视神经脊髓炎诊断进展

视神经脊髓炎是主要累及视神经和脊髓的中枢神经系统自身免疫性疾病,既往被认为是多发性硬化的一种亚型.自发现NMO-IgG以来,越来越多的证据提示视神经脊髓炎为一独立疾病.脊髓MRI所显示的长节段横贯性脊髓炎是其最具特征性的影像学改变,Wingerchuk诊断标准和美国国立多发性硬化协会推荐的诊断标准为目前常用标准.随着对视神经脊髓炎认识的加深,对视神经炎、脊髓炎及其脑部表现等临床症状的鉴别诊断水平也有所提高.     

多发性硬化的临床诊断

多发性硬化是中枢神经系统炎性脱髓鞘疾病.目前病因未阐明,发病机制与自身免疫性反应有关.临床表现的显著特点为时间多发性(多次发作)和空间多发性(多个病变部位).常用辅助检查手段包括头部或脊髓MRI、脑脊液免疫学、诱发电位等.最新的诊断标准为2010年McDonald标准.目前认为,多发性硬化与视神经脊髓炎在临床、病理、影像等方面不同,由于该病临床表现的多样性及缺乏特异性生物学诊断指标,其诊断仍然是临床医师面临的最大难题之一.     

视神经脊髓炎谱系疾病神经眼科表现

视神经脊髓炎谱系疾病包括经典视神经脊髓炎、视神经脊髓型多发性硬化、视神经脊髓炎限定型,以及伴系统性自身免疫性疾病或视神经脊髓炎特征性影像学表现的孤立性视神经炎或脊髓炎。其特征性神经眼科表现包括双侧同时或相继发生的视神经炎、视盘水肿和盘周渗出,视功能恢复差,对糖皮质激素有较好的反应性和依赖性。这些临床特征与水通道蛋白4抗体检测和头部MRI检查相结合,可为视神经脊髓炎谱系疾病与多发性硬化的早期鉴别诊断提供帮助。视神经脊髓炎谱系疾病可能出现某些特征性眼球运动异常,尚待进一步研究。     

脑脊液寡克隆区带在多发性硬化和视神经脊髓炎中的应用

目的探讨脑脊液寡克隆区带在多发性硬化和视神经脊髓炎2组疾病中的应用价值。方法通过等电聚焦电泳加免疫固定染色的实验室方法检测多发性硬化和视神经脊髓炎2组患者的脑脊液寡克隆区带,比较2组疾病的脑脊液寡克隆区带特点。结果多发性硬化患者共120例,其中脑脊液寡克隆区带阳性39例,阳性率为32.5%;视神经脊髓炎患者共45例,其中脑脊液寡克隆区带阳性4例,阳性率为8.9%。多发性硬化组脑脊液寡克隆区带阳性率明显高于视神经脊髓炎组,差异有统计学意义(P=0.002)。结论本结果对2组疾病的鉴别诊断具有一定的指导意义,且支持多发性硬化和视神经脊髓炎发病机制不同的观点。     

顽固性呃逆、呕吐的症状对于鉴别急性炎性脱髓鞘疾病和预示复发的临床意义

目的 探讨顽固性呃逆、呕吐(intractable hiccup and nausea,IHN)症状对于鉴别急性炎性脱髓鞘疾病和预示复发的意义.方法 回顾分析1995年～2011年北京协和医院病房收治的临床诊断的24例存在IHN的急性炎性脱髓鞘疾病患者的临床和影像学资料,其中,分析IHN在不同疾病组中的分布,IHN与其他复发症状的关系、IHN相对应的延髓责任病灶的影像学特点、以及血清水通道蛋白4抗体检测结果.结果 24例急性炎性脱随鞘疾病患者包括视神经脊髓炎12例,多发性硬化5例,复发性长节段脊髓炎6例,急性播散性脑脊膜炎1例,共发生IHN35次,其中40％发生在急性脱髓鞘事件之前.视神经脊髓炎患者组、复发性长节段脊髓炎患者组、多发性硬化患者组各组间相互比较,发现复发性长节段脊髓炎组延髓病灶与高颈段脊髓相连的比例是5/9,较多发性硬化组1/9比例明显增高(P =0.039 ＜0.05);视神经脊髓炎组患者血清水通道蛋白4阳性的比例是3/6,较多发性硬化组患者比例0/5明显增高(P =0.032 ＜0.05).各组患者在IHN与伴随症状的时间关系、伴随症状、出现延髓病灶的比例以及延髓病灶强化情况方面相比,无明显差异.结论 IHN可以作为鉴别急性炎性脱髓鞘疾病的参考,往往发生在其他症状之前,预示疾病复发.     

