Pyridostigmine Bromide

溴化吡唑斯的明
  • 文章类型: Case Reports
    背景:老年重症肌无力患者在适当的诊断和反应下可以有良好的预后,尽管很难区分老年患者重症肌无力的加重和年龄相关的变化。因此,了解老年重症肌无力患者的临床特点和安全性评估方法对医师有重要意义。
    方法:一名82岁男性,6个月前诊断为重症肌无力,日常生活没有困难。在高尔夫球场上摔倒后,第1天被诊断为右侧股骨颈骨折,第12天接受了右侧全髋关节置换手术,在第32天被转移到我们医院接受康复治疗。然而,转移后立即,患者在训练期间表现出疲劳和吞食困难。
    方法:本病例诊断为重症肌无力加重。
    方法:开始吡唑斯的明,预期在第54天立即生效。
    结果:他的症状和身体功能立即得到改善,步行距离和食物摄入量增加。从这个临床过程中,据判断,免疫抑制治疗被认为是向广泛性重症肌无力的过渡.出于这个原因,他在安排神经科出院后就诊后出院,因此。
    结论:更好地了解老年重症肌无力的特点可以相对安全地评估病情并改善其诊断和治疗。
    BACKGROUND: Patients with elderly-onset myasthenia gravis can have a good prognosis with appropriate diagnosis and response, although it is difficult to differentiate between exacerbations of myasthenia gravis in elderly patients and age-related changes. Therefore, it is important for physicians to understand the clinical characteristics and safe assessment methods for patients with elderly-onset myasthenia gravis.
    METHODS: An 82-year-old male diagnosed with myasthenia gravis 6 months prior had no difficulty in daily living. After falling on a golf course, he was diagnosed with a right femoral neck fracture on the 1st day and underwent right total hip replacement surgery on the 12th day, being transferred to our hospital for rehabilitation therapy on the 32nd day. However, immediately after transfer, the patient showed fatigability during training and difficulty swallowing food.
    METHODS: This case was diagnosed as an exacerbation of myasthenia gravis.
    METHODS: Pyridostigmine was initiated with the expectation of immediate effect on the 54th day.
    RESULTS: His symptoms and physical functions improved immediately, and walking distance and food intake increased. From this clinical course, it was judged that immunosuppressive therapy was indicated as a transition to generalized myasthenia gravis. For this reason, he was discharged after arranging postdischarge visits to the department of neurology, accordingly.
    CONCLUSIONS: A better understanding of the characteristics of elderly-onset myasthenia gravis may allow for relatively safe assessment of the condition and improve its diagnosis and treatment.
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  • 文章类型: Case Reports
    背景技术重症肌无力是与胸腺瘤强烈相关的神经肌肉病症。尽管重症肌无力与其他肿瘤的存在并不常见,大约50%的胸腺瘤患者患有重症肌无力。由于重症肌无力和霍奇金淋巴瘤患者的多例报道,应考虑胸腺霍奇金淋巴瘤。在这份报告中,我们介绍了一例24岁的重症肌无力女性,偶然发现胸腺瘤合并胸腺霍奇金淋巴瘤.病例报告1例24岁女性,已知1例白癜风,有2年复视和偶发前纵隔肿块病史。经过调查,重症肌无力由吡啶斯的明诊断和治疗,泼尼松龙,还有硫唑嘌呤.关于前纵隔肿块,根据重症肌无力的存在和放射学检查结果怀疑胸腺瘤。她接受了扩大的经胸骨胸腺切除术。解剖胸腺的最终组织病理学报告显示,霍奇金淋巴瘤病理与胸腺瘤并存。确诊后的霍奇金淋巴瘤结节性硬化IIA型,化疗6个周期。四年的随访显示没有霍奇金淋巴瘤的证据。然而,尽管霍奇金淋巴瘤缓解,但重症肌无力症状持续.结论重症肌无力与霍奇金淋巴瘤之间的关系尚不清楚。先前的报告显示,霍奇金淋巴瘤治疗后,重症肌无力消退,这表明肌无力可能是霍奇金淋巴瘤的并发症。然而,在我们的案例中,重症肌无力在霍奇金淋巴瘤治疗后持续存在;因此,需要进一步的研究来探索这种关联.
