酷似肿瘤的脊髓炎性脱髓鞘假瘤

目的总结脊髓脱髓鞘似瘤的临床、影像学和病理特点。方法回顾分析6例脊髓脱髓鞘假瘤的临床、影像学特征，对其中2例误诊为髓内肿瘤术后病理表现进行分析。结果脊髓脱髓鞘假瘤常以急性或亚急性起病，最常累及颈髓和胸髓，可伴有周围神经受损：脊髓脱髓鞘假瘤单时相起病，症状、体征提示为单病灶；MRI检金病灶多单发（偶可多发），占位水肿效应明显，增强后可呈非闭合性花环样或环肜强化；病理为炎性脱髓鞘改变，白质内髓鞘破坏并水肿，病灶内以小血管周围大量淋巴细胞浸润、巨噬细胞聚集为主要表现；绐激素治疗后好转。结论脊髓炎性脱髓鞘假瘤MRI酷似髓内肿瘤，容易造成误诊。可试用激素治疗或组织活检，不要急于手术。     

脑内脱髓鞘假瘤

目的探讨脑内脱髓鞘假瘤的病理特点。方法回顾性分析11例经手术证实的脑内脱髓鞘假瘤的临床与影像学表现、病理特点、治疗效果及随访情况。结果脱髓鞘假瘤以急性起病为主，病程呈单时相；影像学多表现为皮质下单一病灶、其强化呈非闭合性或与脑室垂直：病理学发现病灶呈炎性脱髓鞘样改变。结论脱髓鞘假瘤临床表现各异，典型的临床及影像学表现有助于诊断；组织病理学检查是确诊的金标准。脱髓鞘假瘤不宜手术治疗，肾上腺皮质激素对此病治疗效果好。     

中枢神经系统炎性脱髓鞘性假瘤的诊断和治疗

目的:描述中枢神经系统炎性脱髓鞘性假瘤的临床表现,探讨该病的病理学特征以及MRI诊断价值,以期加强对本病的认识并提高诊断的正确性。方法:对2例经手术病理证实中枢神经系统炎性脱髓鞘性假瘤进行MRI检查,对其临床表现、影像学表现进行了回顾性分析。结果:MRI显示2例病变均表现为局限性肿块呈均匀长T1、长T2信号,临近未见伴有多发病灶。术前均被误诊为胶质瘤,经手术切除病变,病理证实为中枢神经系统炎性脱髓鞘性假瘤。结论:中枢神经系统炎性脱髓鞘性假瘤的临床和影像学诊断较为困难,常将本病误诊为肿瘤,此时可以先使用激素试验治疗,以避免手术或放射治疗造成的严重损伤。     

多发性硬化PET代谢显像与MRI和病理对照研究

目的 比较磁共振成像(MRI)检查和正电子发射断层显像(PET)及病理对判定脑内多发性硬化(MS)，尤其是脱髓鞘假瘤及疾病分期的价值。方法 经临床明确诊断的MS2例．均经过MRI扫描，T1、T2加权，行PET显像，将两者进行比较，其中1例病灶做病理检查。结果 2例MRI扫描T1WI表现为低信号．T2WI可见明确高信号，例2呈团块状。PET显像：例1右侧顶叶、左侧小脑半球等处可见形态欠规则的稍高代谢灶；例2左侧顶叶、颞叶及枕叶皮层及皮层下显示较大范围代谢降低区。病理学检查证实病灶处有大量泡沫样细胞(CD68^ )及增生的胶质细胞，病灶区血管周围T淋巴细胞浸润呈套袖状，髓鞘染色显示髓鞘脱失。结论 使用PET技术可辅助判定脑内白质区病灶是急性期还是非急性期，并可协助鉴别脱髓鞘假瘤与颅内肿瘤，同时能为MS患者脑功能异常与病蛮的范围的关系提供良好依据。     

