Transverse myelitis

横贯性脊髓炎
  • 文章类型: Case Reports
    在SLE患者中,并发NMOSD可表现为视神经炎和横贯性脊髓炎。AQP-4抗体阳性证实诊断。及时治疗对于控制急性症状和预防复发至关重要,正如一名年轻患者的视神经炎和广泛的脊髓病变所强调的那样。
    视神经脊髓炎谱系障碍(NMOSD)是一种罕见的中枢神经系统自身免疫性疾病,影响视神经和脊髓。它与针对水通道蛋白4(AQP-4)和/或髓鞘少突胶质细胞糖蛋白的自身抗体相关。它是根据临床诊断的,放射学,和血清学标准,并在急性期用免疫抑制剂治疗。长期免疫抑制对于预防潜在的复发至关重要。在这个案例报告中,我们介绍了一个19岁女性患者的系统性红斑狼疮(SLE),她的左眼出现模糊和视力丧失。光学相干断层扫描正常,但是钆增强的颈背MRI显示多个病变从脑干延伸到C7-T1交界处,提示纵向广泛的横贯性脊髓炎(LETM),其中最大的是颈脊髓交界处的囊性病变。造影剂注射也显示左视神经炎。脑脊液分析显示IgG和红细胞计数升高,但没有寡克隆带。患者AQP-4自身抗体检测呈阳性,确认NMOSD的诊断。静脉注射甲基强的松龙治疗导致部分改善,但是病人出现了严重的神经症状,包括四肢瘫痪和膀胱和肠功能障碍。此病例说明在鉴别诊断存在视神经炎和/或脊髓炎的SLE患者中考虑NMOSD的重要性。尤其是当MRI检查结果提示LETM时。早期诊断和坚持治疗对于防止进一步复发和有害后遗症至关重要。
    UNASSIGNED: In patients with SLE, concurrent NMOSD can manifest with optic neuritis and transverse myelitis. AQP-4 antibody positivity confirms the diagnosis. Prompt treatment is critical to manage the acute symptoms and prevent relapses, as highlighted by a young patient\'s case with optic neuritis and extensive spinal cord lesions.
    UNASSIGNED: Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disorder of the central nervous system that affects the optic nerve and spinal cord. It is associated with autoantibodies against aquaporin-4 (AQP-4) and/or myelin oligodendrocytes glycoproteins. It is diagnosed based on clinical, radiological, and serological criteria, and treated with immunosuppressants in the acute phase. Long-term immunosuppression is essential to prevent potential relapses. In this case report, we present the case of a 19-year-old female patient with systemic lupus erythematosus (SLE), who presented with blurriness and loss of vision in her left eye. Optical coherence tomography was normal, but a gadolinium-enhanced cervico-dorsal MRI showed multiple lesions extending from the brainstem to the C7-T1 junction suggestive of longitudinally extensive transverse myelitis (LETM), the largest of which was a cystic lesion at the cervico-spinal junction. A contrast injection also revealed left optic neuritis. Cerebrospinal fluid analysis showed elevated IgG and red blood cell count, but no oligoclonal bands. The patient tested positive for AQP-4 autoantibodies, confirming the diagnosis of NMOSD. Treatment with intravenous methylprednisolone led to partial improvement, but the patient experienced a relapse with severe neurological symptoms, including tetraplegia and bladder and bowel dysfunction. This case illustrates the importance of considering NMOSD in the differential diagnosis of patients with SLE who present with optic neuritis and/or myelitis, especially when MRI findings are suggestive of LETM. Early diagnosis and adherence to treatment are crucial to prevent further relapses and deleterious sequelae.
