MRI在神经白塞综合征诊疗中的应用

目的 探讨神经白塞综合征脑部病理变化与临床神经系统表现的相互关系，为此病的早期诊治提供依据。方法 采用MRI对患者颅脑进行检查，以发现脑部病变部位、性质、程度等异常变化，与临床症状体征结合分析。结果 本文结果证实神经白塞综合征患者脑部都有相应的病理改变，MRI检查结果均有明显的影象学变化，并与症状体征相对应。结论 神经白塞综合征可通过MRI检查发现脑部早期病理改变，为此病的早诊断、早治疗提供依据，对治疗效果与顸后均有重要价值。     

以周围神经病为首发症状的6例结节性多动脉炎的临床病理特点

目的回顾性分析6例以周围神经病为首发症状的结节性多动脉炎(PAN)患者的临床和病理特点,为早期诊断提供帮助。方法总结分析6例PAN患者的临床特点,作为首发症状的周围神经病的起病方式,进展过程,症状与体征的演变,以及电生理检查与腓肠神经组织病理学特点。同时总结6例患者的其他伴随症状,血液学检查,血管超声等检查手段的诊断意义。结果6例患者多数起病较急,下肢受累早,且症状比上肢重。感觉神经受累出现早,且刺激性症状比较突出。症状与体征不对称,尤其在病程早期更明显,后期可表现为远端对称或不对称性的多发性周围神经病。本组患者神经传导以波幅下降为主,感觉和运动神经均受累。3例患者行腓肠神经活检术,仅1例患者发现血管炎的典型病理改变。结论结节性多动脉炎以周围神经病为首发症状时诊断较困难。应详细询问病史,仔细查体。实验室检查如血沉快,C反应蛋白升高,血白细胞增多,血管彩超发现小动脉瘤或血管狭窄等具有提示意义。电生理检查可明确神经病变的部位和程度,神经和肌肉活检可证实诊断。     

周围神经病、共济失调、视网膜色素变性综合征的临床和病理学特点(附1例报告)

目的探讨表现为周围神经病、共济失调、视网膜色素变性综合征(NARP)的线粒体病的临床、病理及基因突变特点。方法回顾性分析一个线粒体病家系中表现为NARP综合征患者的临床、病理及基因检测资料。结果该患者首发症状为多饮多尿、记忆减退,客观检查发现腱反射消失和小脑体征。头颅MRI提示明显的小脑萎缩。EMG提示运动感觉神经传导速度减慢、诱发电位波幅降低。眼底荧光造影见到"胡椒与盐"样改变。肌肉活检Gomori染色可见破碎红纤维。全基因测序提示线粒体基因3243AG杂合突变。结论线粒体基因3243AG突变也可表现糖尿病和NARP综合征,临床上早期识别至关重要。     

动态MRI检测颈椎健康者、颈肩部不适者脊髓型颈椎病未确诊及确诊者的价值

背景：目前MRI对于颈椎病的检查多是常规的中立仰卧位静态平扫,是在非运动和非承重的条件下完成的,容易忽略脊髓在人体中承重和动态下脊髓的器质性改变。 目的：分析颈椎动态MRI结合临床表现早期诊断脊髓型颈椎病的临床意义。 方法：对随机抽取的正常人群10名(正常组)、有颈肩部不适的亚健康人群20名(亚健康组)、有脊髓型颈椎病症状体征但常规MRI不支持的患者20名(MRI正常脊髓型颈椎病组)、符合现行脊髓型颈椎病诊断标准的患者20名(脊髓型颈椎病组)进行JOA评分,颈椎正侧位片、颈椎中立位MRI及颈椎动态MRI检查,并随访18个月以上,观察4组研究对象各项观察指标的变化情况及4组人群的转归情况。 结果与结论：脊髓型颈椎病组20例患者均在入组半年内手术治疗;MRI正常脊髓型颈椎病组20例中有16例在入组16个月内行手术治疗,其中4例入组1年内在中立位MRI检查尚无明显改变时即行手术治疗,12例在入组1年后颈椎中立位MRI也已有脊髓型颈椎病的典型改变时行手术治疗,前者治疗效果明显优于后者(P < 0.05);亚健康组有4例出现了脊髓型颈椎病的症状体征,发现此4例患者动态颈椎MRI检查均有椎管变窄脊髓受压征象,但中立位MRI仅发现1例由此改变,且此例动态MRI在入组12个月时已经发现脊髓受压。在正常组随访中动态MRI与中立位MRI检查均发现1例有椎管变窄脊髓受压征象,但患者没有任何不适症状,考虑为假阳性;二者假阳性率相同。结果表明,颈椎动态MRI检查较中立位MRI能够更早期、更真实客观地发现脊髓受压征象;在临床症状、体征检查的基础上将常规的中立位MRI检查与动态MRI检查相结合将会更早期发现脊髓型颈椎病。     

