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.     

视神经脊髓炎的头部MRI异常表现及对应临床症状

目的研究视神经脊髓炎头部MRI的特异表现及对应临床症状。方法回顾性分析中国医科大学附属第一医院神经内科自2008年1月²012年7月收治的22例NMO脑部MRI检查结果及相关临床表现。结果本组22例患者中12例头部MRI正常,10例发现脑部病灶,病变累及侧脑室旁白质3例,丘脑1例,第三脑室和中脑导水管周边5例,桥脑5例,延髓5例,皮质0例;头部MRI病灶为点状或线样损害,左右对称。临床表现为顽固性呃逆、恶心呕吐、复视、眼震、过度睡眠、血糖增高、中枢性高热等。结论 NMO患者脑室管膜周边和下丘脑处病灶具有特异性,所有病例并未见皮质改变;并能引起顽固性呃逆、恶心呕吐(IHN)、过度睡眠、内分泌改变、复视、眼震等特征性临床表现。     

经MRI确诊的延髓背外侧综合征

应用MRI研究延髓背外侧综合征。具有本病临床表现的11例患者均行头颅CT及MRI检查。眩晕、呕吐、眼球震颤、声音嘶哑、同侧面部及对侧肢体痛温觉障碍、同侧软腭麻痹、同侧肢体共济失调、同侧Horner征是最常见的临床特征。MRI显示一侧延髓背外侧梗塞10例,延髓背外及腹外侧梗塞1例,合并同侧小脑梗塞2例。CT均未发现延髓及小脑病灶。MRI为目前研究延髓背外侧综合征最佳手段,小脑区梗塞不容忽视。     

视神经脊髓炎脑部病灶的影像学特征及临床表现

目的 探讨视神经脊髓炎(NMO)脑部病灶的影像学特征及主要临床表现.方法 回顾性分析南京医科大学附属脑科医院神经内科自2002年1月至2008年5月收治的19例NMO患者脑部MRI检查结果及相关病史资料.结果 本组19例患者中15例行头颅MRI检查,7例发现脑部病灶;病变累及大脑半球3例,丘脑2例,下丘脑1例,第三脑室和中脑导水管周边1例,中脑1例,桥脑1例,延髓2例;MRI上病灶为点状、斑片状或线样,呈T1低信号、T2和FLAIR高信号改变.临床表现恶心、呕吐、呃逆、复视、眼震、面部麻木、认知功能障碍及嗜睡等.结论 NMO可出现不同部位脑部病灶,以脑室管膜周边和下丘脑处病灶具有疾病特征性.部分NMO患者出现脑部受累表现,少数患者以脑部症状为首发临床事件.     

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。     

视神经脊髓炎患者33例脑部磁共振分析

目的 探讨视神经脊髓炎(neuromyelitis optica,NMO)患者脑部MRI影像学表现.方法 收集满足最新NMO诊断标准且脑部MRI表现不符合多发性硬化诊断标准的患者33例,均行脑部和脊髓MRI检查,分析其MRI影像学特点.结果 33例NMO中,脑部正常表现者5例(15.2%),异常表现28例(84.8%),其中脑内实质有明确病灶22例(66.7%),另6例(18.2%)脑内虽未见明确病灶,但深部脑白质显示了肉眼可视的对称性弥漫性脱髓鞘高信号影.22例明确病灶中,15例病灶数≥2个,7例为单个病灶.幕上近皮质、皮质下和深部脑白质区的点状非特异性病灶最多,少数为非典型的斑片状融合病灶.幕下脑干是易受累的部位(14/33,42.4%),特别是延髓(7/33,21.2%).结论 NMO患者出现脑内异常较为常见,有脑部的异常不能排除NMO的诊断.认识NMO脑内病灶对完善NMO诊断标准有帮助.     

