带状疱疹性脊髓炎7例临床特征分析

目的探讨带状疱疹性脊髓炎的临床特点、影像学特征、诊疗及预后。方法对7例带状疱疹性脊髓炎患者的临床资料进行回顾性分析,并进行临床随访。结果 7例患者均有特征性皮疹及脊髓损害症状。5例以皮疹首发,2例以脊髓损害症状首发。4例受累皮节与脊髓节段相符,3例受累皮节低于脊髓损害水平。7例脊髓MR均表现为受累脊髓节段性不规则长T_1、长T_2信号,2例有强化。胸髓病变5例,颈髓2例。7例患者均予激素治疗,5例患者同时予抗病毒治疗。经12~36个月(平均22.7±8.1个月)随访,5例患者痊愈,1例遗留双手指轻微麻木,1例遗留排尿困难。结论带状疱疹性脊髓炎常表现特征性的皮疹和脊髓症状,积极的抗病毒药物和皮质激素治疗,预后较好。     

脊髓脂肪瘤

本文报告较为少见的脊髓脂肪瘤4例,根据治疗结果,提出不同于文献报道的手术方法。 临床资料 一般资料 男性1例,女性2例,年龄12～20岁,病程3月～1年9月,侵犯部位分别为颈延髓、颈胸,胸,胸腰各1例,侵犯脊髓最短者为5节段,最长者9节段。 临床表现 首发症状以一个肢体麻木伴力弱3     

系统性红斑狼疮脊髓损害的临床与影像学分析

系统性红斑狼疮(SLE)是一种累及多系统多器官的具有多种自身抗体的自体免疫性疾病,可引起多种神经系统并发症,其中SLE相关性脊髓损害少见,发生率仅为占SLE的1%～4 %。我们回顾我院1995年1月至2 0 0 2年5月共10 5 2例SLE患者,出现脊髓损害者15例,总结分析其临床表现,实验室检查,发病特点等,对SLE脊髓损害作较深入的探讨。资料与方法:我院住院治疗的SLE患者10 5 2例,均符合美国风湿病协会1982年所制定的诊断标准。有中枢神经系统(CNS)损害者12 4例,其中15例出现脊髓损害的症状体征,约占神经系统损害的12 1%。采用改良LACC标准评价…     

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.     

结核性脑（脊髓）膜炎合并脊髓空洞症的临床及MRI特征

目的总结结核性脑（脊髓）膜炎合并脊髓空洞症的临床以及MRI特点。方法回顾分析9例结核性脑（脊髓）膜炎合并脊髓空洞症患者的临床表现、脑脊液和MRI结果。结果平均发病年龄（26．30±8．30）岁,男女之比约为2：1;呈亚急性或慢性起病。首发症状以发热伴头痛或咳嗽,下肢乏力、麻木及大小便障碍（6例）为主,亦可继发于抗结核治疗结束后6个月至1年;症状与体征不对称,节段性分离性感觉障碍不典型。MRI表现为脊髓中央纵行“串珠”状、“腊肠”样、梭形或细长条形长T1、长T2脑脊液样信号,空洞内及空洞壁无强化;病变部位蛛网膜下隙变窄且伴脊膜强化;脊髓空洞呈多房性,可累及颈髓（5例）、胸髓（8例）、腰髓（6例）或颈胸腰髓同时受累（4例）。病情轻重程度与空洞直径和长度不平行。结论脊髓空洞症可为结核性脑（脊髓）膜炎早期或迟发性并发症。其临床表现可不典型,空洞以胸髓受累常见,具多房性、多节段特点,病情轻重程度与影像学改变可不平行。提高对该病的认识并及时行MRI检查有助于早期诊断。     

