视神经脊髓炎脊髓磁共振成像特征

目的 比较视神经脊髓炎(NMO)与多发性硬化(MS)的脊髓MRI特点,从MBI的角度重新认识NMO.方法 对20例MS患者和23例NMO患者的脊髓MRI进行同顾性分析.结果 NMO患者脊髓MRI多表现为线样延髓征、线样延髓脊髓征、线样脊髓征、脊髓横贯性或次横贯性损伤,且常超过3个节段(23例),而MS患者脊髓MRI病变节段短(≥3个节段者8例,χ2=19.142,P<0.01),常呈偏心性分布(17例,与NMO组比较,χ2=25.256,P<0.01).结论 NMO不同于MS,在MRI方面,病灶的分布有其自身特征,而MS的脊髓病灶与髓鞘走向一致.因此,我们从影像学角度进一步证实NMO是有异于MS的一种独立的疾病.     

以顽固性呃逆和恶心呕吐为首发症状的视神经脊髓炎临床分析

目的 研究以顽固性呃逆和恶心呕吐(IHN)为首发症状的视神经脊髓炎(NMO)的临床特点及MRI表现.方法 收集50例NMO患者的临床资料,对其中5例以IHN为首发症状的NMO患者临床表现及MRI特点进行分析.结果 以IHN为首发症状的NMO患者女性多见,发病较晚,头MRI显示病灶主要分布在第三、四脑室,中脑导水管周围及延髓中央管等室管膜周边,脊髓MRI显示线样延髓脊髓损害,病灶常常为大于3个椎体节段,主要累及灰质,以脊髓中央管为中心呈H型分布.结论 IHN是NMO的独特首发症状,并完全可逆,主要累及孤束核和最后区引起IHN.其MRI病灶具有特异性,多分布在室管膜和脊髓中央管周边(即水通道蛋白4的高表达区),呈线样损害.     

视神经脊髓炎脊髓损害的磁共振研究

目的分析视神经脊髓炎(NMO)和多发性硬化(MS)患者脊髓MRI特点,以及血清抗水通道蛋白4(AQP4)IgG抗体阳性与阴性NMO患者脊髓MRI特点。方法回顾分析贵州省中枢神经系统脱髓鞘疾病数据库中42例NMO和32例有脊髓损害的MS患者的脊髓MRI资料。结果与MS组比较,NMO患者脊髓病灶累及更长的椎体节段(P0.05),在脊髓MRI矢状位上表现为线样征和纵向延展的脊髓损害(LESCL)(P0.05)。轴位T2WI上亮斑状损害(BSLs)以及中心性、横贯性脊髓损害更常见(P0.05);在病灶部位及强化病灶上,NMO和MS组间比较差异无统计学意义。与血清抗AQP4-IgG抗体阴性NMO患者比较,阳性患者线样征、BSLs、中心性损害更常见(P0.05),在脊髓病灶部位、受累椎体节段数、LESCL、横贯性损害及强化病灶方面,抗AQP4-IgG抗体阳性组和阴性组间比较差异无统计学意义。结论除LESCL、线样征、横贯性损害和中心性损害特点外,BSLs可能是另一个有助于鉴别NMO与MS的脊髓病灶MRI特征。BSLs、线样征、中心性损害特点可能与NMO患者抗AQP4-IgG抗体的血清学状态有关。     