    BACKGROUND Myasthenia gravis is a neuromuscular disorder that is strongly associated with thymoma. Although the presence of myasthenia gravis with other tumors is uncommon, approximately 50% of patients with thymoma have myasthenia gravis. Thymic Hodgkin lymphoma should be considered due to the multiple reported cases of patients with myasthenia gravis and Hodgkin lymphoma. In this report, we present the case of 24-year-old woman with myasthenia gravis who was incidentally found to have coexisting thymoma with thymic Hodgkin lymphoma. CASE REPORT A 24-year-old woman with a known case of vitiligo presented with a 2-year history of diplopia and incidental anterior mediastinal mass. Following investigations, myasthenia gravis was diagnosed and managed by pyridostigmine, prednisolone, and azathioprine. Regarding the anterior mediastinal mass, thymoma was suspected based on the presence of myasthenia gravis and radiological findings. She underwent extended transsternal thymectomy. The final histopathological report of the dissected thymus disclosed Hodgkin lymphoma pathology coexisting with thymoma. After the diagnosis of Hodgkin lymphoma nodular sclerosis type IIA was confirmed, 6 cycles of chemotherapy were administered. Four years of follow-up revealed no evidence of Hodgkin lymphoma. However, her symptoms of myasthenia gravis persisted despite Hodgkin lymphoma remission. CONCLUSIONS There is an unclear association between myasthenia gravies and Hodgkin lymphoma. Prior reports revealed regression of myasthenia gravies following Hodgkin lymphoma management, which suggests that myasthenia could be a complication of Hodgkin lymphoma. However, in our case, myasthenia gravis persisted after Hodgkin lymphoma management; therefore, further studies are needed to explore this association.
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  • 文章类型: Case Reports
    背景:面部发作的感觉和运动神经元病(FOSMN)是一种非常罕见的疾病,到目前为止,尸检证据与神经退行性变有关。重症肌无力(MG)是突触后神经肌肉接头的抗体介导和补体参与的获得性自身免疫性疾病。关于两者之间是否有关联的报道很少。在这项研究中,我们介绍了一个被诊断为FOSMN并连续性MG的病例.
    方法:患者主诉是右侧面部麻木和右眼睑不完全闭合,其次是言语不清和吞咽困难,症状逐渐进展。患者血清抗AchR和抗Titin抗体呈阳性。
    方法:患者诊断为FOSMN合并MG。
    方法:患者经溴吡斯的明和泼尼松龙治疗后症状缓解。
    结果:症状有所改善。
    结论:面部发作的感觉和运动神经元病和MG具有不同的临床特征。因此,我们报道了一个罕见的病例,其中两种情况同时存在。免疫功能紊乱可能是这种关联的发病机制,虽然没有确切的证据支持,需要进一步的研究。
    BACKGROUND: Facial-onset sensory and motor neuronopathy (FOSMN) is a greatly rare disease, so far, autopsy evidence that is associated with neurodegenerative. Myasthenia gravis (MG) is an antibody-mediated and complement-involved acquired autoimmune disorder of the post-synaptic neuromuscular junction. There have been few reports about if there is related between the 2. In this study, we present the case of a man who was diagnosed as FOSMN with MG in continuity.
    METHODS: The patient chief complaints were right-side facial numbness and right-eyelid incomplete closure, followed by slurred speech and dysphagia, and the symptoms gradually progressed. The patient serum was positive for anti-AchR and anti-Titin antibodies.
    METHODS: The patient was diagnosed FOSMN with MG.
    METHODS: The patient symptoms were relieved after pyridostigmine bromide and prednisolone treatment.
    RESULTS: Symptoms have improved.
    CONCLUSIONS: Facial-onset sensory and motor neuronopathy and MG have disparate clinical features. Therefore, we reported a rare case in which the 2 conditions concurrently existed. Immune dysfunction might be the pathogenesis of this association, while there is no definite evidence to support it, further studies are needed.