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  • 文章类型: Case Reports
    Loeys-Dietz综合征(LDS)是一种非常罕见的常染色体显性遗传的结缔组织疾病,以心血管的参与为特征,肌肉骨骼,和皮肤系统,随着畸形的面部特征。目前,关于这种疾病的数据有限。该病例提供了一个17岁男孩的临床观察,该男孩患有感觉运动性轻瘫,并经遗传证实为LDS。LDS的主要症状包括动脉瘤,动脉弯曲,超端粒,和双歧悬垂。然而,并不是所有的病人都有这种症状。遗传确认对于准确诊断至关重要。LDS的预后不同于其模仿,比如马凡氏综合症,Beals综合征,Ehlers-Danlos综合征,和Shprintzen-Goldberg综合征.该疾病的管理需要多学科方法来解决其各种表现。这种方法可以帮助增加预期寿命并改善这些患者的生活质量。
    Loeys-Dietz syndrome (LDS) is a very rare connective tissue disorder with autosomal dominant inheritance, characterized by the involvement of the cardiovascular, musculoskeletal, and cutaneous systems, along with dysmorphic facial features. Currently, there are limited data regarding this disease. This case presents a clinical observation of a 17-year-old boy with acute onset of sensorimotor paraparesis and genetically confirmed LDS. The predominant symptoms of LDS include arterial aneurysms, arterial tortuosity, hypertelorism, and bifid uvula. However, this constellation of symptoms is not found in all patients with the disease. Genetic confirmation is essential for an accurate diagnosis. The prognosis for LDS differs from its mimics, such as Marfan syndrome, Beals syndrome, Ehlers-Danlos syndrome, and Shprintzen-Goldberg syndrome. Management of the disease warrants a multidisciplinary approach to address its various manifestations. Such an approach can help increase the life expectancy and improve the quality of life for these patients.
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  • 文章类型: Case Reports
    背景:实验研究表明,重复的经脊髓磁刺激(TsMS)可减少脱髓鞘,并使脊髓损伤(SCI)后恢复。然而,TsMS在SCI患者中的有用性仍不清楚.因此,这项研究的主要目的是评估TsMS联合运动疗法对SCI症状的影响。我们描述了由于视神经脊髓炎(NMO)相关的横贯性脊髓炎引起的SCI患者的TsMS和运动疗法的治疗方案。
    方法:一名患有NMO谱系障碍的23岁白人男性于2014年开始出现症状,包括腰椎疼痛,并在两个下肢逐渐失去力量和敏感性。五个月后,症状自发改善,没有感觉运动缺陷。两年后,2016年,症状复发,双下肢力量和敏感性完全丧失。最初,在15个疗程中提供了物理治疗,目标是恢复运动感觉,改善平衡和功能移动.随后,TsMS(10Hz,600个脉冲,20秒列车间间隔,在12个疗程的理疗前,在椎旁肌静息运动阈值的90%)应用于第10个胸椎棘突。在三个时间点评估结果:仅在物理治疗之前(T-1),在第一次TsMS联合运动疗法(T0)之前,12次TsMS联合运动疗法(T1)后。患者的步行独立性改善了25%,平衡改善125%,功能流动性提高18.8%。患者总体变化印象量表将患者的总体变化印象评估为“大大改善”。
    结论:TsMS联合运动疗法可以安全有效地改善平衡,独立行走,与NMO相关的横贯性脊髓炎引起的SCI患者的功能活动性。
    BACKGROUND: Experimental studies have shown that repetitive trans-spinal magnetic stimulation (TsMS) decreases demyelination and enables recovery after spinal cord injury (SCI). However, the usefulness of TsMS in humans with SCI remains unclear. Therefore, the main objective of this study is to evaluate the effects of TsMS combined with kinesiotherapy on SCI symptoms. We describe a protocol treatment with TsMS and kinesiotherapy in a patient with SCI due to neuromyelitis optica (NMO)-associated transverse myelitis.
    METHODS: A 23-year-old white male with NMO spectrum disorders started symptoms in 2014 and included lumbar pain evolving into a mild loss of strength and sensitivity in both lower limbs. Five months later, the symptoms improved spontaneously, and there were no sensorimotor deficits. Two years later, in 2016, the symptoms recurred with a total loss of strength and sensitivity in both lower limbs. Initially, physiotherapy was provided in 15 sessions with goals of motor-sensory recovery and improving balance and functional mobility. Subsequently, TsMS (10 Hz, 600 pulses, 20-seconds inter-trains interval, at 90% of resting motor threshold of the paravertebral muscle) was applied at the 10th thoracic vertebral spinous process before physiotherapy in 12 sessions. Outcomes were assessed at three time points: prior to physiotherapy alone (T-1), before the first session of TsMS combined with kinesiotherapy (T0), and after 12 sessions of TsMS combined with kinesiotherapy (T1). The patient showed a 25% improvement in walking independence, a 125% improvement in balance, and an 18.8% improvement in functional mobility. The Patient Global Impression of Change Scale assessed the patient\'s global impression of change as \'much improved\'.