早期多发性硬化和视神经脊髓炎高危综合征的临床特点

目的 探讨早期多发性硬化和视神经脊髓炎高危综合征的临床特点.方法 回顾性分析我院2012-04-2014-01收治的45例早期MS患者和33例NMO高危综合征患者的临床资料,对比分析2组一般资料、机体功能评价、影像学检查和生化检查结果,总结两种疾病的临床特点.结果 2组在括约肌症状、认知障碍、小脑症状、视力受损及运动症状方面比较差异无统计学意义(P＞0.05),在病程、脑干症状、EDSS评分及感觉症状等方面比较差异有统计学意义(P＜0.05).早期MS组患者病灶数量＞9个、Barkhof标准符合率及脑MRI多发性硬化样病灶发生率均高于NMO高位综合征组,差异有统计学意义(P＜0.05).早期MS组患者脊髓病灶发生率、脑脊液白细胞计数异常率、脑脊液蛋白异常率、脑脊液蛋白水平及脑脊液白细胞计数均低于NMO高位综合征组患者,差异有统计学意义(P＜0.05).2组在钆增强扫描病灶发生率方面比较差异无统计学意义(P＞0.05).NMO高位综合征组抗体阳性率显著高于早期MS组,差异有统计学意义(P=0.018).结论 视神经脊髓炎高危综合征和早期多发性硬化具有不同的临床特点,临床诊断中应加以利用进行甄别,避免误诊的发生.     

早期多发性硬化和视神经脊髓炎高危综合征临床特点的比较

目的 比较早期多发性硬化(MS)和视神经脊髓炎(NMO)高危综合征患者的临床特点差异. 方法 回顾性收集广州医科大学附属第二医院神经内科自2004年1月至2013年8月收治的早期MS患者49例和NMO高危综合征患者30例(包括长节段的横贯性脊髓炎22例、复发性视神经炎8例)的临床资料、影像学检查结果、血清中NMO-IgG抗体情况等进行分析和比较. 结果 早期MS患者和NMO高危综合征患者的EDSS评分、病程以及感觉症状、脑干症状比例比较差异均有统计学意义(P＜0.05).早期MS组患者中病灶数＞9个的比例(77.6％)高于NMO高危综合征组患者,差异有统计学意义(P＜0.05).NMO高危综合征组患者在脑脊液蛋白异常率(19例,63.3％)、蛋白水平[(0.57±0.45) g/L]以及脑脊液白细胞计数异常率(19例,63.3％)、白细胞计数[中位数为24.317个/mm3 (0～274个)]方面与早期MS患者组比较差异有统计学意义(P＜0.05).10例NMO高危综合征患者中6例长节段横贯性脊髓炎患者NMO-IgG阳性,13例早期MS患者中2例出现阳性反应,阳性率比较差异有统计学意义(P＜0.05). 结论 早期MS患者和NMO高危综合症的临床特点明显不同,这些差异对早期鉴别MS与NMO具有一定意义.     

视神经脊髓炎患者的甲状腺功能、抗体改变及并发自身免疫性甲状腺病的分析

目的 探讨37例视神经脊髓炎(NMO)患者甲状腺功能及自身抗体的改变,分析并发自身免疫性甲状腺病(AITD)的NMO患者的临床特点.方法 回顾性分析2006～2011年首都医科大学宣武医院收治的37例视神经脊髓炎患者的甲状腺功能( TSH、T3、T4、FT3、FT4)和自身甲状腺抗体(TPOAb、TGAb),38名健康体检者作为对照组,比较2组研究对象的甲状腺功能和自身甲状腺抗体有无差异.同时比较并发AITD的NMO患者(5例)与无AIDT的NMO患者(32例)的临床特征、脊髓磁共振检查结果.结果 (1)NMO组病人TSH、T3、FT3、T4和FT4与健康对照组之间差异无统计学意义;NMO组病人甲状腺自身抗体TPOAb、TGAb均高于健康对照组,两组之间差异有统计学意义(P值均＜0.05).(2)5例(13.5％)并发AITD的NMO患者发病与第一次复发间隔的时间和第一次发作的EDSS评分,与无AITD的NMO患者之间的差异有统计学意义(P值均＜0.05),而两组病人病程持续时间、发作次数、第一年复发阳性率、脊髓MRI病变节段数之间的差异均无统计学意义.结论 NMO患者的甲状腺功能正常,自身甲状腺抗体升高.并发AITD的NMO患者首次发作较轻,发病与首次复发间隔长,但不影响患者临床的总体病程.     

