polyradiculoneuropathy

多发性神经根神经病
  • 文章类型: Case Reports
    背景:严重的疫苗接种后神经系统并发症很少见。慢性炎症性脱髓鞘性多发性神经病(CIDP)是一种免疫介导的多发性神经病,影响周围神经根,这不是很好地描述为疫苗接种后的副作用。这里,我们提出了一种罕见的SARS-CoV-2疫苗接种并发症,诊断为CIDP。
    方法:一名67岁的糖尿病男性在接受第三剂Sinopharm(BBIBP-CorV)疫苗后出现下肢感觉异常和无力。尽管最初被解雇为糖尿病并发症,症状升级,影响所有四肢。肌电图研究显示异常自发活动伴有慢性神经支配改变,这在下肢更为显著。根据临床过程,射线成像,和实验室数据,建立了具有严重轴突脱髓鞘特征的CIDP的诊断。静脉注射免疫球蛋白(IVIg)治疗,泼尼松龙,硫唑嘌呤和硫唑嘌呤可明显改善上肢,但下肢远端肌肉的恢复有限。
    结论:尽管CIDP是COVID-19疫苗接种后的一种罕见并发症,在鉴别诊断中应考虑。及时诊断疫苗诱导的CIDP具有挑战性,任何延误都会对受影响患者的治疗反应产生不利影响。
    BACKGROUND: Severe post-vaccination neurological complications are rare. Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is an immune-mediated polyneuropathy affecting the peripheral nerve roots, which is not well described as a post-vaccination side effect. Here, we present a rare complication of vaccination against SARS-CoV-2, reaching a diagnosis of CIDP.
    METHODS: A 67-year-old diabetic male presented with lower extremity paresthesia and weakness following the third dose of the Sinopharm (BBIBP-CorV) vaccine. Despite initial dismissal as a diabetic complication, symptoms escalated, affecting all extremities. Electromyography study revealed abnormal spontaneous activity with chronic reinnervation changes, which was more significant in the lower extremities. Based on the clinical course, radiographic imaging, and laboratory data, a diagnosis of CIDP with severe axonal demyelinating features was established. Treatment with intravenous immunoglobulin (IVIg), prednisolone, and azathioprine resulted in marked improvement of the upper extremities but limited recovery in distal lower extremity muscles.
    CONCLUSIONS: Although CIDP is a rare complication following COVID-19 vaccination, it should be considered in the differential diagnosis. Timely diagnosis of vaccine-induced CIDP is challenging, and any delay can adversely affect treatment response in affected patients.
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  • 文章类型: Case Reports
    布鲁氏菌病,由布鲁氏菌引起的人畜共患疾病,构成了重大的全球健康问题。在其多样化的临床表现中,神经布鲁氏菌病仍然是一种罕见但令人衰弱的并发症。这里,我们介绍了一例罕见的神经布鲁氏菌病病例,在一名45岁女性中表现异常。患者的临床过程包括进行性下肢无力,肌肉萎缩,和双重视觉,促使全面的诊断评估。值得注意的发现包括多发性神经病,脑脊液和血液中布鲁氏菌凝集滴度升高,异常EMG-NCV测试,用抗生素治疗缓解症状。临床表现,诊断挑战,并讨论了与其他神经系统疾病的区别。该病例强调了在布鲁氏菌病流行的地区考虑神经布鲁氏菌病的重要性,并强调了这种罕见的神经系统并发症的独特临床和放射学特征。早期识别和适当的治疗对于减轻与神经布鲁氏菌病相关的显著发病率至关重要。
    Brucellosis, a zoonotic disease caused by Brucella species, poses a significant global health concern. Among its diverse clinical manifestations, neurobrucellosis remains an infrequent yet debilitating complication. Here, we present a rare case of neurobrucellosis with unusual presentations in a 45-year-old woman. The patient\'s clinical course included progressive lower extremity weakness, muscle wasting, and double vision, prompting a comprehensive diagnostic evaluation. Notable findings included polyneuropathy, elevated brucella agglutination titers in both cerebrospinal fluid and blood, abnormal EMG-NCV tests, and resolving symptoms with antibiotic therapy. The clinical presentation, diagnostic challenges, and differentiation from other neurological conditions are discussed. This case underscores the importance of considering neurobrucellosis in regions where brucellosis is prevalent and highlights this rare neurological complication\'s distinctive clinical and radiological features. Early recognition and appropriate treatment are crucial to mitigate the significant morbidity associated with neurobrucellosis.