视神经脊髓炎的临床特征

目的探讨视神经脊髓炎(NMO)的临床特征。方法回顾分析18例NMO患者的临床资料。结果本组复发型NMO15例(83.3%),女性16例(88.9%),平均发病年龄36岁。所有患者双眼同时或先后出现视力减退,均有横贯性脊髓损害表现。MRI检查显示病灶位于颈髓4例、胸髓6例、颈、胸髓均受累5例,脊髓病灶长度≥3个椎体13例,出现脑部病灶6例。本组每例发病次数平均5次,遗留轻度功能残疾7例、中度功能残疾7例、重度功能残疾2例,死亡2例。结论NMO以复发型多见,女性多见,以双侧视神经受累及长节段脊髓炎为主要临床表现,部分患者出现脑部病灶。NMO复发率高,预后较差。     

亚急性联合变性的脊髓MRI特征

目的分析脊髓亚急性联合变性(SCD)的脊髓MRI影像特征,探讨MRI对SCD的诊断价值。方法回顾性分析临床诊断为SCD患者的临床和脊髓MRI,并复习相关文献进行分析。结果 10例SCD患者MRI均显示颈髓和(或)胸髓后索和侧索T2WI高信号,横轴位呈"八字"征、"圆点"征、"三角"征、"双目望远镜"征以及"小字"征。结论MRI提示脊髓后索和侧索选择性受累是SCD的特征性表现,具有重要诊断价值。     

延髓背外侧梗死的感觉障碍特点及解剖学机制研究(附2例报告)

目的探讨延髓背外侧梗死(LMI)的感觉障碍特点及内在解剖学机制。方法总结2例伴不典型偏身感觉异常的LMI患者的临床及影像学资料,并复习相关文献。结果 2例LMI患者的临床表现均不典型,主要表现为头晕、偏身感觉异常。例1表现为病灶同侧面部及同侧肢体感觉异常(ISSL),主观描述为麻木、发紧,例2表现为病灶对侧肢体痛温觉减退、同侧肢体无力,还合并对侧肢体迟发性感觉异常(先表现为发凉,后出现上肢近端烧灼感)。2例患者DWI上梗死灶分别位于延髓背外侧、外侧。病因考虑为动脉粥样硬化,且合并椎动脉或其分支动脉发育异常。结论 LMI的感觉障碍复杂多样,不典型的有面部感觉异常、ISSL、对侧肢体迟发性感觉异常等,考虑与三叉丘系、内侧丘系、脊髓丘系受累有关。     

Intractable hiccup and nausea with periaqueductal lesions in neuromyelitis optica

Intractable hiccup and nausea (IHN) was found in eight of 47 cases of relapsing neuromyelitis optica (NMO) (17%) but in none of 130 cases of multiple sclerosis (MS). IHN resolved with methylprednisolone. In six cases, MRI detected linear medullary lesions involving the pericanal region, the area postrema, and the nucleus tractus solitarius. Like long and centrally located myelitis, a linear medullary lesion causing IHN may distinguish NMO from MS.     

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.     

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.     

Acute posterior cord lesions in multiple sclerosis. An MRI study of the clinical course in 20 cases

Twenty patients with multiple sclerosis (MS), 19 women and 1 man, with acute proprioceptive sensory disturbances related to the presence of plaques on the posterior columns (posterior column syndrome) at the cervical or thoracic levels of the spinal cord, were selected among 138 new patients with MS assisted in our neurological unit over the past five years. In 17 of these patients, the acute posterior cordonal syndrome was responsible for the first clinical manifestations of the disease. The other 3 patients had a history suggestive of MS. These 20 patients were followed with a minute analysis of neurological function with repeated clinical evaluation combined with repeated MRI study of the spinal cord. Brain MRI (strongly suggestive of MS in 15 patients), evoked potentials (EP) and cerebrospinal fluid electrophoresis analysis (with oligoclonal bands present in all patients were it was performed) were also obtained at least once in each patient. Spinal cord MRI demonstrated more lesions in the cervical region (90 p.100) than in the thoracic regions (10 p.100). Eighty percent of the cervical lesions were located high, between C1 and C4. The most characteristic clinical expression was the deafferentation of one upper limb, preferentially the "useless hand" (Oppenheim) or even a pseudoathetosic or dystonic limb. Propioceptive ataxia or spontaneous cervical or brachial pain were other forms of clinical expression. No major motor deficit or sphincter disorders were noted at any time in the clinical course in any of the patients. There was a good correlation between localization and morphology of the plaques detected by spinal cord MRI and clinical signs. Intrinsic medullary lesions were seen as high intensity signals on T2-weighted images which were enlarged more than the same lesion visualized on T1-weighted images after injection of paramagnetic contrast agents. This reflected the presence of edema extending beyond the main inflammatory lesion. There was also a good correlation between improvement of clinical symptoms and total or, mor frequently, partial reduction of the plaques, analyzed morphologically by successive spinal cord MRI series. The diagnosis of MS was clinically definitive in 60 p.100 of cases and laboratory-supported definitive in 40 p.100. During the follow-up period (average 36 months), 15 patients (75 p.100) presented one or more exacerbations, all of them presenting a favorable course: at last follow-up, 9 patients were asymptomatic, EDSS was 1 in 6 patients, 1.5 in 4 patients and 2 in 1 patient. This study confirms the contribution of serial spinal cord MR studies to understanding the natural history and pathophysiology of medullary forms of MS presenting as a cordonal posterior syndrome. It also shows a good relationship between the clinical manifestations and course of this form of MS and the localization and variable morphology of plaques. Finally, our results suggest the predictive benign course for this medullary form of MS that seems to be almost exclusively restricted to the female gender.     