POEMS综合征八例并文献复习

目的描述POEMS综合征的临床特点,以提高对此病的认识。方法报道8例POEMS综合征患者临床表现、实验室检查及治疗方法并复习有关文献。结果8例患者均有周围神经病变,其中4例患者血有M-蛋白,症状复杂多样。对8例患者进行不同尝试的治疗,结果症状有一定改善。结论POEMS综合征是一少见的多系统损害疾病,发病机制不很清楚,常有多发性神经病、脏器肿大、内分泌病、M-蛋白和皮肤改变症状。早期病例易被误诊,临床应认真分析、检查,以提高临床检出率。     

Lewis-Sumner 综合征6例报告与文献复习

目的探讨Lewis-Sumner综合征(Lewis-Sumner syndrome,LSS)的临床特点,随访其治疗反应及预后。方法回顾性收集6例确诊的Lewis-Sumner综合征患者的临床资料,包括临床特点、实验室检查、颈腰椎核磁、病理和神经电生理学检查及治疗,对其中4例进行电话随访评估疗效及预后。结果 6例患者均表现为慢性感觉运动性神经病,症状体征累及多条孤立的周围神经,而非弥漫性手套、袜套样分布,颅神经可以受累;脑脊液蛋白正常或轻中度增高;颈腰椎核磁可见神经根、神经丛增粗,增强扫描有强化;病理学检查呈脱髓鞘改变特点,电生理学检查的显著特征为持续存在的运动神经传导阻滞;激素治疗均有不同程度改善。结论 LSS表现为慢性感觉运动性多发性单神经病,随病情进展症状体征趋于弥漫性分布,但仍有显著不对称性的特点,激素治疗有效。     

慢性进展型神经白塞综合征临床特点分析

目的总结慢性进展型神经白塞综合征的临床特征。方法回顾分析2018年5月24日收治的1例慢性进展型神经白塞综合征患者的诊断与治疗经过;并以neuro Beh?et's syndrome、neuro Beh?et's disease、chronic progressive,以及白塞、神经白塞、慢性进展为中英文检索词,"AND"为检索策略,计算机检索1980年1月1日至2018年12月1日美国国立医学图书馆生物医学信息检索系统(PubMed)和中国知网中国知识基础设施工程(CNKI)、万方数据库中慢性进展型神经白塞综合征相关临床研究和病例报告,总结其社会人口学、临床表现、实验室和影像学特点,以及治疗原则及预后。结果共检索14篇国外文献计122例患者,结合本文病例,共纳入123例患者。分析结果显示,慢性进展型神经白塞综合征好发于中年男性,高峰发病年龄45～47岁,呈慢性病程;表现为共济失调、假性延髓麻痹和认知功能障碍;脑脊液白细胞介素-6水平升高为其特异性实验室指标,脑干萎缩是特征性影像学改变;治疗原则以糖皮质激素联合免疫抑制剂治疗为主,多预后不良。结论慢性进展型神经白塞综合征是白塞综合征的少见亚型,易误诊或漏诊,临床表现不典型,脑脊液和MRI检查对明确诊断具有重要提示意义,病残率和病死率较高。     