    CONCLUSIONS: TsMS combined with kinesiotherapy may safely and effectively improve balance, walking independence, and functional mobility of patients with SCI due to NMO-associated transverse myelitis.
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  • 文章类型: Case Reports
    横向脊髓炎(TM)是一种经常遇到的住院神经系统疾病,通常有广泛的病因差异,由详细的历史缩小,症状演变的时间剖面,和相关的诊断研究。我们报告了一个罕见的病例,一个39岁的男人,他出现了亚急性发作的头痛和混乱,三天后出现四肢瘫痪和反射障碍。他被诊断出患有与爱泼斯坦-巴尔病毒(EBV)相关的急性纵向广泛性横贯性脊髓炎(LETM),最初表现为链球菌性脑膜炎。由于这两种病因的报道不足,我们将我们的病例与文献中的少数类似病例进行了比较,以指导对与EBV和链球菌性脑膜炎相关的副传染性TM的临床和放射学发现的讨论.读者将面临挑战,将我们患者的脊髓炎归因于这些副感染源之一,并鼓励他们在急性环境中评估罕见的感染性病因。
    Transverse myelitis (TM) is a frequently encountered inpatient neurological condition, usually with a broad differential of etiologies narrowed down by detailed history, temporal profile of symptom evolution, and pertinent diagnostic studies. We report a rare case of a 39-year-old man who presented with subacute onset of headaches and confusion, and three days later developed quadriplegia and areflexia. He was diagnosed with acute longitudinally extensive transverse myelitis (LETM) related to Epstein-Barr virus (EBV) superimposed on an initial presentation of streptococcal meningitis. As both etiologies are under-reported, we compare our case to the few similar cases in the literature to guide discussion of the clinical and radiologic findings of parainfectious TM related to EBV and streptococcal meningitis. Readers will have the challenge of attributing our patient\'s myelitis to one of these parainfectious sources and are encouraged to evaluate for rare infectious etiologies in acute settings.
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  • 文章类型: Case Reports
    急性横贯性脊髓炎(ATM)是一种由多种病因引起的炎症性疾病,从感染后到自身免疫。很少,在COVID-19感染和COVID-19疫苗接种后,都报告了ATM病例。我们描述了我们在COVID-19感染后使用ATM的经验,并进行了文献综述。
    我们报告了1例COVID19感染后纵向广泛ATM,他还接受了恢复期血浆疗法,并对COVID-19感染和COVID-19疫苗接种后报告的ATM病例进行了全面的文献综述。使用PubMed和Google学者进行文献检索,并提供关键字和经过同行评审的文章。搜索包括2020年1月至2022年9月的所有案例。
    共有60例ATM病例报告与COVID19后感染有关,23例ATM病例报告与COVID19疫苗接种后相关。在新冠肺炎后ATM病例中,报告的平均年龄为49岁,年龄最小的为7个月.总共55%(33)是纵向广泛的ATM。最常见的症状是下肢无力。活检报告1例坏死性脊髓炎,另一例重叠有GBS综合征和纵向ATM。没有病例报告感染后使用恢复期血浆治疗。几乎所有的ATM病例都用类固醇治疗,但有些病例需要额外的治疗,因为并非所有病例都有充分的反应.6例(10%)对类固醇加血浆置换有反应,5例(8%)对类固醇+IVIG有反应,尤其是在儿科年龄组。1例报告用依库珠单抗治疗后出现阳性反应,另一种是英夫利昔单抗。2例(3%)仍处于旁状态。在covid-19疫苗后的ATM病例中,4例(17%)报告为纵向广泛ATM。5例(21%)在接种疫苗后一周内出现症状。几乎所有人都报告了对类固醇的反应,除了一例在疫苗接种后第58天报告死亡。
    ATM,在急性COVID-19感染的情况下,已在多个病例中被描述,是COVID-19疫苗接种的罕见并发症。
    Acute transverse myelitis (ATM) is an inflammatory disorder caused by many etiologies, from postinfectious to autoimmune. Rarely, ATM cases have been reported after both COVID-19 infection and COVID-19 vaccination. We described our experience with ATM after COVID-19 infection and conducted a literature review.