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  • 文章类型: Case Reports
    背景:重症肌无力是一种自身免疫性疾病,影响神经肌肉接头并引起肌肉无力和疲劳(肌无力)。当重症肌无力的临床表现被隔离到眼部肌肉时,只会导致微弱的眼球运动,它被称为眼部重症肌无力,可以模拟1和1/2综合征。
    方法:一名50多岁的非裔美国女性,既往有高血压病史,出现在我们的门诊,主诉视力模糊两周。她的症状与面部不适和全身头痛有关。在她初次陈述时的身体检查中,左上眼睑有明显肿胀伴下垂。她的眼外运动显示出右眼外展和内收的缺陷,左眼不会加成,虽然向外运动是正常的。向上看时,左眼未能完全抬起/抬起,伪1和½综合征。还注意到Cogan盖子抽搐。大脑和眼眶的成像排除了中央原因。根据抗乙酰胆碱受体抗体阳性对眼肌重症肌无力进行诊断。口服120毫克吡啶斯的明,患者主观和客观地经历了改善,患者口服吡啶斯的明和泼尼松出院。六个月后,泼尼松已经逐渐减少,患者出现肌无力危象,接受血浆置换和静脉注射免疫球蛋白治疗。从肌无力危机中恢复过来后,开始了efgartigimod输注,帮助我们的病人恢复正常生活.
    结论:我们的患者出现“视力模糊”,被发现是由于明显的眼球运动异常而导致的双眼复视。在努力排除中心病因后,我们的结论是,她的表现是由于外周病因。她的血清学和表现有助于确认眼部重症肌无力的诊断。此外,在大多数情况下,我们的患者在服用吡啶斯的明时也发展为全身性重症肌无力。在我们的患者从肌无力危机中康复后开始的Efgartigimod输注帮助她恢复正常生活。
    BACKGROUND: Myasthenia gravis is an autoimmune condition affecting the neuromuscular junction and causing muscle weakness along with fatigue (myasthenia). When the clinical manifestations of myasthenia gravis are isolated to the eye muscles, only causing weak eye movements, it is referred to as ocular myasthenia gravis, which can mimic a 1 and ½ syndrome.
    METHODS: An African-American female in her fifties with past medical history of hypertension presented to our outpatient clinic with complaints of blurred vision for two weeks. Her symptoms were associated with facial discomfort and a generalized headache. On physical examination upon her initial presentation, there was demonstratable swelling of the left upper eyelid with drooping. Her extraocular movements revealed defects with the abduction and adduction of the right eye, and the left eye would not adduct, although the outward movement was normal. The left eye failed to lift/elevate completely when looking upwards, a pseudo 1 and ½ syndrome. A positive Cogan lid twitch was also noticed. Imaging of the brain and orbit ruled out central causes. Diagnosis of ocular myasthenia gravis was made in accordance with positive anti-acetylcholine receptor antibodies. With 120 mg pyridostigmine oral dose, the patient experienced improvement subjectively and objectively, and the patient was discharged on oral pyridostigmine and prednisone. Six months later, with prednisone having been tapered off, the patient developed a myasthenic crisis and was treated with plasmapheresis and intravenous immunoglobulins. After recovering from the myasthenic crisis, efgartigimod infusions were instituted, which helped our patient restore normal life.
    CONCLUSIONS: Our patient who presented with \"blurred vision\" was discovered to have binocular diplopia due to significant dysconjugate eye movements. After diligently ruling out central etiologies, we concluded that her presentation was due to a peripheral etiology. Her serologies and her presentation helped confirm a diagnosis of ocular myasthenia gravis. Also, as in most cases, our patient also progressed to develop generalized myasthenia gravis while on pyridostigmine. Efgartigimod infusions instituted after our patient recovered from a myasthenic crisis have helped her restore a normal life.