副肿瘤综合征

副肿瘤综合征是恶性肿瘤的破坏性远隔效应,它可影响神经系统的任何一个部位。多数神经系统副肿瘤综合征是免疫介导的,神经系统异常通常在肿瘤之前出现,症状和体征多种多样,取决于受损的部位。诊断主要依靠临床表现和相关辅助检查。近年来副肿瘤综合征患者血清或脑脊液中特异性抗神经元抗体的发现可作为肿瘤相关疾病的诊断标志,并为癌肿早期甚至超早期诊断提供了依据。治疗主要包括两个方面：一是针对神经系统副肿瘤综合征的免疫治疗;二是针对肿瘤的手术、放疗、化疗等。副肿瘤综合征的神经病理改变及肿瘤发现是否及时是影响预后的重要因素。     

神经系统白塞综合征的研究进展

白塞综合征(Behcet’s syndrome,BS)是一种少见的自身免疫病,神经白塞综合征(NBS)是兼有神经系统损害的白塞综合征,通常见于炎症性和血管性中枢神经系统疾病的鉴别诊断,NBS的临床和神经影像主要有两种变现形式:脑实质型NBS(p-NBS)和实质外脑静脉窦血栓形成(CVST)。最近研究发现,人类和小鼠神经纤维抗体显示NBS链球菌和结核分枝杆菌热休克蛋白(HSP)之间存在交叉反应,脊髓磁共振成像(MRI)有"百吉饼征"和"运动神经元"类型表现,英夫利昔单抗能有效预防复发及稳定残疾症状。原发性NBS通常累及脑干-间脑区部位,头痛、肌无力、共济失调和偏瘫是其主要临床特征。     

POEMS综合征18例临床分析及文献复习

目的分析POEMS综合征的临床特点,以提高对此病的认识。方法回顾性分析18例POEMS综合征患者临床表现、实验室检查及治疗方法并复习有关文献。结果18例患者均有周围神经病变,8例M-蛋白阳性,症状复杂多样。对14例患者进行不同尝试的治疗,部分患者症状有一定改善。结论POEMS综合征是一少见的多系统损害疾病,常有多发性神经病、脏器肿大、内分泌病、M-蛋白和皮肤改变等症状。临床应认真分析、检查,以提高临床检出率。     

脑静脉窦血栓形成的CT、MRI影像特点及文献回顾

目的探讨脑静脉窦血栓形成的CT、MRI影像特点,以便早期诊断、及时治疗、有效减少误诊误治. 方法回顾性分析5例脑静脉窦血栓患者的影像特征及相关文献. 结果5例患者均有不同程度的头痛、烦躁、神经系统定位体征,MRI表现为一侧或双侧顶叶近大脑镰处梗塞或夹杂有出血性改变. 结论头痛、烦躁、神经系统定位体征是脑静脉窦血栓的常见症状和体征.MRI的特征性表现为脑肿胀,顶叶的局部性梗死,多数伴有出血.     

脑型肝豆状核变性临床特征与MRI分析(附12例报告)

目的:探讨肝豆状核变性的临床特点与头颅MRI特征,为早期诊断及治疗提供参考。方法:回顾性归纳分析12例肝豆状核变性患者的临床表现及神经影像学特点。结果:平均发病年龄23.5岁;以锥体外系症状为首发10例;性格改变1例;学习成绩下降1例,K-F环均为阳性;头颅MRI特征为对称性基底节区、丘脑、中脑及桥脑异常信号。结论:肝豆状核变性临床表现多样,血清铜蓝蛋白检测、角膜K-F环及头颅MRI检查对诊断本病有重要意义。     

无症状脑梗塞MRI、MRA及TCD的临床对比研究

目的 探讨无症状脑梗塞(SCI)的临床特征与头颅MRI、MRA、TCD改变。方法 对50例病人进行临床分析，并进行头颅MRI、MRA和TCD检测，加以对比分析。结果 MRI检出梗塞灶的病人行MRA检查，异常检出率为93.02％，TCD异常检出率为94.87％。结论 MRI是确诊SCI重要条件之一，并能清晰、准确显示小脑、脑干梗塞；MRA能显示病变血管；TCD对SCI有早期辅助诊断价值，对治诊及预后有很好指导作用。     