    UNASSIGNED: We reported a case of longitudinally extensive ATM after COVID 19 infection, who also received convalescent plasma therapy, and present a comprehensive literature review of ATM cases reported after COVID-19 infection and COVID-19 vaccination. The literature search was done using PubMed and Google scholar with keywords and selected peer-reviewed articles. The search included all cases from Jan 2020 to Sept 2022.
    UNASSIGNED: A total of 60 ATM cases reported association with post COVID 19 infection, and 23 ATM cases reported association with post COVID 19 vaccinations. Among post COVID 19 ATM cases, the mean age was 49 years and the youngest reported was 7-month-old. A total of 55% (33) were longitudinally extensive ATM. The most common symptom was lower extremity weakness. One case was reported as necrotizing myelitis on biopsy, and another case overlapped with syndrome of GBS and longitudinal ATM. No cases reported using convalescent plasma therapy after infection. Almost all the ATM cases were treated with steroids, but some cases needed additional treatment since not all responded adequately. Six cases (10%) responded with steroids plus plasmapheresis, and 5 cases (8%) responded with steroids + IVIG, especially in the pediatric age group. One case reported a positive response after treatment with eculizumab, and another with infliximab. Two cases (3%) remained paraparetic. Among post covid-19 vaccine ATM cases, 4 cases (17%) were reported as longitudinally extensive ATM. Five cases (21%) had symptom onset within a week after vaccination. Almost all reported a response to steroids except for one case which reported fatality after the 58th day after vaccination.
    UNASSIGNED: ATM, in the setting of acute COVID-19 infection, has been described in multiple cases and is a rare complication of COVID-19 vaccination.
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  • 文章类型: Case Reports
    结节病引起的LETM代表结节病的一种罕见但危及生命的神经系统表现,以脊髓炎症为特征,和相关的神经缺陷。结节病应纳入LETM的鉴别诊断,特别是在没有肺部受累的患者中。及时识别和管理是优化结果和防止长期残疾的必要条件。
    结节病是一种多系统炎症性肉芽肿性疾病,其特征是形成非干酪样肉芽肿。虽然结节病通常会影响皮肤,淋巴结,和肺,结节病的神经系统受累也有报道。纵向广泛性横贯性脊髓炎(LETM)是一种罕见但有据可查的严重表现。我们报告了一例由结节病引起的LETM病例,该病例患有进行性双侧下肢无力的53岁男性,尿潴留,和感觉异常.实验室评估显示炎症标志物升高。脊柱的磁共振成像显示与横贯性脊髓炎一致的高强度信号。脑脊液分析显示淋巴细胞增多和蛋白质水平升高。胸部CT显示肺门淋巴结肿大。胸内淋巴结活检显示非干酪性肉芽肿与结节病一致。在排除所有其他可能的病因后,对结节病引起的LETM进行了诊断。开始大剂量泼尼松后病情逐渐好转,霉酚酸酯,和康复策略。我们的病例强调了结节病引起的LETM的及时诊断和管理的重要性,并强调结节病必须包括在LETM的鉴别诊断中。尤其是在没有肺部受累的情况下。
    UNASSIGNED: Sarcoidosis-induced LETM represents a rare but life-threatening neurological manifestation of sarcoidosis, characterized by spinal cord inflammation, and associated neurological deficits. Sarcoidosis should be included in the differential diagnosis of LETM, particularly in patients with no lung involvement. Prompt recognition and management are obligatory to optimize outcomes and prevent long-term disability.
    UNASSIGNED: Sarcoidosis is a multisystem inflammatory granulomatous disorder characterized by the formation of noncaseating granulomas. Although sarcoidosis commonly affects the skin, lymph nodes, and lungs, neurological involvement of sarcoidosis has also been reported. Longitudinally extensive transverse myelitis (LETM) is a rare but well-documented serious manifestation of neuroscoidosis. We report a case of LETM caused by sarcoidosis in a 53-year-old male who presented with progressive bilateral lower extremity weakness, urinary retention, and paresthesia. Laboratory evaluations revealed elevated inflammatory markers. Magnetic resonance imaging of the spine showed hyperintense signals consistent with transverse myelitis. Cerebrospinal fluid analysis revealed lymphocytic pleocytosis and elevated protein levels. Chest computed tomography showed hilar lymphadenopathy. A biopsy of the intrathoracic lymph node showed noncaseating granulomas consistent with sarcoidosis. A diagnosis of sarcoidosis-induced LETM was made after ruling out all other possible etiologies. His condition improved gradually after starting high-dose prednisone, mycophenolate, and rehabilitation strategies. Our case underscores the importance of prompt diagnosis and management of sarcoidosis-induced LETM and highlights that sarcoidosis must be included among differential diagnoses of LETM, especially in cases with no lung involvement.