引起顽固性呃逆、呕吐的视神经脊髓炎的临床表现和影像学研究

目的 探讨引起顽固性呃逆、呕吐(IHN)的视神经脊髓炎(NMO)患者的临床表现和脑干、脊髓MRI特点.方法 收集中山大学附属第三医院神经科17例NMO患者的临床资料,对其中8例合并IHN的NMO患者临床表现及MRI特点进行分析.结果 IHN在NMO患者中常见,本组17例NMO中有8例合并IHN,临床上表现为IHN、复视、眼球震颤,其中6例表现有线样延髓征(LML)或线样延髓脊髓征(LMSL).脊髓纵向MRI显示病灶常常大于3个椎体节段,且以脊髓中央管为中心;轴位脊髓MRI表现为部分性或横贯性,以脊髓的后角或侧角为主,前角受累较少. 结论 引起IHN的NMO临床上多伴有复视和眼球震颤,延髓脊髓MRI常常可见LML或LMSL征,而且病灶以脊髓中央管为中心,后角或侧角受累为主,这些可与多发性硬化相鉴别.     

特发性脱髓鞘性视神经炎的临床转归

目的 了解特发性脱髓鞘性视神经炎(IDON)临床转归、转化为多发性硬化(MS)或视神经脊髓炎(NMO)的比例以及相关影响因素.方法 对确诊且临床资料完整的IDON患者进行病例回顾及随访,记录视功能和其他神经功能变化以及MS或NMO转化率,应用卡方检验分析不同临床特征对转化率的影响.结果 共入组资料完整且完成随访的IDON患者107例.多数患者视力恢复较好,12例(11.2%)在随访期间转化为MS或NMO.全部12例患者均符合2005年修订的McDonald诊断标准,其中4例符合1999年NMO诊断标准,其余8例中部分表现为"视神经脊髓型MS".复发性IDON较首次发病患者、伴头颅MRI异常较MRI正常者转化为MS或NMO的比例高,分别为23.1%和4.4%(χ2=6.899,P<0.01)以及18.2%和8.1%.是否伴有视乳头水肿以及不同视力损害程度组之间转化为MS或NMO的比例没有差异.结论 该组IDON患者转化为MS或NMO的比例为11.2%.复发性IDON和伴有头颅MRI异常的患者更易转化为MS或NMO.     

脊髓型多发性硬化临床诊断分析

目的 总结分析脊髓型多发性硬化(MS)的临床特点和MRI表现.方法 回顾性分析21例脊髓型MS的临床特点和MRI表现,所有患者行脊髓和颅脑MRI检查.结果 脊髓型MS除有脊髓病变的临床表现外,临床症状和体征的多样性是其特点,如感觉障碍、肢体无力、视力障碍等;脊髓内病灶的MRI特点是不规则斑片状和条带状异常信号,位于脊髓两侧和后部,在T:WI像上为高或稍高信号,在T1WI上为等信号或稍低信号;80.9%(17/21)脊髓型MS合并脑内病灶.结论 脊髓型MS临床表现呈多样性,MRI可以准确显示脊髓内病灶,颅脑MRI检查有助于脊髓型MS的诊断.     

脊髓型多发性硬化—附11例临床分析

报告 11例脊髓型多发性硬化 (MS) ,占同期多发性硬化住院病人 18.6 %。其临床表现为脊髓损害的症状和体征 ,病程中有缓解与复发 ,或呈慢性进展。颈段脊髓 2例 ,胸段脊髓 9例 ,均作了磁共振扫描 (MRI) ,其特点是 :脊髓内病灶的间断分布与其长轴走行一致的椭圆形或条索状病灶 ,病灶呈偏心分布。本资料及文献报道有一些 MS患者以脊髓受损为首发部位 ,并在较长时间内表现为脊髓受损的临床病征 ,MRI的使用对早期脊髓型 MS诊断有很大价值 ,但脊髓型 MS是独立疾病 ,还是 MS病程中脊髓受损的表现之一 ,在研究中应引起重视     