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  • 文章类型: Case Reports
    尽管取得了进展,在非专业医疗中心,伴有运动和感觉受累的炎性脱髓鞘性多神经根性神经炎的诊断和相关治疗仍然具有挑战性.我们描述了一个7岁女孩入院的案例,患有虚弱,头痛,轻度弥漫性疼痛,步态紊乱,下肢力量下降。一进入我们的医院中心,除了血液检查和大脑和髓质磁共振成像,我们对她的脑脊液进行了分析.该测试证实了白蛋白细胞学解离和肌电图,提示诊断为急性炎性脱髓鞘性多发性神经病并伴有运动和感觉受累。该儿童成功接受了多价免疫球蛋白和神经运动理疗。本报告强调了临床医生不仅要关注他们的方案培训,还要关注正确关联所需测试结果的能力。患者的症状。
    Despite progress, the diagnosis and relative management of inflammatory demyelinating polyradiculoneuritis with motor and sensory involvement remain challenging in non-specialized medical centers. We describe the case of a seven-year-old girl admitted to our hospital, with asthenia, headaches, mild diffuse pain, gait disturbances, and decreased strength of lower limbs. On admission to our hospital center, in addition to the blood tests and the cerebral and medullary magnetic resonance imaging, we performed an analysis of her cerebrospinal fluid. This test confirmed an albuminocytological dissociation and electromyography, which suggested the diagnosis of acute inflammatory demyelinating polyneuropathy with motor and sensory involvement. The child was successfully treated with polyvalent immunoglobulin and neuromotor physiotherapy. This report highlights the importance for the clinician not to focus only on their protocols training but also on the ability to relate correctly the results of the required tests, to the patient\'s symptoms.
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  • 文章类型: Case Reports
    爱泼斯坦-巴尔病毒(EBV)是一种自古以来就感染人类的DNA病毒,能够引起广泛的病理并影响大约90%的人口。一名61岁男性,无明显病史,有5天的失衡和行走困难史。神经系统检查揭示了具体的发现,包括缺乏反应,双边匿名,和步态异常。与格林-巴利综合征相比,腰椎穿刺提示中枢神经系统感染。血清学检测证实EB病毒(EBV)阳性,和静脉注射免疫球蛋白导致显著改善。肌电图结果提示炎性/感染性多神经根病。重复EBV血清学,显示强阳性IgG和阴性IgM,确诊为继发于EBV的多发性神经根神经病。此病例强调了EBV罕见的神经系统并发症以及在此类表现中考虑病毒感染的重要性。
    The Epstein-Barr virus (EBV) is a DNA virus that has been infecting humans since ancient times, capable of causing a wide range of pathologies and affecting approximately 90% of the population. A 61-year-old male with no significant medical history presented with a 5-day history of imbalance and difficulty walking. Neurological examination revealed specific findings, including absent reflexes, bilateral asynergy, and gait abnormalities. Contrasting with Guillain-Barré Syndrome, lumbar puncture suggested a central nervous system infection. Serological testing confirmed Epstein-Barr virus (EBV) positivity, and intravenous immunoglobulin led to significant improvement. Electromyogram results suggested inflammatory/ipnfectious polyradiculopathy. Repeat EBV serology, showing strongly positive IgG and negative IgM, confirmed the diagnosis of Polyradiculoneuropathy secondary to EBV. This case underscores the rare neurological complications of EBV and the importance of considering viral infections in such presentations.