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  • 文章类型: Case Reports
    我们介绍了一例早发性重症肌无力(MG)女性的吡啶斯的明引起的冠状动脉痉挛,该女性在吡啶斯的明剂量上调后几天遭受急性胸部不适。十二导联心电图显示下肢导联ST段抬高并伴有窦性停搏。立即给予舌下硝酸盐,这迅速缓解了她的症状,同时解决了异常的心电图发现。冠状动脉造影显示正常的冠状动脉反映了该疾病的短暂性质。在冠状动脉监护病房的密切监测下,小剂量的吡啶斯的明被再次挑战,并重现了她的胸部不适。用免疫抑制剂代替吡啶斯的明并处方长效硝酸盐后,她没有复发胸部不适,以及控制良好的MG症状。
    We present a case of pyridostigmine-induced coronary artery spasm in a woman with early-onset myasthenia gravis (MG) who suffered from acute chest discomfort a few days after pyridostigmine dose up-titration. Twelve-lead ECG demonstrated ST-segment elevation in inferior limb leads together with sinus arrest. Sublingual nitrate was immediately given, which rapidly relieved her symptoms concomitantly with the resolution of abnormal ECG findings. Coronary angiography showed normal coronary arteries reflecting the transient nature of the disease. A small dose of pyridostigmine was rechallenged under close monitoring in the coronary care unit and reproduced her chest discomfort. After the substitution of pyridostigmine with immunosuppressive agents and prescription of long-acting nitrate, she had no recurrence of chest discomfort, as well as well-controlled MG symptoms.
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  • 文章类型: Case Reports
    重症肌无力(MG)是一种获得性自身免疫性疾病。其临床表现包括上睑下垂,复视,构音障碍,吞咽困难,四肢无力,在严重的情况下,呼吸肌受累。构音障碍作为MG的唯一初始和主要主诉很少见,也很少报道。在本文中,我们报告一例以孤立构音障碍为唯一临床表现的IIIb型MG,并复习相关文献。该患者是一名62岁的男子,他出现了长达20天的言语不清的发作,在过去的9天中恶化了。他的病史包括高血压,糖尿病,和冠心病。入院时最初诊断为短暂性脑缺血发作。仔细重新检查患者的病史显示,他的症状主要包括越来越严重的言语不清,没有饮酒或吞咽困难,休息时未观察到显着改善。肌电图和自身抗体谱分析导致IIIb型MG的诊断。口服溴吡啶斯的明60mg后,他的症状有所改善。喉部MG对鉴别中风有重要意义。遇到MG症状不典型的患者时,有必要进行计算机语音分析。
    Myasthenia gravis (MG) is an acquired autoimmune disease. Its clinical manifestations comprise ptosis, diplopia, dysarthria, dysphagia, limb weakness, and in severe cases, respiratory muscle involvement. Dysarthria as an exclusive initial and primary complaint in MG is rare and seldom reported. In this paper, we report a case of type IIIb MG with isolated dysarthria as the only clinical manifestation and we review the relevant literature. The patient was a 62-year-old man who presented with episodes of slurred speech for 20 days that had worsened in the previous 9 days. His medical history comprised hypertension, diabetes mellitus, and coronary heart disease. The initial diagnosis on admission was transient ischemic attack. Careful re-examination of the patient\'s history revealed that his symptoms mainly involved increasingly worse slurred speech episodes without drinking or swallowing difficulties, and no significant improvement with rest was observed. Electromyography and autoantibody profiling led to a diagnosis of type IIIb MG. His symptoms improved after the oral administration of pyridostigmine bromide 60 mg. Laryngeal MG is important to differentiate from stroke. It is necessary to perform a computerized voice analysis when encountering patients with atypical symptoms of MG.
