spinal cord MRIs (D-F).
一名23岁的女性因头痛、连续癫痫发作和意识水平比一周前下降而住进了急诊室。入院时,血压为230/170毫米汞柱,肌酐水平为7.6毫克/分升。最初的大脑和颈部磁共振成像(MRI)显示,双侧枕叶的液体衰减倒置恢复(FLAIR)出现高信号病变,脊髓出现纵向广泛病变(图1,a-C)。在实验室研究中,抗双链DNA水平为45IU/ml(正常<10IU/ml),抗核抗体滴度高(>1/160)。此外,在肾活检中,报告了狼疮性肾炎。两周后,在高血压治疗后,高信号完全消失(图1,D-F)。图1。轴流衰减反转恢复(FLAIR)脑磁共振成像(MRI)表明,在入院时,顶枕区的高信号病变有利于后部可逆性脑病综合征(PRES)(A,B);矢状T2颈椎MRI显示入院时脊髓纵向广泛病变(C);两周后,大脑和脊髓MRI(D-F)上的高信号完全消失。
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引用次数: 0
Thrombolytic treatment in stroke mimic, inevitable but fortunately safe: An observational study from Iran. 模拟中风的溶栓治疗,不可避免,但幸运的是安全的:一项来自伊朗的观察性研究。
Pub Date : 2019-10-07
Sara Esmaeili, Motahareh Afrakhteh, Maryam Bahadori, Seyedeh Fahimeh Shojaei, Rezan Ashayeri, Masoud Mehrpour

Background: A number of patients with symptoms of acute cerebral ischemia may have other causes called stroke mimics (SM). The prevalence of SM can be as high as 31% in some reports, and these patients are potentially at the risk of intravenous thrombolysis (IVT) therapy and its complications. This study was designed to determine the prevalence of our center's SM (Firoozgar Hospital) among patients who received IVT, their baseline characteristics, final diagnoses, and outcomes. Methods: We reviewed the medical records of all patients who received IVT between June 2015 and May 2018. The following variables were collected: demographic characteristics, past medical history, onset-to-needle (OTN) time, door-to-needle (DTN) time, National Institutes of Health Stroke Scale (NIHSS) score at admission, brain imaging, and all paraclinic findings. Functional outcome at discharge based on modified Rankin Scale (mRS) was also assessed. Results: 10 out of 165 (6.0%) patients including 8 men and 4 women were finally diagnosed with SM. The median age and NIHSS score at presentation were 60 years and 7, respectively. Final diagnoses were seizure (n = 6), hemiplegic migraine (n = 2), conversion (n = 1), and alcohol intoxication (n = 1). All patients were discharged with a mRS score of 0 and 1 without experiencing any thrombolytic adverse effects. Conclusion: None of the patients with SM experienced any adverse effect of tissue plasminogen activator (tPA) including hemorrhage and all of them reached good mRS score. This shows that tPA is generally safe and the risk of treating patients with SM is very low and making a vital treatment decision may outweigh the risk of neglected cases in a time-sensitive setting.