系统性红斑狼疮合并中枢神经系统感染34例临床分析

目的研究分析系统性红斑狼疮(SLE)合并中枢神经系统感染的临床表现。方法回顾性分析2001年1月至2011年12月石家庄平安医院34例SLE合并中枢神经系统感染患者临床资料,提出临床特征及其相关危险因素。结果 34例SLE合并中枢神经系统感染,发生率为1.44%;其中结核性脑膜炎25例(73.53%),新型隐球菌脑膜炎7例(20.59%),病毒性脑膜炎2例(5.88%)。给予内科常规治疗,25例结核性脑膜炎痊愈21例,死亡4例;7例隐球菌脑膜炎痊愈3例,死亡4例。病毒性脑膜炎痊愈1例,死亡1例。本文SLE合并中枢神经系统感染34例中总共死亡9例(26.47%)。全部患者在诊断前均经过糖皮质激素治疗,28例患者应用较大剂量激素治疗,18例患者联合免疫抑制剂治疗。SLE合并中枢神经系统感染的临床特点主要为高热、头痛及意识障碍;狼疮活动度评分(SLEDAI)平均9±3分。脑脊液检查发现糖和氯化物降低明显。结论①系统性红斑狼疮合并中枢神经系统感染以结核性脑膜炎最常见;②肾上腺皮质激素、免疫抑制剂的应用是SLE合并感染的危险因素;③SLE合并中枢神经系统感染与狼疮活动度无关,而神经精神狼疮多发生于狼疮高度活动期,SLEDAI评分对二者鉴别诊断有意义;④狼疮合并中枢神经系统感染以高热、头痛及颅内压增高为主要表现,反复腰穿脑脊液检查对SLE合并中枢神经系统感染的诊断和判断预后有重要价值。     

合并脊髓静脉高压综合征的脊髓血管母细胞瘤的手术治疗

目的 总结合并脊髓静脉高压综合征（VHM）的脊髓血管母细胞瘤的诊治经验。方法 回顾性分析2005~2019年手术治疗的10例脊髓血管母细胞瘤的临床资料,结合术前MRI T2像及病人临床表现评估VHM,并总结脊髓血管母细胞瘤合并VHM的诊治经验。结果 单发肿瘤9例,其中肿瘤位于颈段3例、胸段4例、腰段2例;多发1例（颈段、胸段及腰段均有）。术前存在VHM 6例,术前MRI均可见病变节段以外的脊髓中央区MRI T2高信号,存在明显的脊髓功能损伤症状,如肢体运动功能障碍或大小便功能障碍等。10例肿瘤均全切除,其中1例多发肿瘤一次切除3个胸段肿瘤。无手术死亡病例。结论 脊髓血管母细胞瘤为富含血管的血管性肿瘤,往往有高流量的血流通过,引流静脉常迂曲扩张并同脊髓表面正常引流静脉沟通导致VHM,有症状者应早期手术,避免出现不可逆的神经功能损伤,肿瘤切除后VHM自然治愈。     

以神经系统损害为首发症状的干燥综合征3例报道并文献复习

目的探讨干燥综合征合并神经系统损害的机制、临床表现及治疗方法。方法报告3例以神经系统损害为首发表现的干燥综合征病例,并对相关文献进行复习。结果 3例患者中2例为中枢神经病变(其中1例合并周围神经病变),1例为周围神经病变。经丙种球蛋白、糖皮质激素、营养神经等药物治疗后,患者症状均得到不同程度改善。结论干燥综合征为自身免疫性疾病,因累及部位的不同,合并神经系统病变的临床症状也呈多样性,早期临床症状常缺乏典型性,应详细询问病史并结合实验室检测、影像学及唇腺活检等检查,减少误诊。     

视神经脊髓炎(Devic''''s综合征)临床研究进展

近来研究表明视神经脊髓炎 (NMO)的临床经过、实验室和神经影像学特点均与MS不同。其病理改变限于视神经和脊髓 ,破坏性病变明显 ;临床表现较MS严重 ,单相病程者的严重的索引事件可在短时间内相继发生 ,复发病程者索引事件间隔期较长。CSF特点类似于MS ,但 1/ 3以上患者CSF细胞数 >5 0 /mm3 ,MRI检查显示 88%病例的脊髓纵向融合病变≥ 3个脊柱节段 ,通常为 6～ 10个节段 ,少数发展为局部脊髓萎缩。NMO预后较差 ,以复发病程型为著。     

急性炎性脱髓鞘性多发性神经病四例诊疗体会

目的报告我科收治的临床少见的4例急性炎性脱髓鞘性多发性神经病(AIDP)患者。方法1例疑似肉毒中毒的临床表现,1例以颅内压增高为首发症状,1例为疑似脊髓横贯性损伤,1例以小脑性共济失调为首发症状,住院后,出现四肢软瘫、临床检测肌电图呈神经源性受损,脑脊液有蛋白-细胞分离现象、采用激素及营养神经治疗病情好转。结果作者体会AIDP可以有颅神经、脊髓和小脑损害、高颅压的首发症状,但动态观察仍以运动损害较重,颅神经运动根的选择性损害,主观感觉障碍重于客观感觉障碍,脑脊液的蛋白-细胞分离现象,肌电图神经源性损害,相对良好的预后等特点。结论在临床实践中应注意与其他疾病鉴别。     

Progressive necrotizing myelopathy: part of the spectrum of neuromyelitis optica?