脑内脱髓鞘性假瘤的诊断与治疗（附3例报告）

目的探讨脑内脱髓鞘性假瘤（DPT）的临床表现、影像学特点、病理特征及治疗效果。方法回顾性分析3例DPT的临床资料,并复习相关文献。结果脑内DPT以急性起病为主;影像学多表现为皮质下单一病灶,其强化呈非闭合性、斑片样或垂直于侧脑室分布的火焰样;病理检查发现病灶呈炎性脱髓鞘样改变;皮质类固醇激素治疗后症状均得到明显改善。结论脑内DPT的临床表现与大多颅内肿瘤类似,易误诊为脑胶质瘤;其影像学具有一定的特征性表现;类固醇激素治疗可获得满意疗效。     

脑内脱髓鞘假瘤的MRI表现

目的分析脑内脱髓鞘假瘤的MRI表现,加强对本病的认识,提高诊断准确率。方法回顾性分析10例经手术病理证实的脱髓鞘假瘤患者的MRI表现。结果病灶多位于大脑半球脑白质内,位于脑室周围7例,多垂直于侧脑室长轴,左额叶2例,右颞叶1例,呈圆形或不规则形,边界常不清楚。在T1WI多表现为稍低信号影,T2WI上多表现为稍高信号影。部分病灶伴有不同程度的周围水肿。增强扫描表现为不同程度均匀或不均匀明显强化,8例表现为典型的开环征,病灶边界明显环形强化,2例呈斑片状不均匀强化。结论脑内脱髓鞘假瘤易误诊为肿瘤性病变,临床及放射科医师需要结合临床表现,MRI表现能对本病做出准确的诊断,避免误判、误治给患者带来不必要的损失。     

脑脱髓鞘假瘤的临床和影像学特点(附1例报告)

目的探讨脑脱髓鞘假瘤的临床和影像学特点。方法回顾性分析1例脑脱髓鞘假瘤患者的临床资料。结果本例患者以左侧肢体活动不灵敏、感觉障碍为主,无明显头痛、头晕、恶心等高颅压症状。头颅MRI平扫:右侧额颞顶部颅内占位性病变。头颅MRI强化:右侧额叶可见团片状不均匀异常强化信号影,边界较清楚,周围见片状低信号水肿带。手术切除后病理检验提示脑脱髓鞘假瘤。结论脑脱髓鞘假瘤主要临床表现以神经系统功能障碍为主;影像学上多表现为脑内单发肿块,增强扫描病变多呈弥漫性强化或环形强化,酷似脑内原发肿瘤或转移瘤,常被误诊而行手术切除。     

脊髓脱髓鞘病15例报告

目的　探讨脊髓脱髓鞘病临床及影像学特点并观察其治疗后变化。方法　收集 1 5例 (男 4例、女 1 1例 )脊髓脱髓鞘病患者的临床资料并进行分析总结。结果　经甲基强的松龙冲击治疗后 ,1 2例临床症状改善且脊髓病灶缩小 ,3例临床症状改善不明显但病灶较前缩小。结论　脊髓脱髓鞘病有如下特征 :(1 )急性或亚急性起病 ;(2 )脊髓 MRI显示病灶呈多中心性 ,脊髓中央管扩张不明显 ,病灶呈斑片状强化或不强化 ;(3 )临床症状较重 ,但病灶相对较小 ;(4 )脑脊液寡克隆区带 ( ) ;(5 )激素治疗效果明显 ;(6)头颅 MRI检查发现脑室旁或脑干病灶 ;(7)既往曾有视神经炎或脊髓炎史。对可疑的脊髓炎性脱髓鞘病患者 ,应隔 3～ 6个月复查脊髓 MRI,注意病灶演变。     

炎性脱髓鞘性假瘤1例报告

炎性脱髓鞘性假瘤是中枢神经系统脱髓鞘病变的一种特殊类型.由于其在MRI上多是单一病灶,占位效应及灶周水肿明显,故难与脑脓肿、神经胶质瘤、脑转移瘤等鉴别.现将我院收治的1例术前误诊为星形细胞瘤的炎性脱髓鞘性假瘤的MRI及病理报道如下.     