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  • 文章类型: Journal Article
    背景:我们提出了一个绅士的案例,他发展得迅速进行性视力丧失,眼瘫,严重颅内高压的弛缓性四肢轻瘫。我们回顾了文献中的可用病例,以提高对这种罕见临床实体的认识。病例报告:一名36岁的男子发展迅速进行性视力丧失,眼瘫,和弛缓性四肢轻瘫.他做了大量的检查,只有严重的颅内高压才值得注意,>55厘米的H2O。没有炎症特征,患者对脑脊液分流有反应。文献中很少有类似的案例,但均显示与广泛的神经轴功能障碍相关的颅内压明显升高。同样,这些患者在CSF转流后有所改善,但似乎对免疫疗法无反应.
    结论:我们将这种广泛的神经轴功能障碍颅内高压与多颅神经根神经病(IHP)相关,并将其与类似的免疫介导的临床表现区分开来。临床医生应该意识到这种潜在破坏性临床表现的不同病因,以告知适当和及时的治疗。
    BACKGROUND: We present the case of a gentleman who developed rapidly progressive vision loss, ophthalmo-paresis, and flaccid quadriparesis in the context of severe intracranial hypertension. We reviewed the available cases in the literature to increase awareness of this rare clinical entity.Case Report:A 36-year-old man developed rapidly progressive vision loss, ophthalmo-paresis, and flaccid quadriparesis. He had an extensive workup, only notable for severe intracranial hypertension, >55 cm of H 2 O. No inflammatory features were present, and the patient responded to CSF diversion. Few similar cases are available in the literature, but all show markedly elevated intracranial pressure associated with extensive neuroaxis dysfunction. Similarly, these patients improved with CSF diversion but did not appear to respond to immune-based therapies.
    CONCLUSIONS: We term this extensive neuroaxis dysfunction intracranial hypertension associated with poly-cranio-radicular-neuropathy (IHP) and distinguish it from similar immune-mediated clinical presentations. Clinicians should be aware of the different etiologies of this potentially devastating clinical presentation to inform appropriate and timely treatment.
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  • 文章类型: Case Reports
    黑色素瘤是一种侵袭性恶性肿瘤,历史上的特点是预后差,治疗选择少。BRAF和MEK抑制剂靶向治疗的出现,以及免疫疗法,改变了这种情况,改善了BRAFV600E突变患者的预后.这些疗法通常具有良好的耐受性。神经毒性,尤其是多神经根病,BRAF抑制剂和MEK抑制剂非常罕见,尽管近年来已经描述了一些病例,无论使用的目标疗法组合的类型。
    我们报告了一例BRAFV600E突变的转移性黑色素瘤患者,该患者接受达拉非尼和曲美替尼治疗,出现了脱髓鞘性多神经根神经病。
    这种情况下,再一次,应该提请我们注意罕见的可能性,但潜在的严重副作用,即使在一般耐受性良好的治疗的情况下。特别是在有副作用的情况下,临床医师和患者之间的密切关系对于不良事件的管理和最佳治疗策略的选择非常重要.
    UNASSIGNED: Melanoma is an aggressive malignancy, historically characterized with a poor prognosis and few treatment options. The advent of target therapy with BRAF and MEK inhibitors, as well as immunotherapy, changed this scenario and improved the prognosis of patients with BRAF V600E mutation. These therapies are generally well tolerated. Neurological toxicities, especially polyradiculopathy, are very rare with BRAF inhibitors and MEK inhibitors although some cases have been described in recent years, regardless of the type of target therapies combination used.
    UNASSIGNED: We report the case of a patient with BRAF V600E-mutated metastatic melanoma treated with dabrafenib and trametinib who has developed a demyelinating polyradiculoneuropathy.
    UNASSIGNED: This case, once more, should draw our attention to the possibility of rare, but potentially serious side effects, even in the case of generally well-tolerated treatments. Especially in the presence of side effects, it is important a close relationship between clinicians and patients for the management of adverse events and the choice of the best treatment strategy.