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  • 文章类型: Case Reports
    软骨发育不全是由成纤维细胞生长因子受体3(FGFR3)基因的“功能获得”突变引起的常染色体显性遗传的最常见的骨骼发育不良。由于骨化加速和椎管狭窄继发于椎弓根间距减小而导致的大孔压迫是软骨发育不全的标志。由G380R核苷酸对取代驱动。在严重的情况下,肢体无力和神经源性跛行需要手术减压。很少,神经系统疾病可能模仿压迫性脊柱功能障碍,因此,对于软骨发育不全儿童急性神经系统恶化的情况,也必须考虑非手术原因。重症肌无力(MG)是一种自身免疫性疾病,可导致易疲劳的肌肉无力。文献中没有报道软骨发育不全中的重症肌无力病例。
    我们报告了一名患有软骨发育不全的儿童,计划接受严重腰椎管狭窄症的减压手术,表现为神经跛行和膝关节无力。在COVID-19大流行期间等待手术时,她出现了广泛的易疲劳性和严重的虚弱,引起了对脐带压迫急性恶化的担忧。紧急调查排除了脊髓压迫,但发现了意外并发的重症肌无力,并伴有乙酰胆碱受体抗体阳性。手术干预被推迟,避免了危及生命的麻醉并发症的潜在风险。她成功地接受了吡啶斯的明的联合治疗,类固醇,硫唑嘌呤,和血浆置换。
    我们报告了第一例软骨发育不全的重症肌无力病例,并回顾了治疗的意义。
    Achondroplasia is the commonest skeletal dysplasia of autosomal dominant inheritance caused by \"gain of function\" mutations in the fibroblast growth factor receptor 3 (FGFR3) gene. Foramen magnum compression due to accelerated ossification and spinal canal stenosis secondary to reduced interpedicular distance is a hallmark of achondroplasia, driven by G380R nucleotide pair substitution. In severe cases, limb weakness and neurogenic claudication will require surgical decompression. Rarely, a neurological condition may mimic the compressive spinal dysfunction and therefore, non-surgical causes must also be considered in cases of acute neurological deterioration in children with achondroplasia. Myasthenia gravis (MG) is an autoimmune condition resulting in fatigable muscle weakness. There are no reported cases of myasthenia gravis in achondroplasia in the literature.
    We report a child with achondroplasia scheduled for decompressive surgery for severe lumbar canal stenosis presenting with neurological claudication and knee weakness. While waiting for surgery during the COVID-19 pandemic, she developed generalized fatigability and severe weakness raising concerns of acute worsening of cord compression. Urgent investigations ruled out spinal cord compression but identified an unexpected concurrent myasthenia gravis with positive antibodies to acetylcholine receptors. The surgical intervention was postponed averting the potential risk of life-threatening anaesthetic complications. She was successfully managed with a combination of pyridostigmine, steroids, azathioprine, and plasma exchange.
    We report the first case of myasthenia gravis in achondroplasia and review implications in the management.
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  • 文章类型: Case Reports
    背景:重症肌无力患者也可能合并自身免疫性疾病。由于重症肌无力和视神经脊髓炎谱系疾病都是由抗体介导的,它们很可能一起发生。然而,由于多发性硬化症是一种自身免疫性疾病,不是由特异性抗体介导的,与视神经脊髓炎谱系疾病相比,它与重症肌无力共同的免疫机制较少。我们在重症肌无力患者中遇到了一例新发展的多发性硬化症。
    方法:一名46岁的男性在6年前被诊断出患有眼部重症肌无力,并一直在服用吡啶斯的明以控制其症状。
    方法:患者出现右侧视神经炎,根据脑部磁共振成像结果怀疑多发性硬化症。然而,未达到所需的诊断标准.
    方法:未开始疾病改善治疗,并监测疾病的临床进展。
    结果:视神经炎发病后一年,患者出现脊髓炎并被诊断为多发性硬化症,提示用疾病修饰疗法治疗。
    结论:重症肌无力患者发生视神经炎时,仔细评估是必要的,同时考虑它可能是脱髓鞘中枢神经系统疾病的首发症状。因此,对神经系统自身免疫性疾病患者进行较短间隔的监测和症状筛查非常重要,比如重症肌无力,即使最初没有怀疑多发性硬化症,以实现多发性硬化症的早期检测。
    BACKGROUND: Patients with myasthenia gravis may also have comorbid autoimmune diseases. Since both myasthenia gravis and neuromyelitis optica spectrum disease are mediated by antibodies, they are likely to occur together. However, since multiple sclerosis is an autoimmune disease that is not mediated by a specific antibody, it has fewer immune mechanisms in common with myasthenia gravis than neuromyelitis optica spectrum disease. We encountered a case of newly developed multiple sclerosis in a patient with myasthenia gravis.
    METHODS: A 46-year-old man was diagnosed with ocular myasthenia gravis 6 years ago and had been taking pyridostigmine to control his symptoms.
