Transverse myelitis

横贯性脊髓炎
  • 文章类型: Journal Article
    背景:本研究的目的是评估横贯性脊髓炎(TM)患者的抗髓鞘少突胶质细胞糖蛋白(MOG)抗体的状态,并比较MOG免疫球蛋白G(IgG)阳性与阴性病例的临床和影像学特征。方法:这项队列研究招募了71例确诊为新发TM的患者,这些患者在伊斯法罕的转诊大学诊所接受随访。伊朗,从2016年11月到2019年1月。用于抗MOG的磁共振成像(MRI)图像和血液样本,抗水通道蛋白4(抗AQP4)(使用基于细胞的技术),并收集患者的血管炎相关抗体.结果通过扩展残疾状态量表(EDSS)评分的演变以及三个月内的脑和脊髓影像学检查结果进行评估。作为随访,所有患者在平均一年的时间内进行了影像学和临床评估。我们比较了MOG-IgG阳性和阴性病例的临床和放射学结果特征。结果:在研究的总人口中,有26.8%的男性和73.2%的女性,平均年龄33±10岁。MOG抗体阳性12例(16.9%),抗AQP4抗体阳性17例(89.5%)。抗MOG抗体阳性与年龄无显著相关性,性别分布,其他自身免疫性疾病的存在,以及复发的次数和间隔。然而,血清阴性和血清阳性患者首次成像时脊柱受累部位有显著差异.结论:在MOG抗体病(MOG-AD)TM患者中,MRI结果表明,在血清阳性病例中,颈胸段优先受累,这可能有助于与非MOG脱髓鞘TM区分。
    Background: The aim of this study was to evaluate the status of anti-myelin oligodendrocyte glycoprotein (MOG) antibodies in patients with transverse myelitis (TM) and compare the clinical and imaging characteristics of MOG immunoglobulin G (IgG)-positive with negative cases. Methods: This cohort study enrolled 71 patients diagnosed with new-onset of TM who were being followed at a referral university clinic in Isfahan, Iran, from November 2016 to January 2019. Magnetic resonance imaging (MRI) images and blood samples for anti-MOG, anti-aquaporin 4 (anti-AQP4) (using the cell-based technique), and vasculitis-related antibodies were collected from patients. Outcomes were assessed by the evolution of the Expanded Disability Status Scale (EDSS) score and brain and spinal cord imaging findings within three months. All patients underwent imaging and clinical assessment during a mean period of one year as a follow-up. We compared the characteristics of clinical and radiological outcomes in MOG-IgG-positive and negative cases. Results: Of the total population studied, there were 26.8% men and 73.2% women, with a mean age of 33 ± 10 years. 12 (16.9%) patients were seropositive for MOG antibody and 17 (89.5%) were positive for anti-AQP4 antibodies. There was no significant association between anti-MOG antibody seropositivity and age, gender distribution, the presence of other autoimmune diseases, and number and interval of relapses. However, the involvement site of the spine at first imaging was significantly different between seronegative and seropositive patients. Conclusion: In patients with MOG antibody disease (MOG-AD) TM, the MRI findings suggest a preferential involvement of the cervical-thoracic section in seropositive cases which may help differentiate from non-MOG demyelination TM.
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  • 文章类型: Observational Study
    背景:脱髓鞘疾病(DD)是一组与脑或脊髓区域丢失和损伤相关的慢性神经系统疾病。这些情况可能会从生命的早期阶段引发神经功能受损和残疾。关于DD的流行病学数据仍然不足以用于墨西哥医疗保健系统的决策。本研究旨在根据墨西哥国家脱髓鞘疾病登记处的数据描述DD的流行病学。
    方法:横截面,基于注册表,进行了观察性研究。我们分析了408例多发性硬化症的报告(331例,81%),视神经脊髓炎谱系障碍(67,16%),慢性复发性炎性视神经病变(5,1%),临床孤立综合征(4,0.9%),以及2021年报告的自身免疫性脑炎(1.0.2%)。
    结果:从首次出现症状到诊断出任何DD的时间约为3年。在40%的患者中检测到治疗失败史。据估计,NMOSD占所有疾病的20%。有证据表明,使用名牌和仿制IFN药物产品会导致治疗失败的增加。
    结论:我们的研究小组建议加强教育计划和活动的基础上的诊断和临床管理的改进,以首次接触的医生和专科医生,并提高整个人群的认识。
    BACKGROUND: Demyelinating diseases (DD) are a group of chronic neurological diseases associated with loss and injury of brain or spinal cord regions. These conditions could trigger impairment of neurological functions and disability from earlier stages of life. Epidemiological data on DD remains insufficient for decision-making in the Mexican healthcare system. This study aims to describe the epidemiology of DD based on data from Mexico\'s National Registry of Demyelinating Diseases.