背景:许多有急性脑缺血症状的患者可能有其他原因,称为脑卒中模拟(SM)。在一些报道中,SM的患病率可高达31%,这些患者可能面临静脉溶栓治疗及其并发症的风险。本研究旨在确定我们中心的SM (Firoozgar医院)在接受IVT的患者中的患病率,他们的基线特征,最终诊断和结果。方法:我们回顾了2015年6月至2018年5月期间所有接受IVT治疗的患者的病历。收集以下变量:人口统计学特征、既往病史、从发病到针(OTN)时间、从门到针(DTN)时间、入院时美国国立卫生研究院卒中量表(NIHSS)评分、脑成像和所有临床旁诊结果。出院时的功能预后也根据改良Rankin量表(mRS)进行评估。结果:165例患者中有10例(6.0%)最终确诊为SM,其中男8例,女4例。就诊时的中位年龄和NIHSS评分分别为60岁和7岁。最终诊断为癫痫发作(n = 6)、偏瘫性偏头痛(n = 2)、转化(n = 1)和酒精中毒(n = 1)。所有患者出院时mRS评分分别为0和1,没有出现任何溶栓不良反应。结论:SM患者均未出现组织纤溶酶原激活剂(tPA)出血等不良反应,均达到良好的mRS评分。这表明tPA通常是安全的,治疗SM患者的风险非常低,在时间敏感的情况下,做出重要的治疗决定可能超过被忽视病例的风险。
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引用次数: 0
A survey from Turkey and Iran on comparison of risk factors and etiology in ischemic stroke. 土耳其与伊朗缺血性脑卒中危险因素及病因的比较调查。
Pub Date : 2019-10-07
Gulcin Benbir Senel, Ayse Deniz Elmali, Kaveh Mehrvar, Mehdi Farhoudi, Mohammad Aboutalebi, Mahsa Rezaei, Birsen Ince
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引用次数: 0
Thrombolytic treatment in stroke mimic, inevitable but fortunately safe: An observational study from Iran 模拟中风的溶栓治疗,不可避免但幸运的是安全:来自伊朗的一项观察性研究
Pub Date : 2019-10-07 DOI: 10.18502/ijnl.v18i4.2188
S. Esmaeili, M. Afrakhteh, M. Bahadori, Seyedeh Fahimeh Shojaei, R. Ashayeri, M. Mehrpour
Background: A number of patients with symptoms of acute cerebral ischemia may have other causes called stroke mimics (SM). The prevalence of SM can be as high as 31% in some reports, and these patients are potentially at the risk of intravenous thrombolysis (IVT) therapy and its complications. This study was designed to determine the prevalence of our center’s SM (Firoozgar Hospital) among patients who received IVT, their baseline characteristics, final diagnoses, and outcomes. Methods: We reviewed the medical records of all patients who received IVT between June 2015 and May 2018. The following variables were collected: demographic characteristics, past medical history, onset-to-needle (OTN) time, door-to-needle (DTN) time, National Institutes of Health Stroke Scale (NIHSS) score at admission, brain imaging, and all paraclinic findings. Functional outcome at discharge based on modified Rankin Scale (mRS) was also assessed. Results: 10 out of 165 (6.0%) patients including 8 men and 4 women were finally diagnosed with SM. The median age and NIHSS score at presentation were 60 years and 7, respectively. Final diagnoses were seizure (n = 6), hemiplegic migraine (n = 2), conversion (n = 1), and alcohol intoxication (n = 1). All patients were discharged with a mRS score of 0 and 1 without experiencing any thrombolytic adverse effects. Conclusion: None of the patients with SM experienced any adverse effect of tissue plasminogen activator (tPA) including hemorrhage and all of them reached good mRS score. This shows that tPA is generally safe and the risk of treating patients with SM is very low and making a vital treatment decision may outweigh the risk of neglected cases in a time-sensitive setting.
