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Evaluation of glucose-6-phosphate dehydrogenase serum level in patients with multiple sclerosis and neuromyelitis optica. 多发性硬化症和视神经脊髓炎患者血清葡萄糖-6-磷酸脱氢酶水平的评价。
Pub Date : 2019-10-07
Niloofar Chitsaz, Leila Dehghani, Amir Safi, Nazgol Esmalian-Afyouni, Vahid Shaygannejad, Majid Rezvani, Karim Sohrabi, Kaykhosro Moridi, Milad Moayednia

Background: Multiple sclerosis (MS) and neuromyelitis optica (NMO) are both demyelinating disorders and oxidative stress is suggested to have a role in their pathogenesis. Glucose-6-phosphate dehydrogenase (G6PD) produces nicotinamide adenine dinucleotide phosphate (NADPH) via the pentose phosphate pathway. NADPH is not only involved in the synthesis of fatty acids necessary for myelination, but also it is involved in the defense against oxidative stress. Prescribing supplementary vitamin D as a part of the MS treatment plan can increase G6PD gene expression. The aim of this study was to determine the serum level of G6PD in patients with MS and NMO and its relationship with vitamin D, since it is yet to be explored thoroughly. Methods: In this case-control study, subjects were divided into three experimental and control groups. The experimental groups comprised 50 patients with relapsing-remitting MS (RRMS) who had a history of vitamin D consumption, 50 newly-diagnosed MS patients, and 50 patients with NMO. Control group included 65 healthy individuals. Serum level of G6PD was measured and compared among these groups. Results: No significant difference was seen between the G6PD level in patients with MS and NMO, but it should be noted that this level was significantly lower than the healthy group. G6PD serum level was significantly higher in patients with MS who had previously consumed supplementary vitamin D compared to those who had not. Conclusion: G6PD deficiency is observed in patients with MS and NMO. Also, supplementary vitamin D may induce favorable results on the G6PD level.

背景:多发性硬化症(MS)和视神经脊髓炎(NMO)都是脱髓鞘疾病,氧化应激被认为在其发病机制中起作用。葡萄糖-6-磷酸脱氢酶(G6PD)通过戊糖磷酸途径产生烟酰胺腺嘌呤二核苷酸磷酸(NADPH)。NADPH不仅参与髓鞘形成所需脂肪酸的合成,还参与氧化应激的防御。作为MS治疗计划的一部分,处方补充维生素D可以增加G6PD基因表达。本研究的目的是确定MS和NMO患者血清G6PD水平及其与维生素D的关系,因为它还没有被深入探讨。方法:采用病例对照研究,将受试者分为实验组和对照组。实验组包括50名有维生素D摄入史的复发缓解型多发性硬化症(RRMS)患者,50名新诊断的多发性硬化症患者和50名NMO患者。对照组包括65名健康个体。测定各组血清G6PD水平并进行比较。结果:MS患者与NMO患者G6PD水平无显著差异,但值得注意的是,G6PD水平明显低于健康组。与未补充维生素D的患者相比,先前服用维生素D的MS患者血清G6PD水平明显更高。结论:MS合并NMO患者存在G6PD缺乏。此外,补充维生素D可能会对G6PD水平产生有利的影响。
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引用次数: 0
“Disability Grief”: A patient’s allegorical expression of her disability “残疾悲伤”:一个病人对自己残疾的寓言式表达
Pub Date : 2019-10-07 DOI: 10.18502/ijnl.v18i4.2194
A. Moghadasi
Multiple sclerosis (MS) is an autoimmune disabling disease of the central nervous system, and can lead to a wide range of symptoms. Although the most common form of MS is relapsing-remitting, most of the patients also will develop disability in the future due to the natural course of the disease.1,2 The disease onset mostly occurs in 27 years of age.3 Therefore, considering the symptoms and complications of the disease, it can virtually affect the whole life of the patient. Unfortunately, there is not any accurate study of the opinions of patients with MS regarding their illness. However, in the daily work of treatment, one of the well-known cases of severe discomfort expressed by patients is the fear of future disability. This issue is aggravated when a person starts to lose abilities. When the patient experiences disability in walking and a decrease in his/her daily activities, thinking about the future becomes one of his/her major concerns. Most patients express these concerns