    METHODS: A cross-sectional, registry-based, observational study was performed. We analyzed 408 reports of multiple sclerosis (331, 81%), neuromyelitis optica spectrum disorder (67, 16%), chronic recurrent inflammatory optic neuropathy (5, 1%), clinically isolated syndrome (4, 0.9%), and autoimmune encephalitis (1, 0.2%) reported across 2021.
    RESULTS: The time from first symptoms to diagnosis of any DD was about 3 years. A treatment failure history was detected in 40% of patients. It was estimated that NMOSD accounts for 20% of all disorders. There was evidence that the use of brand-name and generic IFN drug products lead to increased therapeutic failures.
    CONCLUSIONS: Our research team suggests reinforcing educational programs and activities based on diagnosis and clinical management improvement to first-contact physicians and specialty doctors and promoting awareness in the whole population.
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  • 文章类型: Journal Article
    这项研究的目的是研究人口统计学,临床,放射学特征,和抗髓鞘少突胶质细胞糖蛋白(MOG)抗体谱障碍的结果,并将这些特征与抗MOG抗体阴性的患者进行比较。MOG抗体相关疾病(MOGAD)和水通道蛋白4(AQP4)抗体相关疾病是免疫学上不同的病理。我们的目的是比较MOG抗体相关疾病与AQP4抗体相关疾病和血清阴性脱髓鞘疾病(非多发性硬化症)的临床和放射学特征。
    这是一项前瞻性和队列研究,于2019年1月至2021年5月在印度北部的顶级三级护理机构进行。我们比较了临床,实验室,和MOGAD患者的放射学发现,AQP4抗体相关疾病,血清阴性脱髓鞘病.
    共有103名患者——41名MOGAD患者,37例AQP4抗体相关疾病和25例血清阴性脱髓鞘疾病。双视神经炎是MOGAD患者中最常见的表型(18/41),而脊髓炎是AQP4(30/37)和血清阴性组(13/25)中最常见的表型。皮质,并皮质病变,眼前段视神经炎,光学鞘增强,和圆锥累及脊髓炎是放射学发现,将MOGAD与AQP4相关疾病分开。两组的最低点扩展残疾状态量表(EDSS)和视敏度相似。与AQP4抗体组相比,MOG抗体组的末次随访EDSS明显更好(1[0-8]vs.3.5[0-8];P=0.03)。脑炎,脊髓炎,癫痫发作在年轻人群中更为常见(<18vs.>18岁)在MOGAD(9vs.2,P=0.001;9vs.7,P=0.03;6vs.0,P=0.001)。
    我们确定了一些临床和放射学特征,可以帮助医生区分MOGAD和AQP4-免疫球蛋白G+视神经脊髓炎谱系障碍。由于两组之间的治疗反应可能有所不同,因此分化至关重要。
    UNASSIGNED: The aim of the study was to study the demographical, clinical, radiological features, and outcome of anti-myelin oligodendrocyte glycoprotein (MOG) antibody spectrum disorder and compare these features with patients negative for anti-MOG antibody. MOG antibody-associated disease (MOGAD) and aquaporin-4 (AQP4) antibody-related diseases are immunologically distinct pathologies. Our aim was to compare the clinical and radiological features of MOG antibody-related diseases with AQP4 antibody-related diseases and seronegative demyelinating diseases (Non-multiple sclerosis).