视神经脊髓炎和多发性硬化患者颈髓扩散张量成像研究

目的通过扩散张量成像(DTI)比较视神经脊髓炎和多发性硬化患者与正常对照者常规MRI表现正常脊髓的扩散性差异,并探讨其临床应用价值。方法采用平面回波成像技术对10例视神经脊髓炎、14例多发性硬化患者和13例正常对照者进行颈髓DTI检查,分别测量颈椎C2~5水平前索、侧索、后索和灰质兴趣区的部分各向异性(FA)和平均扩散率(MD)。结果与正常对照组相比,视神经脊髓炎组患者前索、侧索、后索FA值降低(均P0.05),左侧侧索、后索、灰质MD值升高(均P≤0.05);多发性硬化组患者右侧侧索、后索FA值降低(均P0.05)。与多发性硬化患者相比,视神经脊髓炎患者侧索FA值更低,左侧侧索和右侧后索MD值更高(均P0.05)。结论 DTI可以检出视神经脊髓炎和多发性硬化患者常规MRI表现正常脊髓的水分子扩散异常,进而发现二者脊髓扩散指标的差异性,为早期诊断与鉴别诊断提供重要信息。     

回顾性比较Poser标准和McDonald标准

目的比较Poser标准和McDonald标准在多发性硬化诊断中的差异。方法对80例新诊断为多发性硬化患者的临床资料进行回顾性分析,分别应用Poser标准和McDonald标准对其进行诊断分类。结果80例患者中48例(60.00%)行脊髓MRI检查,阳性率为83.33%(40/48)。应用Poser标准,41例(51.25%)为临床确诊,10例(12.50%)为实验室确诊,17例(21.25%)为临床可能,余12例(15.00%)未能进行诊断分类。应用McDonald标准,45例(56.25%)为确诊多发性硬化,35例(43.75%)为可能多发性硬化。经Poser标准临床确诊的41例患者中,除4例不完全符合McDonald标准中确诊多发性硬化条件外,其余37例均符合;实验室确诊的10例患者中,4例符合McDonald标准中确诊多发性硬化条件,其余6例为可能多发性硬化。经McDonald标准确诊的45例患者中,37例符合Poser标准中临床确诊条件,4例为实验室确诊,1例为临床可能,余3例为原发进展型多发性硬化。18例患者(33.33%)脑脊液寡克隆区带检测阳性,26例(40.63%)IgG合成率升高。4例患者脑脊液寡克隆区带或IgG检测呈阳性反应且MRI病灶≥2个,经McDonald标准诊断为确诊多发性硬化。结论McDonald标准较Poser标准更为完善,尤其适用于原发进展型多发性硬化及临床孤立综合征患者。同时应用脊髓MRI检查以及脑脊液寡克隆区带和IgG检测有利于提高McDonald标准诊断的敏感性。     

视神经脊髓炎研究发展史

19世纪中叶对视神经脊髓炎的认识是双侧同时发病的视神经炎和脊髓炎,而后续研究则发现两侧视神经炎呈先后发病,一般相隔数周、数月甚至数年。自2004年水通道蛋白4抗体被发现以来,"视神经脊髓炎"的概念遇到挑战并被修改。因此,2007年提出"视神经脊髓炎谱系疾病"的概念,并由此产生一系列不同命名,包括视神经脊髓型多发性硬化、视神经脊髓炎谱系疾病、视神经脊髓炎疾病谱系、视神经脊髓炎谱系、视神经脊髓炎扩展谱系等。笔者拟通过对不同时期"视神经脊髓炎"概念的回顾,对19~21世纪有关视神经脊髓炎研究的历史沿革进行全面复习,前瞻未来研究方向。     

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.     