This case series reviews the clinical, radiographic and laboratory findings of five patients with progressive idiopathic myelopathy with evidence of cord necrosis who presented in our institution over a 5 year period ending in May 2005. Patients fulfilling the following criteria were included: (1) presentation with myelopathy without overt visual involvement at initial presentation; (2) demonstration with magnetic resonance imaging (MRI) of contiguously abnormal signal in the spinal cord spanning at least three vertebral segments without evidence of arteriovenous malformation or significant disk disease; (3) absence of systemic disease or neoplasm. All patients were women, identified themselves as African American and were older than 35 years. Pain was reported at initial presentation in four cases. The distinctive feature was a relapsing course with intervening variable improvement of function and progression to quadriplegia in less than 4 years. An increased IgG index and/or oligoclonal banding was detected in two patients. The leukocyte count in the cerebrospinal fluid (CSF) was elevated in all cases but in only one specimen did the count exceed 50 cells. None of the patients initially had clinical signs of an optic neuropathy but unilaterally prolonged visual evoked potentials were present in one individual who went on to developed optic neuritis 19 months after the first clinical presentation. Another patient developed optic neuritis 45 months after disease onset. Immunomodulatory and plasma exchange therapy were of some benefit at least early in the course but the disease progressed despite these interventions. Neuromyelitis optica (NMO)-IgG antibody, a serum or CSF marker described in individuals with classic NMO and optico-spinal multiple sclerosis (MS), was present in all cases. On the basis of shared clinical and imaging features in the cord, progressive necrotizing myelopathy observed in this case series exhibits key features of a limited form of NMO (Devic's disease) and opticospinal MS. The presence of NMO-IgG antibody marker suggests that progressive necrotizing myelopathy is part of a disease spectrum of which traditional NMO is a select presentation.     

Lupus-related myelopathy: report of three cases and review of the literature.

Transverse myelopathy is an uncommon complication of systemic lupus erythematosus (SLE). Three patients with SLE are reported who developed transverse myelopathy, including the neuropathological findings in one patient on whom necropsy was performed. Paraparesis was present in all three cases, but definite sensory changes were present in only one patient. In two patients, the CSF findings were remarkable for elevated protein and depressed glucose concentrations. Microscopic examination of the brain demonstrated small, scattered foci of recent necrosis consistent with microinfarctions. Striking abnormalities were found in the spinal cord at all levels, including multiple foci of vacuolar spongy degeneration in the peripheral white matter, as well as ballooning of myelin sheaths, swollen axons, myelin pallor, and loss of glial nuclei. The pathological findings in previously reported cases of SLE-related transverse myelopathy are reviewed, and the possible pathogenesis of the findings in our case are discussed.     

脊髓型颈椎病合并胸椎管狭窄症的诊断与治疗

目的：探讨脊髓型颈椎病合并胸椎管狭窄症的诊断与治疗。方法：回顾性分析4例脊髓型颈椎病合并胸椎管狭窄症的诊断与治疗。其中2例先行颈椎减压和稳定手术，后行胸椎板切除减压术；1例颈、胸椎同时行减压；1例先行胸椎板切除减压术，后行颈椎板成形术。结果：神经病学损害完全恢复2例，明显改善2例。结论：脊髓型颈椎病合并胸椎管狭窄症手术治疗可取得良好的效果。     

Myelopathy as the main presenting feature of systemic lupus erythematosus

Myelopathy is a rare complication of systemic lupus erythematosus (SLE). Only 34 cases have been fully described in the literature. 5 other cases have been listed among large surveys of SLE patients. It is particularly unusual to find evidence of spinal cord involvement as the presenting feature of the disease. 2 cases are described in which SLE presented as a myelopathy and both made a satisfactory recovery with steroid treatment. The literature concerning SLE-myelopathy is reviewed.     

