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。     

~1H-MRS在瘤样炎性脱髓鞘病与低级别胶质瘤鉴别诊断中的应用

目的探讨氢质子磁共振波谱(1H-MRS)在瘤样炎性脱髓鞘病(TIDD)与低级别胶质瘤(LGG)鉴别诊断中的应用价值。方法选取12例经手术/活检病理证实或治疗后随诊证实的TIDD患者为病例组,10例经手术病理证实的LGG患者为对照组。采用3.0T磁共振(MRI)成像系统,所有患者手术和/或药物治疗前全部行横轴位T1WI-FLAIR、FSE-T2WI、T2WI-FLAIR及1H-MRS检查。结果与对侧正常脑组织相比较,TIDD组患者病灶侧Cho/Cr比值升高,NAA/Cr及NAA/Cho比值降低,差异有统计学意义(p<0.05)。与对侧正常脑组织相比较,LGG组患者病灶侧Cho/Cr比值升高,NAA/Cr及NAA/Cho比值降低,差异有统计学意义(p<0.05)。TIDD组与LGG组病灶区比较,Cho/Cr比值、NAA/Cho比值和Lac/Cr峰差异p<0.05,而NAA/Cr比值差异p>0.05。结论1H-MRS能够无创性检测脑组织代谢物含量。LGG患者比TIDD患者Cho/Cr比值增高、NAA/Cho比值降低及Lac/Cr峰降低。1H-MRS对TIDD和LGG的诊断和鉴别具有重要临床价值。     

脑与脊髓同时受累的瘤样炎性脱髓鞘病一例

目的了解瘤样炎性脱髓鞘病(TIDD)的临床表现、诊断及治疗方法。方法介绍我院收治的1例脑与脊髓同时受累的TIDD患者的临床表现、影像学特点及治疗方法。结果本病临床症状多种多样,出现头晕、头痛及肢体无力等症状,进展迅速,头颅与脊髓影像学检查可见异常信号。给予大剂量注射用甲泼尼龙琥珀酸钠冲击治疗,效果显著,预后较好。结论本病为中枢神经系统脱髓鞘疾病,临床表现多种多样,易与颅内肿瘤相混淆,激素冲击治疗有效。     

肿瘤样炎性脱髓鞘病的CT、MRI表现与临床

目的 探讨貌似肿瘤的中枢神经系统炎性脱髓鞘病的临床与影像学特点,分析误诊原因,并归纳诊断及鉴别诊断要点。方法 对14例经术后病理检查确诊中的中枢神经系统炎性脱髓鞘病患者的临床资料、影像学改变进行分析。结果 中枢神经系统炎性脱髓鞘病CT或MRI检查可以出现水肿及占位效应,增强后可以有环形强化,很难与中枢神经系统肿瘤相鉴别。本组14例患者术前均误诊为中枢神经系统肿瘤。结论 中枢神经系统炎性脱髓鞘病CT或MRI可以有肿瘤样的非典型性表面,不易鉴别,容易造成误诊。应结合临床表现、病程、实验室检查及影像学特征多方面进行鉴别诊断。     

假瘤样脱髓鞘病

中枢神经炎性脱髓鞘病,以多发性硬化多见。其特点是病灶多发,具有缓解复发的临床表现。但近年来发现部分炎性脱髓鞘病临床表现无明显缓解复发,病灶孤立,影像学检查呈现环状或弥散增强的占位效应,常常被误诊中枢神经系统肿瘤,多数由病理学确诊,称为假瘤样脱髓鞘病。现报道两例并复习献,分析探讨这种疾病的临床特点。     

经病理证实的以瘤样脱髓鞘病为首发表现的多发性硬化临床分析

目的研究经病理证实的以瘤样脱髓鞘病变(tumefactive demyelinating lesions,TDL)为首发表现的多发性硬化(multiple sclerosis,MS)患者的临床、影像学及病理学特点。方法收集2011-01-01-2013-08-05就诊于海军总医院以TDL为首发表现的MS患者6例。6例患者均在首次发病时行脑立体定向活检术,分析所有患者的临床资料及诊治经过,并定期随访24个月(最后1例患者末次随访日期为2015-10-20)。结果 6例患者中男4例、女2例,年龄12~44岁,平均(33.5±12.4)岁。6例患者中:(1)首次发病(TDL表现)表现为头痛3例、肢体无力2例、头晕伴耳鸣1例;实验室检查:IgG合成指数3例升高;病灶部位:分布于侧脑室旁6例、基底节区3例、额叶4例、延髓3例、颞叶2例、胼胝体2例。(2)复发(MS确诊后表现)表现为肢体麻木伴感觉异常3例、伴肢体无力2例,头晕伴耳鸣1例,1例随访仅见2次影像学复发,无神经系统功能缺损症状;实验室检查:脑脊液寡克隆区带(OCB)3例阳性,血清MBP升高4例,脑脊液MBP升高3例;病灶部位:多累及额叶4例、侧脑室旁4例、颞叶3例、基底节区3例、胼胝体2例、延髓3例、大脑脚2例、桥脑2例。6例均以TDL起病,TDL出现距MS确诊的时间约6~24个月,平均(12.0±7.6)个月。所有患者均有≥2次临床或影像学发作史,平均复发次数为(3.2±1.0)次,年平均复发次数约(1.5±0.5)次/年。结论以TDL首发的MS临床表现多样,多以头痛起病,首次发病与复发期的脑MRI病灶多位于脑室旁,复发期脑脊液OCB可能由阴性转为阳性。重视复发期脑脊液OCB和脑MRI的动态复查有助于判断TDL的临床转归,减少MS的临床漏诊。     