Brain abnormalities in neuromyelitis optica

BACKGROUND: Neuromyelitis optica (NMO) is a severe demyelinating disease defined principally by its tendency to selectively affect optic nerves and the spinal cord causing recurrent attacks of blindness and paralysis. Contemporary diagnostic criteria require absence of clinical disease outside the optic nerve or spinal cord. We have, however, frequently encountered patients with a well-established diagnosis of NMO in whom either asymptomatic or symptomatic brain lesions develop suggesting that the diagnostic criteria for NMO should be revised. OBJECTIVE: To describe the magnetic resonance image (MRI) brain findings in NMO. DESIGN: Observational, retrospective case series.Patients We ascertained patients through a clinical biospecimens database of individuals with definite or suspected NMO. We included patients who (1) satisfied the 1999 criteria of Wingerchuk et al for NMO except for the absolute criterion of lacking symptoms beyond the optic nerve and spinal cord and the supportive criterion of having a normal brain MRI at onset; (2) had MRI evidence of a spinal cord lesion extending 3 vertebral segments or more (the most specific nonserological feature to differentiate NMO from MS); and (3) were evaluated neurologically and by brain MRI at the Mayo Clinic. MAIN OUTCOME MEASURES: Magnetic resonance images were classified as normal or as abnormal with either nonspecific, multiple sclerosis-like or atypical abnormalities. We evaluated whether brain lesions were symptomatic and analyzed the neuropathologic features of a single brain biopsy specimen. RESULTS: Sixty patients (53 women [88%]) fulfilled these inclusion criteria. The mean +/- SD age at onset was 37.2 +/- 18.4 years and the mean +/- SD duration of follow-up was 6.0 +/- 5.6 years. Neuromyelitis optica-IgG was detected in 41 patients (68%). Brain MRI lesions were detected in 36 patients (60%). Most were nonspecific, but 6 patients (10%) had multiple sclerosis-like lesions, usually asymptomatic. Another 5 patients (8%), mostly children, had diencephalic, brainstem or cerebral lesions, atypical for multiple sclerosis. When present, symptoms of brain involvement were subtle, except in 1 patient who was comatose and had large cerebral lesions. CONCLUSIONS: Asymptomatic brain lesions are common in NMO, and symptomatic brain lesions do not exclude the diagnosis of NMO. These observations justify revision of diagnostic criteria for NMO to allow for brain involvement.     

视神经脊髓炎与多发性硬化的临床表现和视神经脊髓炎IgG抗体阳性率的对比

目的 比较视神经脊髓炎(NMO)和多发性硬化(MS)在临床表现、辅助检查等方面的不同;比较NMO和MS等脱髓鞘疾病患者血清NMO-IgG抗体的阳性率,判断该抗体能否作为鉴别诊断的一项实验室依据.方法 对34例NMO、22例MS、24例高危综合征、5例临床孤立综合征以及35例其他神经科疾病患者进行NMO-IgG检测,并对其中NMO、MS患者的人口学、临床表现、免疫学指标、脑脊液、头颅MRI等资料进行对比.结果 NMO的起病年龄较MS大且年龄跨度更广;从年复发率和进展指数来看,NMO更为严重,预后更差;NMO长节段脊髓损害者比MS多.NMO-IgG在NMO组和高危综合征组的阳性率分别为58.8%(20/34)和45.8%(11/24),高于MS组(1/22)、临床孤立综合征组(1/5)和其他疾病组(1/35;X2=37.2,P<0.01).NMO-IgG阳性率与脊髓病变长度相关.结论 NMO和MS在临床表现、辅助检查等方面都有所不同,提示NMO与MS可能是2种不同的疾病.NMO-IgG在NMO患者中的阳性率高于MS患者,可以作为鉴别诊断的一项实验室依据.     