副肿瘤性脊髓病的临床和影像特点

目的报道副肿瘤性脊髓病的临床表现、影像和血清学特征。方法检索宣武医院2010年1月至2019年6月期间的副肿瘤性脊髓病住院患者,系统分析其临床表现、血清学和影像学数据。结果10例患者中符合确定的副肿瘤综合征7例,可能的副肿瘤综合征3例。患者中亚急性起病3例,慢性起病7例,脊髓病起病后确诊时间平均为10.1个月。10例患者中7例表现为单纯副肿瘤性脊髓病,2例伴有周围神经病,1例伴有僵人综合征。10例患者中9例患者行腰穿检查脑脊液,其中8例有异常,5例淋巴细胞增多,5例蛋白升高,6例寡克隆区带阳性;7例患者神经肿瘤抗体阳性,分别为抗amphiphysin抗体1例、抗CRMP-5抗体1例、抗Hu抗体3例、抗Yo抗体1例、抗Ri抗体1例;7例患者病理证实为恶性肿瘤。10例患者中,脊髓磁共振异常5例,其中长脊髓病变4例(其中累及椎体节段14个1例,6个1例,3个2例),短脊髓病变1例,病灶均对称性累及传导束或者脊髓灰质。10例患者中9例接受治疗,其中单纯手术治疗3例,单纯糖皮质激素治疗1例,手术+糖皮质激素治疗1例,糖皮质激素+肿瘤化疗治疗2例,手术+糖皮质激素+肿瘤化疗治疗3例,患者临床症状均无明显改善。结论副肿瘤性脊髓病罕见,多表现为慢性进行性脊髓病且多先于肿瘤出现;多数患者脑脊液表现异常,影像学可表现为脊髓长节段病灶。该病治疗上以肿瘤治疗为主,免疫治疗为辅。     

Acute myelopathy. Retrospective clinical, laboratory, MRI and outcome analysis of 49 cases.

Forty nine consecutive cases of acute myelopathy were related to the following pathologies: 31 MS, four spinal cord infarction, four parainfectious, one antiphospolipid syndrome and nine of unknown origin. Sensory deficits were most frequent in MS and in myelopathy of unknown origin. In spinal cord infarction motor deficits and sphincter dysfunction were present in all cases. In parainfectious myelopathy sensory-motor deficits and sphincter dysfunction were most frequent. Myelopathy extended over less than two vertebral segments in MS and in myelopathy of unknown origin. Myelopathy extended over more than two vertebral segments in spinal cord infarction and in parainfectious myelopathy. The clinical outcome was good in MS, parainfectious myelopathy and myelopathy of unknown origin, but poor in spinal cord infarction. Our findings suggest that various aetiologies of acute myelopathy may be differentiated on the basis of distinct clinical, MRI, laboratory and outcome data.     

Progressive necrotic myelopathy: clinical course in 9 patients

OBJECTIVE: To review the clinical, laboratory, and radiological findings of 9 patients who had progressive idiopathic myelopathy with evidence of spinal cord necrosis. DESIGN AND METHODS: We reviewed personally examined cases of myelopathy that fulfilled the following criteria: (1) regional loss of reflexes, flaccidity, and muscle atrophy; (2) magnetic resonance imaging showing a shrunken or cavitated cord without evidence of arteriovenous malformation; (3) electromyogram showing denervation over several contiguous spinal cord sgements with preservation of sensory potentials in some cases; and (4) the absence of evidence of systemic disease or neoplasm. RESULTS: The illness began in these patients after the age of 40 years, with prominent burning or tingling limb pain, occasionally with radicular features or with less well-defined back, neck, or abdominal pain. Leg or infrequently arm weakness appeared concurrently or soon after the onset of pain. The most distinctive feature was a saltatory progression of symptoms, punctuated by both acute and subacute worsenings approximately every 3 to 9 months, culminating in paraplegia or tetraplegia. The distinguishing clinical findings, together indicative of destruction of gray matter elements of the cord, were limb atrophy, persistent areflexia, and flaccidity. The concentration of cerebrospinal fluid protein was typically elevated between 500 g/L and 1000 g/L, without oligoclonal bands, accompanied infrequently by pleocytosis. Magnetic resonance imaging showed features suggesting cord necrosis, specifically swelling, T2-weighted hyperintensity, and gadolinium enhancement over several spinal cord segments, succeeded months later by atrophy in the same regions. Necrosis of the cord was found in biopsy material from one patient and postmortem pathology in another case, but inflammation and blood vessel abnormalities were absent. Only 2 patients had prolonged visual evoked responses. The disease progressed despite immune-modulating treatments although several patients had brief epochs of limited improvement. CONCLUSIONS: The saltatory course, prolonged visual evoked responses in 2 patients, and a cranial abnormality on magnetic resonance imaging in another, raised the possibility of a link to multiple sclerosis. However, the normal cranial magnetic resonance imaging scans in 6 other patients, uniformly absent oligoclonal bands, and poor response to treatment were atypical for multiple sclerosis. On the basis of shared clinical and laboratory features, idiopathic progressive necrotic myelopathy is indistinguishable from a limited form of Devic disease.     