in their visits. However, a patient who has other abilities such as poetry, writing a story, or painting, can more effectively portray concerns. The painting reported here is the allegorical view of a patient about her disability. The patient is a 37-year-old woman who has had MS for 15 years. Her illness started with the right eye optic neuritis. She has had six attacks during this period and, after 9 years, her disease entered the secondary progressive phase. Her symptom in this stage was the weakness of the right lower extremity, which was gradually deteriorating such as difficulty in walking. Now, she is unable to walk without help. This disability has affected all aspects of her life, and has greatly diminished her quality of life. She has been interested in drawing since childhood, and has drawn as a non-professional artist. One of her paintings reported here (Figure 1), according to the patient herself, reflects her perception and grief over her progressive disability. She believes that she should say goodbye to her ability and good days of the past. She painted herself as someone (when she could walk unrestrictedly) moving away along with a balloon
多发性硬化症(MS)是一种中枢神经系统自身免疫性致残性疾病,可导致多种症状。尽管多发性硬化症最常见的形式是复发-缓解,但由于疾病的自然病程,大多数患者将来也会出现残疾。1,2疾病发作大多发生在27岁。3因此,考虑到疾病的症状和并发症,它实际上会影响患者的一生。不幸的是,对于多发性硬化症患者对其疾病的看法,没有任何准确的研究。然而,在日常治疗工作中,患者表达的严重不适的一个众所周知的例子是对未来残疾的恐惧。当一个人开始丧失能力时,这个问题就会加剧。当患者出现行走障碍和日常活动减少时,思考未来成为他/她的主要担忧之一。大多数患者在就诊时都表达了这些担忧。然而,具有诗歌、写故事或绘画等其他能力的患者可以更有效地表达担忧。这里报道的这幅画是一位病人对自己残疾的寓言式看法。患者是一名37岁的女性,患有多发性硬化症已有15年。她的病始于右眼视神经炎。在此期间,她有六次发作,9年后,她的疾病进入了继发性进展阶段。她在这一阶段的症状是右下肢无力,并逐渐恶化,如行走困难。现在,没有人帮助,她无法行走。这种残疾影响了她生活的方方面面,大大降低了她的生活质量。她从小就对绘画感兴趣,并以非专业艺术家的身份进行绘画。据患者自己说,她在这里报道的一幅画(图1)反映了她对自己逐渐残疾的看法和悲伤。她认为她应该告别自己的能力和过去的好日子。她把自己描绘成一个(当她可以不受限制地走路时)随着气球离开的人
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引用次数: 1
Ptosis following botulinum toxin injection in hemifacial spasm 半面肌痉挛注射肉毒杆菌毒素后上睑下垂
Pub Date : 2019-10-07 DOI: 10.18502/ijnl.v18i4.2192
M. Jamshidian-Tehrani, Hadi Z. Mehrjardi, Abolfazl Kasaee, S. Yadegari
Hemifacial spasm (HFS) is characterized by irregular involuntary tonic or clonic contractions of muscles innervated by the seventh cranial nerve. Patients usually need long-term treatment, as spontaneous remission is infrequent. Repeated botulinum toxin injection has been shown as a safe and successful treatment for symptomatic relief in patients with HFS.1 Side effects are usually mild and transient. Ptosis has been reported in about 24% of patients with HFS probably due to diffusion of toxin to levator palpebrae superioris muscle.2 However, the frequency of ptosis in patients with HFS has not been addressed yet, due to causes other than botulinum toxin side effect. Herein, we present a case of HFS who presented to our clinic with complaint of complete ptosis and progressive pain early after botulinum toxin injection. Despite initial negative evaluations, further work up revealed a compressive lesion. A 75-year-old man presented with left severe ptosis since 3 weeks ago (Figure 1). He was known case of left HFS since 4 years ago, and botulinum toxin had been regularly injected for his symptom relief in orbicularis oculi, corrugator, and procerus muscles.   Figure 1. Left Blepharoptosis one week after botulinum toxin injection   The patient stated that this new ptosis had begun within a week after his last Dysport (Ipsen, Ltd., Slough, Berkshire, UK) injection while he had no ptosis in his previous injections. In past medical history, he had ischemic heart disease, hypertension, cataract extraction of both eyes, and glaucoma surgery on his right eye. He had been admitted to the neurology ward of a general hospital.