Cerebral amyloid angiopathy manifested as a brain tumour. Clinical and neuropathological characteristics of two cases

We present two cases (female and male patients, aged 64 and 38, respectively) of focal mass lesions mimicking a brain tumour: one with cognitive function deficit, memory troubles, behavioral changes and left hemiparesis, the other with difficulty in orientation and right hemiparesis. General physical and neurological examinations, laboratory tests and neuroimaging were used to diagnose the cases. Both of them showed nonspecific changes in the brain tissue and the brain tumour was suspected. In the first case MRI scan revealed two pathological masses in the right frontal region and hemorrhagical focus with destructions inside lesions. Second patient's MRI scan revealed a pathological mass at the interface of the left temporal and occipital regions. The neurosurgical procedure was performed. The final diagnosis was established on the basis of neuropathological examination of postoperative material. On light microscopy examination a severe cerebral amyloid angiopathy (CAA) was revealed. Amyloidoma was excluded due to the absence of amorphous material and eosynophylic masses. Tumefactive CAA is a rare condition. These two cases of focal, tumefactive, masslike lesions of diffuse cerebral amyloid angiopathy are reported because of diagnostic dilemmas. In patients with history of memory disfunction, neurological deterioration and different multiple changes observed in CT and MRI scans, such as hemorrhagic infarcts and ischemic cerebral lesions, CAA should be suspected. The imaging findings make a distinction between tumefactive CAA and brain tumours like gliomas difficult. A differential diagnosis of CAA and amyloidoma plays a significant role in a neuropathological examination.     

Decreased Cerebral Blood Flow in Neuro-Behcet''s Syndrome Patients With Neuropsychiatric Manifestations and Normal Magnetic Resonance Imaging—A Preliminary Report

Involvement of the brain is one of the most important complications of Behcet's disease, but its diagnosis is difficult because of the lack of effective imaging tools. Therefore, technetium‐99m ethyl cysteinate dimer (Tc‐99m ECD) single‐photon emission computed tomography (SPECT) of the brain was used to detect abnormal regional cerebral blood flow in patients with neuro‐Behcet's syndrome (NBS). Tc‐99m ECD brain SPECT was per‐formed to detect hypoperfusion areas of the brain in 10 NBS patients with definite neuropsychiatric symptoms or signs and normal brain magnetic resonance imaging (MRI) findings. Tc‐99m ECD brain SPECT demonstrated hypoperfusion areas of the brain in all of the 10 NBS patients. The parietal lobes were the most common areas with hypoperfusion lesions. Tc‐99m ECD brain SPECT is a more sensitive and useful tool than brain MRI for detecting hypoperfusion areas of the brain in NBS patients.     

抗磷脂综合征7例患者临床、影像及病理特点分析

目的探讨抗磷脂综合征(antiphospholipid syndrome,APS)的临床表现、生化检查、神经影像和皮肤病理特点。方法回顾性分析7例诊断为APS患者的临床资料、神经影像和皮肤病理资料。结果慢性起病3例,临床表现为反复头痛、记忆力下降为主;神经影像学检查结果示病灶分布于大脑皮质下白质及侧脑室旁白质,呈脑梗死或脱髓鞘改变,颅内中等血管狭窄、小血管闭塞或继发扩张;皮肤活检病理检查:光镜示真皮内小血管周围少量淋巴细胞浸润,电镜示上皮组织下小血管管壁增厚、管腔狭窄或闭塞。结论 APS以反复头痛或脑梗死为主要临床表现,影像学以多灶性、不对称性、脑白质缺血或脱髓鞘信号改变为特点,皮肤病理检查以小血管狭窄或闭塞为特点。熟悉APS的临床、影像学与病理学特点有利于对其做出早期诊断。     