    UNASSIGNED: This was a prospective and cohort study conducted at an apex tertiary care institute in the northern part of India from Jan 2019 to May 2021. We compared clinical, laboratory, and radiological findings of patients with MOGAD, AQP4 antibody-related diseases, and seronegative demyelinating disease.
    UNASSIGNED: There were a total of 103 patients - 41 patients of MOGAD, 37 patients of AQP4 antibody-related diseases and 25 seronegative demyelinating disease. Bilateral optic neuritis was the most frequent phenotype in patients with MOGAD (18/41) whereas myelitis was the most common phenotype in the AQP4 (30/37) and seronegative groups (13/25). Cortical, juxtacortical lesions, anterior segment optic neuritis, optic sheath enhancement, and conus involvement in myelitis were radiological findings that separated MOGAD from AQP4 related diseases. Nadir Expanded Disability Status Scale (EDSS) and visual acuity were similar across the groups. Last follow-up EDSS was significantly better in the MOG antibody group as compared to AQP4 antibody group (1 [0-8] vs. 3.5 [0-8]; P = 0.03). Encephalitis, myelitis, and seizures were more common in the younger population (<18 vs. >18 years) in MOGAD (9 vs. 2, P = 0.001; 9 vs. 7, P = 0.03; 6 vs. 0, P = 0.001).
    UNASSIGNED: We identified several clinical and radiological features that can help physicians to distinguish MOGAD from AQP4-immunoglobulin G+neuromyelitis optica spectrum disorder. Differentiation is vital as treatment response might vary among both groups.
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  • 文章类型: Journal Article
    BACKGROUND: Central nervous system inflammatory demyelinating diseases (CNSIDDs) have notable interracial heterogeneity. The epidemiology of CNSIDDs in Thailand, a mainland Southeast Asian country, is unknown.
    OBJECTIVE: To determine the cumulative incidence, point prevalence, and disease burden of neuromyelitis optica spectrum disorder (NMOSD) and other CNSIDDs in Thailand using population-based data of Chumphon.
    METHODS: Searching for CNSIDD patients at a public secondary care hospital in Chumphon, the only neurology center in the province, from January 2016 to December 2021 was implemented using relevant ICD-10-CM codes. All diagnoses were individually ascertained by a retrospective chart review. Cumulative incidence, point prevalence, attack rate, mortality rate, and disability-adjusted life years (DALYs) were calculated.
    RESULTS: Aquaporin 4-IgG-positive NMOSD was the most prevalent CNSIDD in the Thai population at 3.08 (1.76-5.38) per 100,000 persons. The prevalence of multiple sclerosis (MS) followed at 0.77 (0.26-2.26) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) at 0.51(0.14-1.87) per 100,000 adults. In the pediatric population, the incidence of acute disseminated encephalomyelitis was 0.28 (0.08-1.02) per 100,000 persons/year. Among other idiopathic demyelinating diseases, idiopathic optic neuritis had the highest incidence at 0.58 (0.24-0.92) per 100,000 persons/year, followed by acute transverse myelitis at 0.44 (0.14-0.74). Idiopathic demyelinating brainstem syndrome was also observed at 0.04 (0.01-0.25) per 100,000 persons/year. Although most had a fair recovery, disability was worst among NMOSD patients with DALYs of 3.61 (3.00-4.36) years per 100,000 persons. Mortality rate was the highest in NMOSD as well.
    CONCLUSIONS: CNSIDDs are rare diseases in Thailand. The prevalence is comparable to that of East Asian populations. A nationwide CNSIDDs registry would better elaborate the epidemiology of these diseases.
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  • 文章类型: Systematic Review
    We aimed to provide insights into transverse myelitis (TM) following COVID-19 by analyzing cases treated at tertiary care neurology centers and a systemic review of the literature.
    The retrospective observational multi-center study was conducted at the four university neurology departments in Croatia, Slovenia, Serbia, and Austria. We searched for acute myelitis cases that occurred during or after COVID-19. A systemic review of the literature on COVID-19 and transverse myelitis was performed.