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  • 文章类型: Case Reports
    BACKGROUND: Transverse myelitis (TM) is a demyelinating inflammatory disease that presents with motor, sensory, and autonomic dysfunction, which may be acute or subacute. COVID-19-associated TM has been described in a scarce number of patients.
    METHODS: A 15-year-old previously healthy male patient with respiratory disease before his neurological deterioration presented to the emergency room after developing a complete medullary syndrome located at the cervical-dorsal level, with ascending and symmetric paraparesis that rapidly progressed to paraplegia, with sensory dysfunction from the T3 level, sphincter dysfunction and sudden ventilatory deterioration that required mechanical ventilation. Magnetic resonance imaging was compatible with acute TM. Inflammatory and non-inflammatory etiologies were discarded. In addition, a positive severe acute respiratory syndrome coronavirus 2 test was obtained. Treatment included steroid pulses and plasmapheresis, with an insidious evolution.
    CONCLUSIONS: COVID-19 is an infrequent cause of TM and should be suspected when other etiologies have been ruled out.
    UNASSIGNED: La mielitis transversa (MT) es una enfermedad inflamatoria desmielinizante que se presenta con disfunción motora, sensitiva y autonómica, de forma aguda o subaguda. La MT asociada al COVID-19 se ha escrito en un escaso número de pacientes.
    UNASSIGNED: Se presenta el caso de un masculino de 15 años previamente sano, quien cursaba con un cuadro respiratorio y que desarrollo un deterioro neurológico súbito que involucro un síndrome medular completo localizado en el nivel cérvico dorsal, con paraparesia simétrica que progreso a la paraplejia, con disfunción sensitiva desde el nivel medular de T3, disfunción de esfínteres y deterioro ventilatorio que requirió manejo avanzado de la vía aérea. Su resonancia magnética fue compatible con mielitis transversa aguda. Se descartaron causas inflamatorias y no inflamatorias de la patología. Además, se obtuvo un resultado positivo de SARS-COV-2. Se inició tratamiento con pulsos de metilprednisolona y plasmaféresis, con una evolución insidiosa.
    UNASSIGNED: El COVID-19 es una causa infrecuente de MT y debe sospecharse cuando otras causas han sido descartadas.
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  • 文章类型: Case Reports
    我们报告了一名55岁男子在接种2019年冠状病毒病(COVID-19)后患有多症状性横贯性脊髓炎的病例。根据病程以及影像学和实验室检查的结果诊断患者。我们排除了其他最可能的疾病原因。诊断的快速开始允许静脉类固醇的早期治疗,然后血浆置换和使用生物反馈平台的现代康复方法的实施,其中,还有一个外骨骼.病人重返工作岗位,但由于持续的症状损害了患者的生活质量,康复过程一直持续到今天。
    We report the case of a 55-year-old man with multi-symptomatic transverse myelitis after vaccination against coronavirus disease 2019 (COVID-19). The patient was diagnosed based on the course of the disease and the results of imaging and laboratory tests. We excluded other most probable causes of the disease. The quick start of diagnosis allowed for early treatment with intravenous steroids and then plasmapheresis and the implementation of modern rehabilitation methods using biofeedback platforms, among others, and an exoskeleton. The patient returned to work, but the rehabilitation process continues to this day due to persistent symptoms that impair the patient\'s quality of life.
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  • 文章类型: Case Reports
    脊髓结节病是一种罕见的疾病,可表现为纵向广泛的横贯性脊髓炎。目前的成像可能表明这种病理,但最终的诊断依赖于组织学发现.教学要点:考虑神经结节病在纵向广泛横贯性脊髓炎的鉴别诊断。
    Spinal cord sarcoidosis is a rare condition that can present as a longitudinally extensive transverse myelitis. Current imaging may suggest this pathology, but the final diagnosis relies on the histologic findings. Teaching point: Considering neurosarcoidosis in the differential diagnosis of longitudinally extensive transverse myelitis.