MRI增强扫描对脑内脱髓鞘假瘤的诊断价值

目的探讨MRI增强扫描对大脑脱髓鞘假瘤(demyelination pseudotumor,DPT)的诊断价值。方法回顾性分析9例经病理或临床证实的大脑DPT患者MRI平扫及增强扫描的表现。结果病灶多发1例,单发8例。MRI平扫显示,病变在T1WI上呈低信号,T2WI上呈高信号,DWI上呈略低、等或略高信号;增强扫描2例病灶内可见斑片状强化,余7例均见垂直于侧脑室的线条状强化。结论 MRI增强扫描对于DPT的诊断具有重要价值。垂直于侧脑室的线条状强化是大脑DPT的典型MRI表现。     

Rare infections mimicking MS

The diagnosis of multiple sclerosis (MS), despite well defined clinical criteria is not always simple. On many occasions it is difficult to differentiate MS from various non-MS idiopathic demyelinating disorders, specific and infectious inflammatory diseases or non-inflammatory demyelinating diseases. Clinicians should be aware of various clinical and MRI “red flags” that may point to the other diagnosis and demand further diagnostic evaluation. It is generally accepted that atypical clinical symptoms or atypical neuroimaging signs determine necessity for broad differential diagnostic work up. Of the infectious diseases that are most commonly mistaken for MS the clinician should take into account Whipple's disease, Lyme disease, Syphilis, HIV/AIDS, Brucellosis, HHV-6 infection, Hepatitis C, Mycoplasma and Creutzfeld-Jacob disease, among others. Cat scratch disease caused by Bartonella hensellae, Mediterranean spotted fever caused by Riketssia connore and Leptospirosis caused by different Leptospira serovars rarely cause focal neurological deficit and demyelinating MRI changes similar to MS. When atypical clinical and neuroimaging presentations are present, serology on rare infectious diseases that may mimic MS may be warranted. This review will focus on the infectious diseases mimicking MS with presentation of rare illustrative cases.     

Monofocal large inflammatory demyelinating lesion,mimicking brain glioma

Here we report two cases of pathologically confirmed tumor-like demyelinating lesions. In comparison with common primary demyelinating diseases, our cases demonstrated atypical radiologic features, such as a large monofocal lesion with mild brain edema, and open ring-like or focal enhancement on magnetic resonance images, suggesting brain tumors. The clinical manifestations included focal neurologic signs due to the lesions, monophasic episodes without relapse over a long follow-up period, and efficacy of oral corticosteroid therapy. Histological analysis of brain biopsy specimens showed the inflammatory demyelination and preserved axons without tumor cells. The present cases suggest the importance of considering inflammatory demyelinating disease in the different diagnosis of monofocal tumor-like lesion.     

肿瘤样脱髓鞘病3例临床病理分析

目的探讨肿瘤样脱髓鞘病临床和病理学特点。方法对3例肿瘤样脱髓鞘病患者的临床特征进行了分析,并对活检脑组织进行了组织病理学和免疫组化研究。结果3例患者均呈亚急性起病,临床表现各不相同,有复发缓解过程。头部MRI显示3例患者分别在额、顶叶及小脑半球单发大面积病变。组织病理学观察均显示中心区组织高度水肿,呈空泡样变,有囊性坏死区。白质髓鞘高度崩解,轴索相对保留完好。免疫组化染色显示大量炎性细胞主要是淋巴细胞及少量单核吞噬细胞围绕血管呈袖套样浸润。结论肿瘤样脱髓鞘病临床表现各异,当影像学表现为孤立、单发、巨大病灶时,应想到脱髓鞘疾病的可能,病理学检查对确定诊断有重要价值。     

瘤样炎性脱髓鞘病MRI的临床意义

目的分析瘤样炎性脱髓鞘病(TIDD)临床、影像与病理特点,探讨磁共振(MRI)检查对TIDD的诊断价值。方法对39例TIDD患者进行头颅、脊髓CT和MRI扫描,并分析临床和影像学特点。结果病灶在头颅CT均为低密度,常规MRI扫描呈T1WI低信号、T2WI高信号,T1WI增强扫描44%可见"开环征",发病早期DWI可见高信号,FLAIR较T1WI及T2WI更清晰显示病灶及其范围。结论 DWI能发现TIDD早期病变,FLAIR较常规T1WI、T2WI敏感,"开环征"是磁共振诊断TIDD的重要辅助依据。     