    We identified 76 persons with TM associated with COVID-19, 13 from the multi-center study and 63 from the literature review. Most of the participants (55.6%) had an intermediate latency, 25.4% had short and 19% long latency from COVID-19 symptoms to TM. The clinical presentation consisted of the typical TM signs. More than half of the participants had inflammatory changes in the CSF, with rare patients having intrathecal OCB synthesis and positive serology for anti-MOG or anti-AQP4 antibodies. Persons with autonomic symptoms and CSF pleocytosis were significantly more common to have an intermediate latency of 8 to 21 days from COVID-19 to TM (p = 0.005 and p = 0.003; respectively). According to logistic regression analysis, only participants with lesions evident on spinal cord MRI compared to normal spinal cord MRI had reduced risks for poor recovery. >80% of participants were treated with a combination of corticosteroids and intravenous immunoglobulins or plasma exchange with 73% having incomplete recovery.
    Our study further characterizes clinical, laboratory, and MRI features, as well as treatment of TM associated with COVID-19.
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  • 文章类型: Journal Article
    神经结节病可以影响脊髓炎相对常见的神经系统的所有部分。这项研究的目的是描述临床特征,可归因于神经结节病的脊髓炎患者的治疗和预后。
    我们对神经结节病相关性脊髓炎进行了回顾性队列研究和系统评价和荟萃分析。
    从2015年到2020年,在我们的诊所发现的153名神经结节病患者中有41名(27%)患有脊髓炎。神经结节病的分类在三个(7%)中明确,29例(71%)可能,9例(22%)可能。发病时的中位年龄(四分位距)为49(41-53)岁,其中20名患者为女性(49%)。出现的症状包括41例患者中的31例(78%)的肌肉无力,35例(88%)感觉丧失和30例(75%)排尿异常。脊柱磁共振成像显示36例患者中有27例(75%)纵向广泛脊髓炎,脑脊液检查显示21例(81%)白细胞计数升高。41例患者中38例(93%)的初始治疗包括糖皮质激素,41例患者中有21例(51%)加用甲氨蝶呤或硫唑嘌呤,41例患者中有10例(24%)加用英夫利昔单抗。治疗导致缓解,39例患者中有37例(95%)的疾病得到改善或稳定。尽管治疗,30例患者中有18例(60%)在随访结束时无法独立行走(中位数为36个月)。对2000年至2020年之间发表的文献的回顾确定了215例具有可比临床特征和辅助研究结果的患者。
    在27%的神经结节病患者中观察到结节病相关脊髓炎。尽管治疗通常会导致疾病活动减少,经常发生导致步行丧失的残余神经功能缺损。
    Neurosarcoidosis can affect all parts of the nervous system of which myelitis is relatively frequent. The aim of this study was to describe clinical characteristics, treatment and prognosis of patients with myelitis attributable to neurosarcoidosis.
    We performed a retrospective cohort study and a systematic review and meta-analysis of neurosarcoidosis-associated myelitis.
    Myelitis was identified in 41 of 153 (27%) neurosarcoidosis patients seen at our clinic from 2015 to 2020. Classification of neurosarcoidosis was definite in three (7%), probable in 29 (71%) and possible in nine patients (22%). The median (interquartile range) age at onset was 49 (41-53) years and 20 of the patients were female (49%). The presenting symptoms included muscle weakness in 31 of 41 patients (78%), sensory loss in 35 (88%) and micturition abnormalities in 30 (75%). Spinal magnetic resonance imaging showed longitudinally extensive myelitis in 27 of 36 patients (75%) and cerebrospinal fluid examination showed an elevated leukocyte count in 21 patients (81%). Initial treatment consisted of glucocorticoids in 38 of 41 patients (93%), with additional methotrexate or azathioprine in 21 of 41 patients (51%) and infliximab in 10 of 41 patients (24%). Treatment led to remission, improvement or stabilization of disease in 37 of 39 patients (95%). Despite treatment, 18 of 30 patients (60%) could not walk independently at the end of follow-up (median 36 months). A review of the literature published between 2000 and 2020 identified 215 patients with comparable clinical characteristics and results of ancillary investigations.