视神经脊髓炎与多发性硬化的临床表现和视神经脊髓炎IgG抗体阳性率的对比

目的 比较视神经脊髓炎(NMO)和多发性硬化(MS)在临床表现、辅助检查等方面的不同;比较NMO和MS等脱髓鞘疾病患者血清NMO-IgG抗体的阳性率,判断该抗体能否作为鉴别诊断的一项实验室依据.方法 对34例NMO、22例MS、24例高危综合征、5例临床孤立综合征以及35例其他神经科疾病患者进行NMO-IgG检测,并对其中NMO、MS患者的人口学、临床表现、免疫学指标、脑脊液、头颅MRI等资料进行对比.结果 NMO的起病年龄较MS大且年龄跨度更广;从年复发率和进展指数来看,NMO更为严重,预后更差;NMO长节段脊髓损害者比MS多.NMO-IgG在NMO组和高危综合征组的阳性率分别为58.8%(20/34)和45.8%(11/24),高于MS组(1/22)、临床孤立综合征组(1/5)和其他疾病组(1/35;X2=37.2,P<0.01).NMO-IgG阳性率与脊髓病变长度相关.结论 NMO和MS在临床表现、辅助检查等方面都有所不同,提示NMO与MS可能是2种不同的疾病.NMO-IgG在NMO患者中的阳性率高于MS患者,可以作为鉴别诊断的一项实验室依据.     

视神经脊髓炎与多发性硬化的临床表现和视神经脊髓炎IgG抗体阳性率的对比

目的 比较视神经脊髓炎(NMO)和多发性硬化(MS)在临床表现、辅助检查等方面的不同;比较NMO和MS等脱髓鞘疾病患者血清NMO-IgG抗体的阳性率,判断该抗体能否作为鉴别诊断的一项实验室依据.方法 对34例NMO、22例MS、24例高危综合征、5例临床孤立综合征以及35例其他神经科疾病患者进行NMO-IgG检测,并对其中NMO、MS患者的人口学、临床表现、免疫学指标、脑脊液、头颅MRI等资料进行对比.结果 NMO的起病年龄较MS大且年龄跨度更广;从年复发率和进展指数来看,NMO更为严重,预后更差;NMO长节段脊髓损害者比MS多.NMO-IgG在NMO组和高危综合征组的阳性率分别为58.8%(20/34)和45.8%(11/24),高于MS组(1/22)、临床孤立综合征组(1/5)和其他疾病组(1/35;X2=37.2,P<0.01).NMO-IgG阳性率与脊髓病变长度相关.结论 NMO和MS在临床表现、辅助检查等方面都有所不同,提示NMO与MS可能是2种不同的疾病.NMO-IgG在NMO患者中的阳性率高于MS患者,可以作为鉴别诊断的一项实验室依据.     

Preferential spinal central gray matter involvement in neuromyelitis optica

Objective To delineate the MRI features that distinguish neuromyelitis optica (NMO) from multiple sclerosis (MS). Methods We compared the distribution of the spinal cord lesions by analyzing 1) lesion area, 2) lesion density (by superimposing the lesions onto the standard sections of the cervical and thoracic cord with appropriate transparencies using computer software), and 3) T1-hypointensity in axial sections of MRI in NMO and MS. Results In NMO, 60–70% of the cervical and thoracic cord MRI lesions occupied more than half of the cord area and mainly involved the central gray matter in the acute stage. In the chronic stage, half or more of the lesions were localized at the central gray matter region. The lesion superimposition analysis also revealed much higher densities in the central gray matter region than in the peripheral white matter regions. Two patients with NMO had T1-hypointense lesions in the central region. In contrast, over 80% of the lesions in MS were localized in the lateral and posterior white matter regions of the cord in the chronic as well as acute stage. Lesion densities were much higher in the lateral and posterior white matter regions than in the central gray matter region. None of the lesions in MS were T1-hypointense. Conclusions These MRI findings strongly suggest a preferential involvement in the spinal central gray matter in NMO which is distinct from MS.     