背景:许多有急性脑缺血症状的患者可能有其他被称为中风模拟物(SM)的原因。在一些报道中,SM的患病率可能高达31%,这些患者可能面临静脉溶栓(IVT)治疗及其并发症的风险。本研究旨在确定我们中心的SM(菲鲁兹加医院)在接受IVT的患者中的患病率、他们的基线特征、最终诊断和结果。方法:我们回顾了2015年6月至2018年5月期间接受IVT的所有患者的医疗记录。收集了以下变量:人口统计学特征、既往病史、起针时间(OTN)、门到针时间(DTN)、入院时美国国立卫生研究院卒中量表(NIHSS)评分、脑成像和所有临床旁检查结果。还评估了基于改良兰金量表(mRS)的出院时的功能结果。结果:165名患者中有10名(6.0%)最终被诊断为SM,其中包括8名男性和4名女性。中位年龄和NIHSS评分分别为60岁和7分。最终诊断为癫痫发作(n=6)、偏瘫性偏头痛(n=2)、转换(n=1)和酒精中毒(n=一)。所有患者出院时mRS评分分别为0和1,未出现任何溶栓不良反应。结论:SM患者均未出现组织型纤溶酶原激活剂(tPA)的不良反应,包括出血,均达到良好的mRS评分。这表明tPA通常是安全的,治疗SM患者的风险非常低,在时间敏感的环境中,做出重要的治疗决定可能会超过被忽视病例的风险。
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引用次数: 1
Effectiveness and side effects of dimethyl fumarate in multiple sclerosis after 12 months of follow up: An Iranian clinical trial. 富马酸二甲酯治疗多发性硬化症12个月后的疗效和副作用:一项伊朗临床试验
Pub Date : 2019-10-07
Mohsen Foroughipour, Sahar Gazeran

Background: Multiple sclerosis (MS) is a neurologic disorder with a considerable global burden. During the last decades, some pharmaceutical treatments have been approved for patients with MS. Dimethyl fumarate (DMF) is one of these drugs which has been reported to have early promising results in recent studies, but the efficacy of this drug in patients with MS is still being studied in different parts of the world. In the present study, we evaluated the effectiveness of DMF therapy on reducing relapses, lesions, and disability in Iranian patients with MS. Methods: The present single-arm before-after study was approved by the Ethics Committee of Mashhad University of Medical Sciences, Mashhad, Iran [Iranian Registry of Clinical Trial (IRCT) code: IRCT20190121042439N1]. Every patient who was diagnosed with relapsing MS was considered eligible to enroll in the present clinical trial. Before receiving DMF therapy, the baseline liver function tests and complete blood count were obtained from all individuals. Also, a baseline brain magnetic resonance imaging (MRI) was obtained and Expanded Disability Status Scale (EDSS) was documented from all patients. After receiving 240 mg DMF twice daily for 12 months, the laboratory and imaging measurements as well as EDSS were repeated. Furthermore, the total number of relapses within the study period was recorded. Satisfaction with DMF treatment was determined by answering a yes-no question. Results: A total number of 50 patients enrolled in the study and most of them were female (80%). There was a significant decrease in EDSS score and gadolinium (GD)-enhancing lesions after the study period (P < 0.001 for each). Moreover, the attacks significantly dropped after the study period (P < 0.001) and 86% of patients were satisfied with their treatment. Conclusion: The findings of this study showed that 240 mg DMF administered twice daily can effectively reduce disability and provide satisfaction within the first year of therapy in patients with MS.

背景:多发性硬化症(MS)是一种神经系统疾病,具有相当大的全球负担。在过去的几十年里,一些药物治疗已经被批准用于多发性硬化症患者,富马酸二甲酯(DMF)是这些药物之一,在最近的研究中已经报道了早期有希望的结果,但该药物对多发性硬化症患者的疗效仍在世界不同地区进行研究。在本研究中,我们评估了DMF治疗在减少伊朗多发性硬化患者复发、病变和致残方面的有效性。方法:本单组前后对照研究获得伊朗马什哈德医学大学伦理委员会批准[伊朗临床试验注册(IRCT)代码:IRCT20190121042439N1]。每一位被诊断为复发性多发性硬化症的患者都被认为有资格参加本临床试验。在接受DMF治疗前,所有个体均获得基线肝功能检查和全血细胞计数。同时,获得基线脑磁共振成像(MRI),并记录所有患者的扩展残疾状态量表(EDSS)。在连续12个月每天两次接受240 mg DMF后,重复实验室和成像测量以及EDSS。此外,还记录了研究期间的总复发次数。对DMF治疗的满意度是通过回答一个是-否问题来确定的。结果:共纳入50例患者,其中以女性居多(80%)。研究结束后,EDSS评分和钆增强病变均显著降低(P < 0.001)。研究结束后发作次数明显减少(P < 0.001), 86%的患者对治疗满意。结论:本研究结果表明,每天两次给予240 mg DMF可有效减少MS患者的残疾,并在治疗的第一年提供满意度。