    Sarcoidosis-associated myelitis is observed in 27% of neurosarcoidosis patients. Although treatment often led to a decrease in disease activity, residual neurological deficits leading to loss of ambulation occurred frequently.
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  • 文章类型: Journal Article
    UNASSIGNED: Efficacy and safety of inebilizumab for treatment of neuromyelitis optica spectrum disorder in adults seropositive for aquaporin-4 (AQP4)-immunoglobulin (Ig) G were demonstrated in the 28-week randomized controlled period of the N-MOmentum study.
    UNASSIGNED: To assess efficacy and safety of long-term inebilizumab treatment.
    UNASSIGNED: Post hoc analysis was performed in 75 AQP4-IgG-seropositive participants receiving inebilizumab for ⩾4 years in the randomized controlled period and open-label extension of the N-MOmentum study.
    UNASSIGNED: Eighteen attacks occurred in 13 participants during inebilizumab treatment (annualized attack rate, 0.052 attacks/person-year). Twelve attacks occurred during the first year of treatment, and two each occurred in years 2-4. Disability scores remained stable throughout ⩾4 years of treatment. Inebilizumab was well tolerated, with two (2.7%) serious treatment-emergent adverse events related to inebilizumab and no deaths. Immunoglobulin G levels decreased over time; however, correlation between severe infections and low IgG levels could not be determined because of their small numbers.
    UNASSIGNED: These results from the N-MOmentum study continue to support use of inebilizumab for treatment of neuromyelitis optica spectrum disorder. Furthermore, the findings suggest that efficacy of inebilizumab may be enhanced after the first year of treatment, warranting additional long-term investigation.
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  • 文章类型: Journal Article
    UNASSIGNED: To identify the demographic and clinical characteristics of patients with transverse myelitis (TM) and to compare functional status between those patients and a matched group with traumatic spinal cord injury (T-SCI).
    UNASSIGNED: Retrospective study.
    UNASSIGNED: A tertiary rehabilitation hospital.
    UNASSIGNED: The demographic and clinical characteristics of 484 T-SCI patients and 25 TM patients were compared. Functional status was further analyzed by matching the two groups.
    UNASSIGNED: The matched patients were compared in terms of motor and sensory functions, bladder and bowel symptoms, ambulation level, the Rivermead Mobility Index, and SCI-related medical complications.
    UNASSIGNED: The mean age of the TM patients was 35.6 years and was similar to that of the T-SCI patients. There were significantly more females in the TM group (P = 0.017). Individuals with TM had fewer cervical injuries (P = 0.032) and a higher rate of paraplegia (P = 0.047) and were more often incomplete (P = 0.009) than those with T-SCI. Sensory function was significantly better in the TM group compared to the matched T-SCI group (P = 0.05). Independent ambulation frequency was higher in the TM patients. The SCI-related complications seen in the TM group were as common as those in the T-SCI group.
    UNASSIGNED: The TM and T-SCI groups differed in terms of the demographic and clinical characteristics recorded. Additionally, when matched for these differences, functional status was slightly better in the TM group. However, like T-SCI, TM was a significant cause of disability and SCI-related complications were common.