貌似肿瘤的中枢神经系统脱髓鞘病

目的总结貌似肿瘤的中枢神经系统炎性脱髓鞘病病例,分析其临床、影像学及病理特点,归纳临床误诊原因并探讨诊断及鉴别诊断要点.方法分析经术后病理证实的14例临床资料,包括病史、发病情况、临床症状、体征、手术中肉眼所见,影像学包括CT/MRI表现,病理包括HE染色,Luxolfastblue(Lfb)髓鞘染色,Bodian神经纤维染色,还进行了CD68,胶质纤维酸性蛋白(GFAP)及神经丝蛋白(NF)等免疫组化染色.结果中枢神经系统脱髓鞘病临床上可出现头痛、癫等症状,急性期CT/MRI可以出现水肿等占位效应、增强后可以有环形强化,冰冻切片可以观察到胶质细胞增生等.结论中枢神经系统脱髓鞘病可以有肿瘤样的非典型性表现,临床容易造成误诊.应在临床、影像学及病理学等多方面进行鉴别诊断.     

De Seze et al. criteria: application to a series of 14 patients presenting a first severe acute demyelinating event

INTRODUCTION: The application of de Seze et al. criteria (2007) to patients presenting a first severe acute demyelinating event helps to distinguish acute disseminated encephalomyelitis (ADEM) from other CNS inflammatory diseases, with 83% sensitivity and 95% specificity. We applied these criteria to 14 patients who presented a first severe acute demyelinating event and whose later clinical course enabled clear identification of the neurological diagnosis. METHOD/PATIENTS: This study concerned 14 patients who presented a first acute demyelinating event. Initially, there were 16 patients but two were excluded because their initial clinical condition (isolated acute retrobulbar optic neuritis in one and acute cervical myelitis in the other) would have excluded them in the princeps article. We identified 11 women (78.6%) and three men (21.4%) with a mean age of 33.7+/-12.5 years. Follow-up ranged from three months to 11.5 years after the initial episode (average four years). At last follow-up, the diagnosis was ADEM in seven patients (50%) and multiple sclerosis (MS) in seven (50%). Five of seven patients in the MS group had a tumor-like presentation (71.4%), this parameter partly explaining the initial discrepancy in diagnosis. When applied to our series, de Seze criteria for ADEM exhibited 85.7% sensitivity and 71.4% specificity. CONCLUSION: Applying the new criteria, we did not find the same sensitivity, specificity, and positive and negative predictive values as in the original article. The lack of specificity arose from the misclassification of MS patients with a tumor-like presentation (two out of five false negatives). One of the explanations is that the clinical criteria used can be part of atypical forms of MS, in particular in its tumor-like presentation. De Seze et al. criteria can be an invaluable help for the clinician in the diagnosis of a first severe demyelinating event. Considering our results, these criteria should not be applied for patients with a tumor-like form of MS. A prospective study in a larger cohort is needed to confirm or invalidate these preliminary results.     

Challenges in multiple sclerosis; how to define occurence of progression

The challenges in MS are related to number of controversies in various aspects of disease but the relationship between relapses and disability progression, or aspects of MS as an inflammatory and/or neurodegenerative disease are extremely important because of its implications on prognosis and therapy of MS. MS was classically regarded as white matter inflammatory disease, while disability progression, brain and spinal cord atrophy were regarded as a consequence of global inflammation of NAWM and secondary involvement of grey matter. More recent histopathology studies, but also new, modern MRI techniques changed this view in MS as a prominent grey and white matter disease. Inflammatory demyelination of grey matter occurs early in MS sometimes even before occurrence of white matter lesions. Inspite of early therapy of MS with immunomodulatory drugs disability progression and neurodegeneration are still important and common part of MS pathogenesis. This indicate that treatment is not adequate to the predicted severity of MS, or perhaps to the basic pathogenetic mechanisms in MS. Beside acute clinical symptoms, conclusions about the severity of the disease are reflection of MRI sensitivity to detect focal WM lesions and insensitivity to detect grey matter lesions which correlate better with clinical symptoms. All presented studies and evaluations point to the necessity of changing the established diagnostic evaluation and treatment in MS. At the earliest stage of MS as well as in follow up of disease it would be necessary to apply a new MRI techniques more available for clinical practice such as DIR brain MR imaging at 3 T because of their sensitivity to detect grey matter lesions. In patient with present cortical lesions even in earliest stages of MS depending on severity of grey matter involvement more efficacious therapy like second or even third line therapy should start.     