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  • 文章类型: Case Reports
    急性播散性脑脊髓炎(ADEM)和横向脊髓炎(TM)属于获得性脱髓鞘综合征的免疫介导疾病。在病例报告和病例系列中的各种疫苗接种后,两者都已在时间关联中进行了描述,并在观察性研究中进行了评估。全球疫苗数据网络(GVDN)最近进行的一项分析发现,腺病毒载体ChAdOx1nCoV-19(AZD1222)和mRNA-1273疫苗后,ADEM和TM病例过多。与大流行前的历史预期背景率相比。建议进一步的流行病学研究来探索这些潜在的关联。我们利用了澳大利亚疫苗数据链,疫苗安全健康链接(VSHL)为此进行自控病例系列分析。选择VSHL进行此分析,因为VSHL数据用于GVDN关联研究,它们未包括在GVDN观察到的预期分析中.VSHL数据集包含来自澳大利亚免疫登记册的疫苗接种记录,以及维多利亚州承认的事件数据集的670万人的住院记录。这些数据集用于确定COVID-19疫苗接种后42天风险窗口中G040(ADEM)和G373(TM)ICD-10-AM编码入院的相对发生率(RI),与风险窗口两侧的对照期相比。我们观察到ChAdOx1腺病毒载体COVID-19疫苗接种与ADEM(所有剂量RI:3.74[95CI1.02,13.70])和TM(剂量1RI:2.49[95CI:1.07,5.79])事件入院之间的关联。未观察到COVID-19疫苗mRNA与ADEM或TM之间的关联。这些发现转化为疫苗接种后ADEM(0.78/百万剂)和TM(1.82/百万剂)的绝对风险极小;任何ADEM或TM的潜在风险都应与疫苗接种COVID-19疾病及其并发症的公认保护性益处相权衡。这项研究证明了GVDN合作利用大量人口来检查有关罕见结局的重要疫苗安全性问题的价值。以及相关人口水平数据集的价值,比如VSHL,快速探索已识别的关联。
    Acute Disseminated Encephalomyelitis (ADEM) and Transverse Myelitis (TM) are within the group of immune mediated disorders of acquired demyelinating syndromes. Both have been described in temporal association following various vaccinations in case reports and case series and have been evaluated in observational studies. A recent analysis conducted by The Global Vaccine Data Network (GVDN) observed an excess of ADEM and TM cases following the adenoviral vectored ChAdOx1 nCoV-19 (AZD1222) and mRNA-1273 vaccines, compared with historically expected background rates from prior to the pandemic. Further epidemiologic studies were recommended to explore these potential associations. We utilized an Australian vaccine datalink, Vaccine Safety Health-Link (VSHL), to perform a self-controlled case series analysis for this purpose. VSHL was selected for this analysis as while VSHL data are utilised for GVDN association studies, they were not included in the GVDN observed expected analyses. The VSHL dataset contains vaccination records sourced from the Australian Immunisation Register, and hospital admission records from the Victorian Admitted Episodes Dataset for 6.7 million people. These datasets were used to determine the relative incidence (RI) of G040 (ADEM) and G373 (TM) ICD-10-AM coded admissions in the 42-day risk window following COVID-19 vaccinations as compared to control periods either side of the risk window. We observed associations between ChAdOx1 adenovirus vector COVID-19 vaccination and ADEM (all dose RI: 3.74 [95 %CI 1.02,13.70]) and TM (dose 1 RI: 2.49 [95 %CI: 1.07,5.79]) incident admissions. No associations were observed between mRNA COVID-19 vaccines and ADEM or TM. These findings translate to an extremely small absolute risk of ADEM (0.78 per million doses) and TM (1.82 per million doses) following vaccination; any potential risk of ADEM or TM should be weighed against the well-established protective benefits of vaccination against COVID-19 disease and its complications. This study demonstrates the value of the GVDN collaboration leveraging large population sizes to examine important vaccine safety questions regarding rare outcomes, as well as the value of linked population level datasets, such as VSHL, to rapidly explore associations that are identified.
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