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  • 文章类型: Journal Article
    COVID-19可伴有中枢神经系统和周围神经系统(CNS和PNS)的急性神经系统并发症。在这项研究中,我们估计了在Assiut和Aswan大学医院的COVID-19住院患者中此类并发症的发生频率。
    我们筛查了2020年6月1日至8月10日在上埃及的Assiut和Aswan大学医院收治的所有疑似COVID-19患者。临床和实验室检查,胸部和脑部的CT/MRI,并对每位患者进行神经生理学研究(如果需要)。
    439例确诊/可能的COVID-19;222例出现神经系统表现。其中,117人患有急性神经系统疾病,其余人患有头痛等非特异性神经精神症状,眩晕,和抑郁症。75例患者的中枢神经系统受到影响:55例中风,其他患者惊厥(5),脑炎(6),缺氧性脑病(4),脊髓病(2),多发性硬化症复发(2),和脑膜脑炎(1)。PNS在42例患者中受到影响:大多数患者患有失语症和失语症(31),其他患者患有格林-巴利综合征(4),周围神经病变(3),重症肌无力(MG,2),或肌炎(2)。发烧,呼吸道症状,头痛是最常见的一般症状。高血压,糖尿病,缺血性心脏病是中枢神经系统病变患者中最常见的合并症。
    在COVID-19中,CNS和PNS都受到影响。卒中是中枢神经系统最常见的并发症,和失语症和/或失语症是常见的PNS疾病。然而,有6例脑炎,脊髓脊髓病2例,2例MG,肌炎2例。
    COVID-19 can be accompanied by acute neurological complications of both central and peripheral nervous systems (CNS and PNS). In this study, we estimate the frequency of such complications among hospital inpatients with COVID-19 in Assiut and Aswan university hospitals.
    We screened all patients with suspected COVID-19 admitted from 1 June to 10 August 2020 to the university hospitals of Assiut and Aswan in Upper Egypt. Clinical and laboratory tests, CT/MRI of the chest and brain, and neurophysiology study were performed for each patient if indicated.
    439 patients had confirmed/probable COVID-19; neurological manifestations occurred in 222. Of these, 117 had acute neurological disease and the remainder had nonspecific neuropsychiatric symptoms such as headache, vertigo, and depression. The CNS was affected in 75 patients: 55 had stroke and the others had convulsions (5), encephalitis (6), hypoxic encephalopathy (4), cord myelopathy (2), relapse of multiple sclerosis (2), and meningoencephalitis (1). The PNS was affected in 42 patients: the majority had anosmia and ageusia (31) and the others had Guillain-Barré syndrome (4), peripheral neuropathy (3), myasthenia gravis (MG, 2), or myositis (2). Fever, respiratory symptoms, and headache were the most common general symptoms. Hypertension, diabetes mellitus, and ischemic heart disease were the most common comorbidities in patients with CNS affection.
    In COVID-19, both the CNS and PNS are affected. Stroke was the most common complication for CNS, and anosmia and/or ageusia were common for PNS diseases. However, there were 6 cases of encephalitis, 2 cases of spinal cord myelopathy, 2 cases of MG, and 2 cases of myositis.
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  • 文章类型: Journal Article
    BACKGROUND: Growing evidence shows links between septicaemia and non-multiple sclerosis demyelinating syndromes (NMSDS); nevertheless, epidemiological data are still very limited. This study aimed to explore the relationship between septicaemia and NMSDS in a general population.
    METHODS: The study included 482 781 individuals diagnosed with septicaemia and 1 892 825 age/sex-matched non-septicaemia patients for the comparison. Data were drawn from a population-based nationwide National Health Insurance Research Database Taiwan, from 1 January 2002 to 31 December 2011. The two cohorts of patients with and without septicaemia were followed up for the occurrence of NMSDS. The Cox-proportional hazard regression model was performed to estimate adjusted HR after multivariate adjustment.
    RESULTS: Individuals with septicaemia had a 4.17-fold (95% CI 3.21 to 5.4, p < 0.001) higher risk to develop NMSDS compared with those without septicaemia. Patients aged <65 years had a greater NMSDS risk (<45 years: HR = 6.41, 95% CI 3.65 to 11.3, p < 0.001; 45-64 years: HR = 6.66, 95% CI 3.98 to 11.2, p < 0.001). Furthermore, females with septicaemia and individuals with higher severity of septicaemia were associated with increased risks of developing NMSDS.
    CONCLUSIONS: Our results indicated that patients with septicaemia were likely to develop NMSDS. A possible contributing role of septicaemia in increasing the hazard of NMSDS is proposed, based on the outcome that individuals with higher severity of septicaemia carried elevated threat of encountering NMSDS.
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