Prominent brainstem symptoms/signs in patients with neuromyelitis optica in a Taiwanese population

Neuromyelitis optica (NMO) is a severe demyelinating disease defined principally by its selective effect on the optic nerves and spinal cord. Contemporary diagnostic criteria require an absence of any clinical disease outside the optic nerve or spinal cord. However, we frequently encounter patients with NMO who have previously undetected symptomatic brainstem lesions. We investigated the brainstem symptoms/signs in patients with NMO and their corresponding MRI findings in a Taiwanese population. We evaluated the clinical symptoms/signs, anti-aquaporin-4 antibody titer and corresponding brain MRI of 49 patients with NMO; results were obtained from chart reviews and during clinical visits. A total of 18 (37%) patients with NMO had brainstem symptoms/signs, including diplopia (n = 9, 50%), prolonged hiccup and poor appetite (n = 9, 50%). For these patients, most of their brainstem events occurred during the first demyelinating attack in their NMO course. A higher percentage (77.8%) of patients with brainstem NMO had brain lesions with specific NMO patterns, including lesions involving the hypothalamus (n = 6, 33.3%), midbrain or pons (n = 8, 44.4%), periaqueductal regions (n = 5, 27.7%), and medulla (n = 10, 55.6%). Brainstem symptoms/signs and characteristic NMO imaging findings are common in Taiwanese patients with NMO, and should be considered a part of the illness in addition to optic neuritis and myelitis.     

Interferon beta-1b exacerbates multiple sclerosis with severe optic nerve and spinal cord demyelination

To evaluate the effect of interferon beta-1b (IFNB-1b) on multiple sclerosis (MS) with severe optic nerve and spinal cord demyelination, we examined the relationship between IFNB-1b treatment outcome and the clinical and genetic characteristics of three types of demyelinating diseases of the central nervous system, i.e., neuromyelitis optica (NMO), MS and MS with severe optic-spinal demyelination. Japanese MS frequently carried HLA DPB1*0501, which is associated with NMO. MS with DPB1*0501 showed severe optic-spinal demyelination represented by longitudinally extensive spinal cord lesion, blindness and CSF pleocytosis. IFNB-1b treatment did not succeed in these patients because of the increase of optic nerve and spinal cord relapse and other severe side effects. IFNB-1b should not be administered to demyelinating patients with genetic and clinical characteristics mimicking NMO such as HLA DPB1*0501 allele, longitudinally extensive spinal cord lesion, blindness and CSF pleocytosis even if they have symptomatic cerebral lesions as typically seen in MS. The present study strongly suggests that these patients should be diagnosed as having NMO.     

Significance of gray matter brain lesions in multiple sclerosis and neuromyelitis optica

Multiple sclerosis (MS) and neuromyelitis optica (NMO) are the two main autoimmune diseases of the CNS. In patients with NMO, the target antigen is aquaporin‐4 (AQP4), the most abundant water channel protein in the CNS. AQP4 is mainly expressed on astrocytic endfoot processes at the blood‐brain barrier and in subpial and subendymal regions. MS and NMO are distinct diseases, but they have some common clinical features: both have long been considered autoimmune diseases that primarily affect the white matter (WM). However, because WM demyelination by itself cannot explain the full extent of the clinical disabilities, including cognitive decline in patients with MS and NMO, renewed interest in gray matter (GM) pathology in MS and NMO is emerging. Important hallmarks of WM and GM lesions in MS and NMO may differentially influence neuronal degeneration and demyelination in the brain and spinal cord, given different detrimental effects, including cytokine diffusion, disruption of water homeostasis associated with or without AQP4 (the target antigen in NMO) dynamics, or other unidentified mechanisms. An increase in knowledge of the structure of GM and WM lesions in MS and NMO will result in more targeted therapeutic approaches to these two diseases.