视神经脊髓炎标志性抗体的发现及其意义

视神经脊髓炎是一种选择性损伤视神经和脊髓的中枢神经系统自身免疫性脱髓鞘性疾病。其与多发性硬化在免疫病理、发病机制、临床表现、治疗和预后等方面均不尽相同。关于视神经脊髓炎是多发性硬化的一种亚型还是一种独立的疾病在学术上一直存在争议。最近在视神经脊髓炎患者血清中检测到一种标志性抗体,并发现其特异性靶点为水通道蛋白-4。这一重要研究进展将会对重新认识和进一步研究视神经脊髓炎以及对其临床诊断和治疗产生重要影响。     

Reaction of spinal cord central canal cells to cord transection and their contribution to cord regeneration

After transection, the spinal cord of the eel Anguilla quickly regrows and reconnects, and function recovers. We describe here the changes in the central canal region that accompany this regeneration by using serial semithin plastic sections and immunohistochemistry. The progress of axonal regrowth was followed in material labeled with DiI. The canal of the uninjured cord is surrounded by four cell types: S-100-immunopositive ependymocytes, S-100- and glial fibrillary acidic protein (GFAP)-immunopositive tanycytes, vimentin-immunopositive dorsally located cells, and lateral and ventral liquor-contacting neurons, which label for either gamma-aminobutyric acid (GABA) or tyrosine hydroxylase (TH). After cord transection, a new central canal forms rapidly as small groups of cells at the leading edges of the transection create flat "plates" that serve as templates for subsequent formation of the lateral and dorsal walls. Profile counts and 5-bromo-2'-deoxyuridine immunohistochemistry indicate that these cells are dividing rapidly during the first 20 days of the repair process. The newly formed canal, which bridges the transection by day 10 but is not complete until about day 20, is greatly enlarged (相似文献   

Spinal cord ischemia

Traditional data and recent advances in the field of spinal cord ischemia are reviewed, with special attention to clinical and radiological features, as well as underlying etiology, outcome, and pathophysiology. Acute spinal cord ischemia includes arterial and venous infarction and global ischemia resulting from cardiac arrest or severe hypotension. MRI has become the technique of choice for the imaging diagnosis of spinal cord infarction. Correlation of clinical and MRI data has allowed diagnosis of clinical syndromes due to small infarcts in the central or peripheral arterial territory of the spinal cord. Diffusion-weighted MR imaging may increase the sensitivity and specificity for diagnosis of acute spinal cord infarction. Diagnosis of venous spinal cord infarction remains difficult. As for global ischemia, neuropathological studies demonstrated a great sensitivity of spinal cord to ischemia, with selective vulnerability of lumbosacral neurons. Chronic spinal cord ischemia results in a syndrome of progressive myelopathy. The cause is usually an arteriovenous malformation. Most often, diagnosis may be suspected on MRI, leading to diagnostic, and eventually therapeutic, spinal angiography.     

Spinal cord compression

Back pain and suspected spinal cord compression remains one of the most frequent reasons for neurologic consultation in cancer patients. While treatment generally results in stabilization or improvement, early diagnosis remains the cornerstone of improving neurologic outcome. This article reviews the clinical features, differential diagnosis, and management of neoplastic epidural spinal cord compression.     

Spinal cord sarcoidosis

Three patients with spinal cord involvement by sarcoid are described and the literature is reviewed. Fifteen patients with histologically verified sarcoidosis involving the spinal cord presented with progressive myelopathy due to tumor. Myelography variously showed an extramedullary intradural mass; an intramedullary mass; a thinned, atrophic cord; or normal configuration. Although laminectomy and biopsy are often required to establish the diagnosis, treatment is medical, using corticosteroid drugs. Treatment seldom leads to complete recovery, but neurological deficits may stabilize or improve.     

The spinal cord

Magnetic resonance imaging (MRI) has become the imaging modality of choice in spinal cord diseases. Computerized tomography (CT) myelography remains a valuable alternative and may be performed even on patients with a known sensitivity to iodine contrast media. Ultrasonography provides excellent intraoperative evaluation of spinal cord injury. MRI, however, offers new knowledge about spinal cord disease, especially in trauma, ischemia and degenerative lesions; using this technique direct visualization of ischemic spinal cord lesions is presently possible. Rare infections or degenerative spinal cord lesions are imaged albeit in a non-specific manner. Cervical and thoracic disk disease may cause severe myelopathy, yet close correlation between images and symptomatology remains mandatory.