面肌痉挛(HFS)的特征是第七颅神经支配的肌肉不规则的非自主强直或阵挛性收缩。患者通常需要长期治疗,因为自发缓解的情况很少。反复注射肉毒杆菌毒素已被证明是一种安全、成功的治疗HFS患者症状缓解的方法。1副作用通常是轻微和短暂的。据报道,约24%的HFS患者上睑下垂,可能是由于毒素扩散到上睑提肌。2然而,由于肉毒杆菌毒素副作用以外的原因,HFS患者的上睑下垂频率尚未得到解决。在此,我们报告了一例HFS患者,他们在注射肉毒杆菌毒素后早期出现完全性上睑下垂和进行性疼痛。尽管最初的评估为阴性,但进一步的检查显示有压迫性病变。一名75岁的男性自3周前开始出现左侧严重上睑下垂(图1)。自4年前以来,他一直是已知的左侧HFS病例,并定期注射肉毒杆菌毒素以缓解眼轮匝肌、皱纹肌和前突肌的症状。图1。注射肉毒杆菌毒素一周后左上下垂患者表示,这种新的上下垂是在他最后一次注射Dysport(Ipsen,有限公司,Slough,Berkshire,UK)后一周内开始的,而他之前的注射中没有上下垂。在过去的病史中,他患有缺血性心脏病、高血压、双眼白内障摘除术和右眼青光眼手术。他住进了一家综合医院的神经内科病房。
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引用次数: 0
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
Ali Asghar Okhovat, Siamak Abdi, Farzad Fatehi
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引用次数: 0
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个月随访后的疗效和副作用:一项伊朗临床试验
Pub Date : 2019-10-07 DOI: 10.18502/ijnl.v18i4.2186
M. Foroughipour, S. 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,一些药物治疗已被批准用于多发性硬化症患者。富马酸二甲酯(DMF)是其中一种药物,据报道,在最近的研究中,这种药物具有早期的良好效果,但这种药物对多发性痴呆症患者的疗效仍在世界各地进行研究。在本研究中,我们评估了DMF治疗在减少伊朗MS患者复发、病变和残疾方面的有效性。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治疗之前,对所有个体进行基线肝功能测试和全血细胞计数。此外,获得了基线脑磁共振成像(MRI),并记录了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240mg DMF后,重复实验室和成像测量以及EDSS。此外,还记录了研究期间复发的总次数。通过回答“是”-“否”的问题来确定对DMF处理的满意度。结果:共有50名患者参与研究,其中大多数是女性(80%)。研究期结束后,EDSS评分和钆(GD)增强病变显著降低(P均<0.001)。此外,在研究期后,发作次数显著下降(P<0.001),86%的患者对他们的治疗感到满意。结论:本研究结果表明,每天两次服用240 mg DMF可以有效地减少MS患者的残疾,并在治疗的第一年内提供满意度。
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引用次数: 2
Ptosis following botulinum toxin injection in hemifacial spasm. 半面肌痉挛注射肉毒杆菌毒素后上睑下垂。
Pub Date : 2019-10-07
Mansooreh Jamshidian-Tehrani, Hadi Z Mehrjardi, Abolfazl Kasaee, Samira Yadegari
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引用次数: 0
Evaluation of glucose-6-phosphate dehydrogenase serum level in patients with multiple sclerosis and neuromyelitis optica 多发性硬化症和视神经脊髓炎患者血清葡萄糖-6-磷酸脱氢酶水平的评价
Pub Date : 2019-10-07 DOI: 10.18502/ijnl.v18i4.2185
N. Chitsaz, L. Dehghani, Amir Safi, Nazgol Esmalian-Afyouni, V. Shaygannejad, M. Rezvani, K. Sohrabi, Kaykhosro Moridi, Milad Moayednia
Background: Multiple sclerosis (MS) and neuromyelitis optica (NMO) are both demyelinating disorders and oxidative stress is suggested to have a role in their pathogenesis. Glucose-6-phosphate dehydrogenase (G6PD) produces nicotinamide adenine dinucleotide phosphate (NADPH) via the pentose phosphate pathway. NADPH is not only involved in the synthesis of fatty acids necessary for myelination, but also it is involved in the defense against oxidative stress. Prescribing supplementary vitamin D as a part of the MS treatment plan can increase G6PD gene expression. The aim of this study was to determine the serum level of G6PD in patients with MS and NMO and its relationship with vitamin D, since it is yet to be explored thoroughly. Methods: In this case-control study, subjects