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引用次数: 0
Amyotrophic onset in GCH1 dopa-responsive dystonia. GCH1多巴反应性肌张力障碍的肌萎缩性发病。
Pub Date : 2019-10-07
Seyed Amir Hasan Habibi, Alberto Albanese, Antonio E Elia, Paria Arfa-Fatollahkhani, Neda Hashemi
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引用次数: 0
A survey from Turkey and Iran on comparison of risk factors and etiology in ischemic stroke 土耳其与伊朗缺血性脑卒中危险因素及病因的比较调查
Pub Date : 2019-10-07 DOI: 10.18502/ijnl.v18i4.2189
G. Şenel, A. Elmalı, K. Mehrvar, M. Farhoudi, M. Aboutalebi, M. Rezaei, B. Ince
Background: Multiple sclerosis (MS) is a neurologic disorder with a considerable global burden. During the last decades, some pharmaceutical treatments have been approved for patients with MS. Dimethyl fumarate (DMF) is one of these drugs which has been reported to have early promising results in recent studies, but the efficacy of this drug in patients with MS is still being studied in different parts of the world. In the present study, we evaluated the effectiveness of DMF therapy on reducing relapses, lesions, and disability in Iranian patients with MS.Methods: The present single-arm before-after study was approved by the Ethics Committee of Mashhad University of Medical Sciences, Mashhad, Iran [Iranian Registry of Clinical Trial (IRCT) code: IRCT20190121042439N1]. Every patient who was diagnosed with relapsing MS was considered eligible to enroll in the present clinical trial. Before receiving DMF therapy, the baseline liver function tests and complete blood count were obtained from all individuals. Also, a baseline brain magnetic resonance imaging (MRI) was obtained and Expanded Disability Status Scale (EDSS) was documented from all patients. After receiving 240 mg DMF twice daily for 12 months, the laboratory and imaging measurements as well as EDSS were repeated. Furthermore, the total number of relapses within the study period was recorded. Satisfaction with DMF treatment was determined by answering a yes-no question.Results: A total number of 50 patients enrolled in the study and most of them were female (80%). There was a significant decrease in EDSS score and gadolinium (GD)-enhancing lesions after the study period (P < 0.001 for each). Moreover, the attacks significantly dropped after the study period (P < 0.001) and 86% of patients were satisfied with their treatment.Conclusion: The findings of this study showed that 240 mg DMF administered twice daily can effectively reduce disability and provide satisfaction within the first year of therapy in patients with MS.
背景:多发性硬化症(MS)是一种神经系统疾病,具有相当大的全球负担。在过去的几十年里,一些药物治疗已经被批准用于多发性硬化症患者,富马酸二甲酯(DMF)是这些药物之一,在最近的研究中已经报道了早期有希望的结果,但该药物对多发性硬化症患者的疗效仍在世界不同地区进行研究。在本研究中,我们评估了DMF治疗在减少伊朗多发性硬化患者复发、病变和致残方面的有效性。方法:本单组前后对照研究获得伊朗马什哈德医学大学伦理委员会批准[伊朗临床试验注册(IRCT)代码:IRCT20190121042439N1]。每一位被诊断为复发性多发性硬化症的患者都被认为有资格参加本临床试验。在接受DMF治疗前,所有个体均获得基线肝功能检查和全血细胞计数。同时,获得基线脑磁共振成像(MRI),并记录所有患者的扩展残疾状态量表(EDSS)。在连续12个月每天两次接受240 mg DMF后,重复实验室和成像测量以及EDSS。此外,还记录了研究期间的总复发次数。对DMF治疗的满意度是通过回答一个是-否问题来确定的。结果:共纳入50例患者,其中以女性居多(80%)。研究结束后,EDSS评分和钆增强病变均显著降低(P < 0.001)。研究结束后发作次数明显减少(P < 0.001), 86%的患者对治疗满意。结论:本研究结果表明,每天两次给予240 mg DMF可有效减少MS患者的残疾,并在治疗的第一年提供满意度。
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引用次数: 2
期刊
Iranian Journal of Neurology
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