Tuberculous myelopathy: current aspects of neurologic sequels in the southeast of Iran

OBJECTIVE: The spinal cord may be affected in different ways in the course of tuberculous infection. The objective of this study was to determine the epidemiologic features, clinical manifestations, pathophysiologic mechanisms, neurologic sequels, and treatment outcomes of tuberculous myelopathy in the southeast of Iran in which the disease is endemic. METHOD: A retrospective study was scheduled. All cases of tuberculous myelopathy treated in our hospital over the last 7 years were reviewed. Only those with histologic or microbiologic confirmation or those who responded to antituberculous therapy were included. RESULTS: During a 7-year period, 43 cases of tuberculous myelopathy were found. The most frequent clinical manifestations were backache (86%) and fever (67%). Twenty patients were paraparetic or paraplegic and 40% had kyphosis. Imaging studies revealed thoracic and/or lumbar spine involvement in 92% of patients with tuberculous spondylitis. Fifteen, five and two patients had sensorimotor spinal cord syndrome, radiculomyelitis and intramedullary syringomyelic syndrome, respectively. Forty-seven percent required surgical intervention. There was improvement in 81% of the cases. CONCLUSION: Different pathophysiologic mechanisms acted on the clinical manifestations of spinal neurotuberculosis. A good outcome is expected if the diagnosis is made in the early stages before the appearance of spinal deformity and neurologic deficits.     

Nationwide survey of necrotizing myelopathy associated with malignancy in Japan

Twenty-two cases of transverse myelopathy associated with malignancy were collected in nationwide survey in Japan. Paraneoplastic necrotizing myelopathy (PNM) was suspected in 9 of them and 8 cases were diagnosed as radiation myelopathy (RM), clinically and pathologically. Other 5 cases had different causes of transverse myelopathy. In comparison with PNM and RM, malignant lymphoma, flaccid paraplegia and sphincter dysfunction were highly associated in the patients with PNM. On the other hand, lung cancer, spastic paraplegia, abnormality in spinal MRI and dysesthesia in legs as an initial symptom were prevalent in the cases of RM. Six cases of them were compared pathologically. Although all cases had no metastasis of malignant cells, five cases of PMN showed acute and chronic necrosis and rarefaction of spinal cord with or without perivascular cuffing. One case of RM had focal spinal cord atrophy and no lymphocytes infiltration. Immunohistochemically, herpes simplex virus type 2 (HSV2) infection in spinal cord was shown in the 2 cases of the necrotizing myelopathy. Virus infection such as HSV2 could be one of causes of PNM and virological study must be done in the cases of necrotizing myelopathy associated with malignancy.     

Vitamin B12 extensive thoracic myelopathy: clinical, radiological and prognostic aspects. Two cases report and literature review

The myelopathy caused by vitamin B12 deficiency is known as subacute combined degeneration. It is rare, but a well known cause of demyelination of the dorsal columns of the spinal cord. The magnetic resonance imaging is characterized by an increased signal on T2-weighted images involving the posterior columns of cervical and thoracic cord. There have been few cases in literature with extensive lesions (more than seven levels) of the thoracic spinal cord. The clinical and radiological improvements are possible if the replacement of vitamin B12 is initiated precocious. We present two rare cases of extensive thoracic myelopathy due to vitamin B12 deficiency. The first is a young woman with complete clinical recovery and important radiologic improvement after early treatment. In addition, the second case is an older man with partial response to the treatment. Those cases illustrate the importance of considering vitamin B12 deficiency in any patient, who presents with myelopathy.