were divided into three experimental and control groups. The experimental groups comprised 50 patients with relapsing-remitting MS (RRMS) who had a history of vitamin D consumption, 50 newly-diagnosed MS patients, and 50 patients with NMO. Control group included 65 healthy individuals. Serum level of G6PD was measured and compared among these groups. Results: No significant difference was seen between the G6PD level in patients with MS and NMO, but it should be noted that this level was significantly lower than the healthy group. G6PD serum level was significantly higher in patients with MS who had previously consumed supplementary vitamin D compared to those who had not. Conclusion: G6PD deficiency is observed in patients with MS and NMO. Also, supplementary vitamin D may induce favorable results on the G6PD level.
背景:多发性硬化症(MS)和视神经脊髓炎(NMO)都是脱髓鞘疾病,氧化应激被认为在其发病机制中起作用。葡萄糖-6-磷酸脱氢酶(G6PD)通过戊糖磷酸途径产生烟酰胺腺嘌呤二核苷酸磷酸(NADPH)。NADPH不仅参与髓鞘形成所需脂肪酸的合成,还参与氧化应激的防御。作为MS治疗计划的一部分,处方补充维生素D可以增加G6PD基因表达。本研究的目的是确定MS和NMO患者血清G6PD水平及其与维生素D的关系,因为它还没有被深入探讨。方法:采用病例对照研究,将受试者分为实验组和对照组。实验组包括50名有维生素D摄入史的复发缓解型多发性硬化症(RRMS)患者,50名新诊断的多发性硬化症患者和50名NMO患者。对照组包括65名健康个体。测定各组血清G6PD水平并进行比较。结果:MS患者与NMO患者G6PD水平无显著差异,但值得注意的是,G6PD水平明显低于健康组。与未补充维生素D的患者相比,先前服用维生素D的MS患者血清G6PD水平明显更高。结论:MS合并NMO患者存在G6PD缺乏。此外,补充维生素D可能会对G6PD水平产生有利的影响。
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引用次数: 3
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
Nasim 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: 0
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
Seyed Mohammad Baghbanian, Maryam Ghasemi, Somayeh Sheidaei, Zohreh Hajheydari
{"title":"Bullous pemphigoid as an injection site reaction of glatiramer acetate.","authors":"Seyed Mohammad Baghbanian,&nbsp;Maryam Ghasemi,&nbsp;Somayeh Sheidaei,&nbsp;Zohreh Hajheydari","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":45759,"journal":{"name":"Iranian Journal of Neurology","volume":"18 4","pages":"187-189"},"PeriodicalIF":0.0,"publicationDate":"2019-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7036050/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37693434","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
"Disability Grief": A patient's allegorical expression of her disability. “残疾悲伤”:病人对自己残疾的讽喻表达。
Pub Date : 2019-10-07
Abdorreza Naser Moghadasi
{"title":"\"Disability Grief\": A patient's allegorical expression of her disability.","authors":"Abdorreza Naser Moghadasi","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":45759,"journal":{"name":"Iranian Journal of Neurology","volume":"18 4","pages":"190-191"},"PeriodicalIF":0.0,"publicationDate":"2019-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7036046/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37693435","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
期刊
Iranian Journal of Neurology
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