散发性Creutzfeldt-Jakob病的临床和影像学特点

目的探讨散发性Creutzfeldt-Jakob病(sCJD)的临床和影像学特点。方法回顾性分析4例sCJD患者的临床资料。结果4例sCJD患者均表现为亚急性起病,进行性痴呆,伴有肌阵挛;头颅MRI显示对称性或非对称性大脑皮质彩带样和(或)基底节弥散加权成像(DWI)高信号。结论sCJD的临床特点为进展性痴呆伴肌阵挛,头颅MRI特别是DWI出现高信号为其病变特点。     

假瘤型炎性脱髓鞘病的临床及病理

目的 :探讨假瘤型炎性脱髓病的临床病理特点。方法 :分析 5例假瘤型炎性脱髓鞘病的临床、影像及病理特点。结果 :本组临床特点 :1亚急性或慢性起病 ,逐渐进展。 2症状、体征及 MRI均提示为单一病灶。 3MRI所见病灶比 CT大 ,可有占位效应 ,也可呈周边花边样或环形强化 (例 2呈均匀强化 )。病理呈炎性脱髓鞘改变 ;白质内大片脱髓鞘区 ,血管周围淋巴细胞浸润 ,呈袖套样 ,伴有胶质细胞增生。也可主要累及灰质。结论 :1假瘤型炎症性脱髓鞘病可能是多发性硬化 (MS)中的一种特殊类型 ,或者是 MS与急性播散性脑脊髓炎之间的过渡类型。 2假瘤型炎性脱髓鞘病与胶质瘤不易鉴别 ,可试用肾上腺皮质激素治疗或者进行组织活检 ,不可急于手术。     

中枢神经系统瘤样脱髓鞘病变诊治指南

正瘤样脱髓鞘病变(tumefactive demylinating lesions,TDLs)~([1-3]),既往也称瘤样炎性脱髓鞘病(tumor-like inflammatory demyelinating disease,TIDD)~([4-5]),或脱髓鞘假瘤(demyelinating pseudotumor,DPT)~([6]),是中枢神经系统(central nervous system,CNS)一种相对特殊类型的免疫介导的炎性脱髓鞘病变~([7]),绝大多数为脑内病变,脊髓     

Nerve root hypertrophy in chronic inflammatory demyelinating polyneuropathy

A patient with chronic inflammatory demyelinating polyneuropathy (ClDP) and centrel demyelinating disease is desoribed in whom striking nodular filling defects on multiple lumbar–sacral nerve roots, mimicking neurofibromata, were observed at myelography and magnetic resonance imaging. We suggest that these lesions are secondary to recurrent segmental demyelination and remyelination and that the differential diagnosis of this radiological feature should include CIDP. © 1994 John Wiley & Sons, Inc.     

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。     

Focal cranial nerve involvement in chronic inflammatory demyelinating polyneuropathy: clinical and MRI evidence of peripheral and central lesions

Summary Five cases of chronic inflammatory demyelinating polyneuropathy are described in which cranial nerve involvement accompanied a more generalized neuropathy. Clinical, electrophysiological, radiological and nerve biopsy findings are presented. Cranial nerve lesions in this form of polyneuropathy may be related to lesions of the peripheral nerves or of the central nervous system, when they may be accompanied by MRI evidence of more widespread CNS demyelinating lesions. In cases of early onset, the occurrence of focal cranial nerve lesions may serve to distinguish chronic inflammatory from inherited demyelinating polyneuropathies.     

Pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy

The acute lesions of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) consist of endoneurial foci of chemokine and chemokine receptor expression and T cell and macrophage activation. The myelin protein antigens, P2, P0, and PMP22, each induce experimental autoimmune neuritis in rodent models and might be autoantigens in CIDP. The strongest evidence incriminates P0, to which antibodies have been found in 20% of cases. Failure of regulatory T-cell mechanism is thought to underlie persistent or recurrent disease, differentiating CIDP from the acute inflammatory demyelinating polyradiculoneuropathy form of Guillain-Barré syndrome. Corticosteroids, intravenous immunoglobulin and plasma exchange each provide short term benefit but the possible long-term benefits of immunosuppressive drugs have yet to be confirmed in randomised, controlled trials.     

慢性炎性脱髓鞘性多发性神经病的治疗进展

慢性炎性脱髓鞘性多发性神经病（chronic inflammatory demyelinating polyradiculopathy,CIDP）是一种获得性的免疫介导的周围神经病.临床特征包括进展性或复发性的肢体无力、感觉缺失和腱反射消失等.     

Prospective analysis of gait characteristics in chronic inflammatory demyelinating polyradiculoneuropathy

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a disorder that may lead to functional impairment, including gait abnormalities. Our aim was to analyze gait characteristics in patients with CIDP compared to healthy controls (HC). Moreover, we sought to determine changes of gait parameters after six-month follow-up period. Twenty-four patients with CIDP and 24 HCs performed basic walking task, dual-motor task, dual-mental task, and combined task using the same GAITRite system. Lower limb MRC-SS and lower limb INCAT disability score were assessed. Fourteen patients were retested after six months. Majority of gait parameters showed significant differences in all experimental conditions when compared between CIDP and HCs. The most consistent findings in CIDP were shorter stride length (SL), prolonged cycle time (CT) and double support time (DS), as well as increased variation of SL and of swing time (ST) (p < 0.05). During follow-up, INCAT improved in nine (64.3%) of 14 patients and MRC-SS improved in eight (57.1%) patients. Six-month changes of CT and its variation during combined task significantly differentiated patients with improved vs. non-improved INCAT (p < 0.05). In conclusion, patients with CIDP had slower gait with prolonged DS and with shorter SL compared to HCs. Increased variation of SL and of ST in CIDP may suggest a potential risk for instability and falls. Shorter CT duration and less CT variation during time correlated well with improvement in disability.