视神经脊髓炎与多发性硬化的临床症状、脊髓MRI表现的对比分析

目的 结合视神经脊髓炎(NMO)与多发性硬化(MS)患者的临床症状和脊髓MRI特点探讨两者之间差异发生的机制.方法 回顾性分析中山大学附属第三医院自2004年1月至2007年1月收治的23例NMO患者及21例MS患者的临床资料,比较其临床症状及脊髓MRI上受损部位MRI上的差异.结果 NMO患者多为女性,且首次发病年龄、扩展病残状况评分(EDSS)评分均高于MS患者;双侧深感觉障碍、束带感、直肠或膀胱括约肌功能障碍3种临床症状在NMO、MS患者中的发生率不同,差异均有统计学意义(P<0.05);上述各临床症状基本能在脊髓MRI找到相应受损病灶.结论 NMO是不同于MS的脱髓鞘疾病,其特殊的发病机制导致其临床症状与脊髓MRI均有自己的特点.     

What are the unique characteristics of optico-spinal MS in Japanese and neuromyelitis optica in western populations]

Although the diagnosis of optico-spinal MS (OSMS) is solely based on the unique lesion distribution, the OSMS is clinically characterized by distinctive features which are mostly shared by the clinical characteristics of relapsing neuromyelitis optica (NMO). Conversely, Western investigators appear to consider relapsing NMO as a distinct entity from MS, and distinct characteristics and independent diagnostic criteria for NMO were proposed. However, the key characteristics of OSMS and NMO seem to be quite similar, and therefore the prototype of these disorders are identical which we would like to call OSMS/NMO. Most of the described characteristics of the OSMS/NMO appear to reflect the fulminant nature of each attack and the expansion of each lesion, which we called "attack-related severity". Recently, we found that clinical characteristics seem to be distinctively different between OSMS patients with and without longitudinally extending spinal cord lesions while clinical features of OSMS patients without extending spinal cord lesions are similar to those of CMS patients. To understand the pathomechanisms of OSMS and NMO, the "attack-related severity" must be an important key factor as well as the unique lesion distribution.     

Neuroradiological aspects of Devic's neuromyelitis optica

INTRODUCTION: Neuromyelitis optica (NMO) is a rare inflammatory and demyelinating disorder of the central nervous system, restricted to optical nerves and spinal cord. The main neuroradiological aspects, now summarized into a complete set of diagnosis criteria, are a normal cerebral MRI at onset and longitudinal involvement of the spinal cord concerning more than 3 vertebral segments. The clinical course and frequency of typical lesions remain unknown. OBJECTIVE: We here report neuroradiological data from patients suffering from NMO. METHODS: Brain and spinal cord MRI were systematically reviewed for 32 afro-Caribbean patients. RESULTS: A typical longitudinal spinal lesion was seen in 44.7 percent with or without edema; a lesion involving less than 3 vertebral segments in 26.3 percent and no lesion in 21.1 percent. Longitudinal study of a few bouts suggested a progressive normalisation of spinal cord appearance. Atrophy was negatively correlated with immunosuppressive treatment. Cerebral lesions usually absent at onset were correlated to the follow-up. In a non-recursive condition, patients completed diagnostic criteria for encephalic and spinal lesions in 82.8 percent and 48.1 percent. CONCLUSION: Radiology of spinal bouts showed multiple aspects besides the typical form. The notion of multiple bouts must be added to the spinal criteria to achieve good sensitivity. A typical extensive spinal lesion is usual in the follow-up, but seen after less then half of the bouts. Requiring such a lesion would delay the diagnosis.     

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.