    METHODS: The patient developed right optic neuritis, and multiple sclerosis was suspected based on the brain magnetic resonance imaging findings. However, the required diagnostic criteria were not met.
    METHODS: Disease-modifying therapy was not initiated, and clinical progression of the disease was monitored.
    RESULTS: One year after the onset of optic neuritis, the patient developed myelitis and was diagnosed with multiple sclerosis, prompting treatment with disease-modifying therapy.
    CONCLUSIONS: When optic neuritis occurs in patients with myasthenia gravis, careful evaluation is necessary while considering the possibility that it may be the first symptom of a demyelinating central nervous system disease. Therefore, it is important to conduct shorter-interval monitoring and symptom screening for patients with neurological autoimmune diseases, such as myasthenia gravis, even if multiple sclerosis is not initially suspected, to achieve early detection of multiple sclerosis.
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  • 文章类型: Case Reports
    背景:自身免疫性疾病是指由于自身免疫平衡失调或免疫耐受破坏而引起的人体异常免疫应答和组织损伤的一类疾病。最近的研究表明,自身免疫性疾病的发生受遗传因素的影响,荷尔蒙,免疫学,和环境因素。由于性激素水平在怀孕和产后发生明显变化,在此期间,自身免疫性疾病的发病率和复发率增加。
    方法:一名31岁亚裔妇女因重症肌无力入院,产后3个月接受甲基强的松龙和溴化吡啶斯的明治疗。入院后体格检查和实验室检查提示患者患有原发性胆汁性肝硬化。随后,硫唑嘌呤被添加到治疗中,两种疾病的症状都得到了成功控制。
    结论:该病例表现为产后重症肌无力合并原发性胆汁性肝硬化的罕见病症。鉴于产后免疫状态的波动,当患者出现自身免疫性疾病的临床症状时,需要考虑合并的自身免疫性疾病。因此,详细的物理和实验室检查有助于防止这些疾病的漏诊。
    BACKGROUND: Autoimmune diseases refers to a class of diseases involving abnormal immune response of human body and tissue damage caused by the dysregulation of autoimmune balance or destruction of immune tolerance. Recent research has revealed that the occurrence of autoimmune diseases is influenced by genetic, hormonal, immunological, and environmental factors. As sex hormone levels change obviously during pregnancy and postpartum, the morbidity and recurrence rate of autoimmune diseases increase during this period.
    METHODS: A 31-year-old Asian woman was admitted to our hospital for myasthenia gravis and treated with methylprednisolone and pyridostigmine bromide 3 months postpartum. Physical examination and laboratory inspection after admission suggested that the patient had primary biliary cirrhosis. Subsequently, azathioprine was added to the treatment, and the symptoms of both diseases were successfully controlled.
    CONCLUSIONS: This case exhibits a rare condition of myasthenia gravis combined with primary biliary cirrhosis postpartum. Given the fluctuation of the immune status during the postpartum period, combined autoimmune diseases need to be taken into account when patients develop clinical symptoms of an autoimmune disease. Therefore, detailed physical and laboratory examination can help to prevent the missed diagnosis of these diseases.
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  • 文章类型: Case Reports
    我们介绍了一个有重症肌无力背景的男性,他有一个颈部肿块,被诊断为甲状腺脂肪瘤病,并伴有非常大的胸腺脂肪瘤。切除这些病变后,患者的症状有所改善。虽然胸腺脂肪瘤常见于重症肌无力的背景下,甲状腺脂瘤病是一种罕见的疾病,据我们所知,这两种病变与重症肌无力的同时发现从未被报道过.我们强调了两个实体的重要成像特征以及识别它们的临床重要性。
    We present a case of a man with a background of myasthenia gravis who presented with a neck lump, which was diagnosed as thyrolipomatosis in continuity with a very large thymolipoma. Following removal of these lesions, the patient\'s myaesthenic symptoms improved. While thymolipomas are often seen in the context of myasthenia gravis, thyrolipomatosis is a rare entity and to our knowledge the concurrent finding of both lesions with myasthenia gravis has never been reported. We highlight the important imaging features of both entities and the clinical importance of recognising them.
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