急性氟乙酰胺中毒性脑病6例临床及影像学分析

目的探讨氟乙酰胺中毒性脑病的神经系统临床表现及影像学特点,以提高对本病认识。方法回顾性分析2006年3月~2012年10月在中国医科大学附属盛京医院急诊科诊断氟乙酰胺中毒性脑病患者6例的临床表现及颅脑磁共振(MRI)特点。结果男4例、女2例口服氟乙酰胺后出现头痛、抽搐及其他神经系统表现。颅脑磁共振表现为皮层及皮层下白质弥漫性对称分布的细胞毒性水肿性病变。经过治疗6例患者临床症状部分或全部缓解。6例患者在6~10 d后复查头颅CT(2例)或MRI(4例)显示病灶完全消失。结论本病诊断主要依靠病史及典型临床表现,影像学缺乏特异性,但头颅MRI表现能对早期诊断提供参考。早期诊断及治疗患者预后良好。     

脑深静脉血栓形成两例临床和病理报告

目的：分析脑深静脉血栓形成（DCVT）的临床和病理物点。方法：分析2例患者的临床床及尸检资料,结果：本病临床常表现呼精神障碍。CT、CT血管造影（CTA）、MRI和磁共振血管造影（MRA）等使早期临床诊断成为可能。早期诊断抗凝、溶栓治疗使本病预后趋于良好。DCVT往往累及整个深静脉表现各异,首发症状多为头痛、意识和精神障碍,CT检查显示双侧基底节或丘脑梗死;CTA或MRI（MRA）检查能够辅助临床诊断,早期抗凝和溶栓治疗使预后趋于良好。     

Contribution of routine brain MRI to the differential diagnosis of parkinsonism: a 3-year prospective follow-up study

Various signs on routine brain MRI can help differentiate between Parkinson's disease (PD) and the various forms of atypical parkinsonism (AP). Here, we evaluate what routine brain MRI contributes to the clinical diagnosis, in both early and advanced disease stages. We performed a prospective observational study in 113 patients with parkinsonism, but without definite diagnosis upon inclusion. At baseline, patients received a structured interview, comprehensive and standardized neurological assessment, and brain MRI. The silver standard diagnosis was made after 3 years of follow-up (PD n = 43, AP n = 57), which was based on disease progression, repeat standardized neurological examination and response to treatment. The clinical diagnosis was classified as having either 'low certainty' (lower than 80%) or 'high certainty' (80% or higher). The added diagnostic yield of baseline MRI results were then studied relative to clinical neurological evaluation at presentation, and at follow-up. Sensitivity and specificity for separating AP from PD were calculated for all potentially distinguishing MRI abnormalities described previously in the literature. MRI abnormalities showed moderate to high specificity but limited sensitivity for the diagnosis of AP. These MRI abnormalities contributed little over and above the clinically based diagnosis, except when the clinical diagnosis was uncertain. For these patients, presence of putaminal or cerebellar atrophy was particularly indicative of AP. Routine brain MRI has limited added value for differentiating between PD and AP when clinical certainty is already high, but has some diagnostic value when the clinical diagnosis is still uncertain.     

Clinical and neuroradiological follow-up in mucopolysaccharidosis type III (Sanfilippo syndrome)

Mucopolysaccharidosis type III (Sanfilippo syndrome) is an autosomal recessive disorder characterised by progressive nervous system involvement with mental retardation, behavioural problems and seizures. Three patients, of 20 months to 12 years of age, were followed up for 3 years both clinically and by using brain magnetic resonance imaging (MRI). Our results suggest that in MPS III patients MRI findings, including atrophy and abnormal or delayed myelination, may precede the onset of overt neurological symptoms. The increasing neurological morbidity is accompanied by different degrees of progressive atrophic changes, mainly affecting the cerebral cortex and the corpus callosum. However, it appears that, across subjects, the rate of MRI changes is unrelated to the severity of the clinical phenotype. On this basis it could be argued that in MPS III the worsening of the neurological symptoms might not necessarily reflect only the progressive cerebral abnormalities detectable by MRI.