Quantification and clinical relevance of brain atrophy in multiple sclerosis: a review

Multiple sclerosis is known to be an inflammatory demyelinating disease characterized mainly by multifocal areas of white matter lesions. Recently, cortical lesions and brain atrophy have emerged as new pathological markers of disease progression. Brain tissue loss can now be easily and reproducibly detected and quantified by MRI, which has led to an increasing amount of research correlating brain tissue loss with other MRI markers, such as white matter lesions, and to clinical disabilities (motor or cognitive), in order to assess its clinical relevance. In this review, we summarize the different MRI-based methods used to quantify whole and regional brain atrophy, and discuss the relevance of brain atrophy in MS through its relationship with characteristic brain lesions, disability and cognition. Lastly, we emphasize the role of brain atrophy in different phenotypes of MS. According to this review of the current literature, the grey matter (GM) atrophy is now well-established in MS and has been found to be strongly associated with clinical and cognitive deterioration. This GM volume loss is characterized by a focal atrophy in the deep grey nuclei but also by diffuse cortical atrophy. Phenotypic variation can be associated with different degrees and regional location of brain atrophy but more investigations are necessary to specify the discrepancies against brain atrophy between the different forms of the disease.     

Evidence for central nervous system demyelination in chronic inflammatory demyelinating polyradiculoneuropathy

It is unclear whether sporadic reports of concurrent multiple sclerosis (MS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) represent coincidence or whether these two demyelinating disorders are pathogenically related. We utilized the sensitivity of magnetic resonance imaging (MRI) in detecting central nervous system (CNS) lesions to investigate 16 patients with CIDP. Six of the 16 had periventricular, subcortical, and brainstem white matter lesions indistinguishable from those seen in MS. Three of these patients had definite clinical and laboratory evidence of MS; three others with abnormal MRIs had no findings indicative of CNS disease. Previous reports have indicated that a significant number of MS patients have peripheral nerve demyelination. Our study suggests that many CIDP patients have concurrent CNS demyelination. Taken together, these observations support the existence of a central-peripheral inflammatory demyelinating syndrome. Whether this combined demyelinating syndrome lies on a spectrum between MS and CIDP or is a separate pathogenic entity will require further investigation.     

脑内脱髓鞘假瘤病理、临床与磁共振成像对照分析

目的 探讨脑内脱髓鞘假瘤的MR影像学表现并分析其病理、临床与MRI影像三者之间的相关性. 方法 回顾性分析总结经病理证实的8例病例的病理、临床及MRI表现并加以对照. 结果 临床表现以肢体运动障碍和(或)肢体感觉障碍最常见,亦可见癫痫等少见症状.MRI影像显示病灶均单发、以侵犯皮层下白质为主,"瘤体"多为圆形或类圆形、边界多较清楚,信号多为T1均匀低信号、T2均匀高信号,多明显均一强化,多有明显占位效应及灶周水肿.病理上病变处脑组织脱髓鞘,轴索尚保存.见大量淋巴细胞在血管周围围绕浸润呈套袖现象,此外尚可见大量的巨噬细胞以及胶质细胞增生. 结论 脑内脱髓鞘假瘤病理、临床与MRI影像的相关性较好,MRI是其诊断的重要依据.其发生于幕上时与胶质瘤鉴别诊断相对较易,发生于幕下时与Ⅰ～Ⅱ级胶质瘤不易鉴别,此时应密切结合临床.对于临床与影像学诊断均较困难者,可行肾上腺皮质激素试验性治疗或脑组织活检.