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  • 文章类型: Journal Article
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  • 文章类型: Case Reports
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  • 文章类型: Review
    Guillain-Barré syndrome (GBS) is an immune-mediated disease of the peripheral nervous system that can occur in both children and adults. The classic presentation of GBS is characterized by progressive symmetrical, ascending muscle weakness. Patients with GBS require meticulous monitoring due to the risk of bulbar syndrome, respiratory failure and autonomic dysfunction, which can be life-threatening. Early diagnosis and timely prescription of pathogenetic therapy for GBS are particularly important, especially in young children. Meanwhile, the spectrum of disorders covered by GBS has expanded significantly; its eponym is now designate any variant of acute dysimmune polyneuropathy, and its atypical forms pose a serious diagnostic problem for clinicians. This review article provides an analysis of the data available in the medical literature on GBS in children and discusses the tactics for diagnosing and managing patients with GBS, taking into account the Russian and European clinical recommendations.
    Синдром Гийена—Барре (СГБ) — иммуноопосредованное заболевание периферической нервной системы, встречающееся как у детей, так и у взрослых. Основным проявлением СГБ является симметричная прогрессирующая и восходящая мышечная слабость. Пациенты с СГБ имеют высокий риск развития бульбарного синдрома, дыхательной недостаточности и вегетативной дисфункции, создающих угрозу жизни пациенту. Ранняя диагностика и своевременное назначение патогенетической терапии при СГБ исключительно актуальны. Спектр расстройств, охватываемых рубрикой СГБ, значительно расширился, его эпоним стал применяться для обозначения любого варианта острой дизиммунной полинейропатии, а его атипичные формы представляют серьезную диагностическую проблему. В статье приведен анализ имеющихся данных о СГБ у детей, обсуждается тактика диагностики и ведения пациентов с СГБ с учетом отечественных и зарубежных клинических рекомендаций.
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  • 文章类型: Journal Article
    背景:由于潜在的浆细胞肿瘤,POEMS综合征是一种危及生命的疾病。该综合征的主要标准是多发性神经根神经病,克隆浆细胞疾病,硬化骨病变,血管内皮生长因子升高,还有Castleman病的存在.次要特征包括器官肿大,内分泌病,特征性皮肤变化,乳头水肿,血管外容量超负荷,和血小板增多症。
    方法:POEMS综合征的诊断有三个主要标准,其中两个必须包括多发性神经根神经病和克隆性浆细胞疾病,和至少一个次要标准。
    方法:由于该综合征的发病机制尚未完全了解,风险分层仅限于临床表型,而不是特定的分子标志物。危险因素包括低血清白蛋白,年龄,胸腔积液,肺动脉高压,并降低估计的肾小球滤过率。
    方法:对于患有显性浆细胞瘤的患者,一线治疗是放疗。弥漫性硬化病变或播散性骨髓受累的患者应接受全身治疗。皮质类固醇是暂时的,但是烷化剂和来那度胺是治疗的支柱,前者以低剂量常规治疗或高剂量预处理的形式用于干细胞移植。沙利度胺和硼替佐米也有活性,但需要权衡其获益与加重周围神经病变的风险.根据病例系列,Daratumumab组合似乎也很有希望。及时识别和建立针对浆细胞的支持性护理措施和治疗可获得最佳结果。
    POEMS syndrome is a life-threatening condition due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder, sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis.
    The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma cell disorder, and at least one of the minor criteria.
    Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced estimated glomerular filtration rate.
    For those patients with a dominant plasmacytoma, first-line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement should receive systemic therapy. Corticosteroids are temporizing, but alkylators and lenalidomide are the mainstays of treatment, the former either in the form of low-dose conventional therapy or as high-dose conditioning for stem cell transplantation. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. Daratumumab combinations also appear promising based on case series. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes.
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