12 例多系统萎缩患者的临床特点和诊断分析

目的:研究12例多系统萎缩(multiple system atrophy MSA)患者的临床特点,分析诊断要点。方法:回顾性分析12例多系统萎缩病例,探讨其临床特点和早期诊断的要点。结果:本组病例均为慢性、隐袭性起病,男性居多,男:女=1.4:1。平均发病年龄49.2岁(35.0岁～72.0岁)。平均病程33个月(6个月～60个月)。MSA-P亚型4例,MSA-C亚型8例,以MSA-C亚型占优势。MSA-P亚型中以行动迟缓为主要首发表现(75%),主要体征分别为肌张力增高(100%)、直立性低血压(75%)、锥体束征(75%);MSA-C亚型以行走不稳为主要首发表现(62.5%),主要体征为共济失调(100%),直立性低血压、小脑性语言、锥体束征(分别为62.5%)。结论:多系统萎缩是一种散发性的神经系统变性疾病,成年起病,临床表现多样。对于早期单纯以帕金森综合征、小脑功能障碍或自主神经功能紊乱为主要症状的患者,特别是无家族史者,要注意甄别有无其他系统受累的症状或体征,同时结合影像学检查、左旋多巴类药物治疗反应性等临床特点,对于早期正确诊断MSA有一定帮助。     

不同影像方法对股骨头缺血坏死的诊断价值

目的:研究早期股骨头无菌性坏死(aseptic necrosis of the femoral head,ANFH)的不同影像方法(X-ray、CT、MRI)诊断价值。方法:34例双侧股骨头行X线、CT和MRI检查,分析其对早期ANFH的显示能力。结果:34例患者均存在早期骨坏死表现。X线发现Ⅰ-Ⅱ期ANFH 14例,诊断准确率41.18%;CT发现Ⅰ-Ⅱ期ANFH 19例,诊断准确率57.35%;MRI发现Ⅰ-Ⅱ期ANFH 27例,诊断准确率为77.94%。结论:X线平片难以发现ANFH的早期表现,MR结合螺旋CT扫描既可发现早期病变,又可进行分期,是研究早期ANFH的可靠手段。     

寰枕间隙侧方穿刺移植脐血单个核细胞治疗多系统萎缩的临床观察

目的观察寰枕间隙侧方穿刺移植脐血单个核细胞治疗多系统萎缩(MSA)的安全性及临床疗效。方法选取聊城市人民医院神经内科10例MSA患者进行寰枕间隙侧方穿刺移植脐血单个核细胞治疗,分别观察治疗后3个月、6个月及1年的UMSARS评分,并随访观察有无不良反应。治疗前后评分比较采用t检验。结果治疗后3个月的UMSARS partⅠ评分(18.0±6.02)、治疗后6个月的UMSARS partⅠ评分(12.6±4.43)和治疗后1年的UMSARS partⅠ评分(12.3±3.20)均明显低于治疗前(25.8±4.80),治疗后3个月的UMSARS partⅡ评分(27.3±8.46)、治疗后6个月的UMSARS partⅡ评分(23.2±7.70)和治疗后1年的UMSARS partⅡ评分(22.4±5.93)均明显低于治疗前(34.5±6.79),治疗后3个月、6个月、1年的UMSARS partⅠ、UMSARS partⅡ评分较治疗前比差异具有统计学意义(P0.01)。除1例患者术后第1天低热,余患者均无不良反应。结论寰枕间隙侧方穿刺注射脐血单个核细胞治疗MSA是相对安全并且有效的治疗方法。     

MR 脑图像海马自动分割法在AD 早期诊断中的应用研究

目的:研究磁共振(Magnetic resonance,MR)脑图像中海马的自动分割方法及海马的形态学分析方法,为阿尔茨海默病(Alzheimer’s disease,AD)的早期诊断提供依据。方法:对20例AD患者和60名正常对照者行MRI T1 WI 3D容积扫描,建立海马的三维主动表观模型,并以此模型对每个个体脑部磁共振图像上的海马进行自动识别和三维分割,分别建立正常对照组和AD组的海马统计形状模型,比较AD组与正常对照组间海马形状的差异性。结果:海马三维分割方法与手动分割方法在海马体积测量上无统计学差别(P>0.05);AD患者海马头部发生萎缩(P<0.05)。结论:基于主动表观模型的MR脑图像海马自动识别和三维分割法是准确可靠的;海马头部萎缩可作为AD诊断的依据之一。     

CT和MRI在宫颈癌分期诊断中的临床对比分析

目的:比较CT和MRI在宫颈癌分期诊断中的临床应用价值。方法:回顾性分析宫颈癌患者96例,上述患者均有完整临床资料及术前CT和MRI检查结果,根据术后病理检查结果,比较两种检查方法影像学诊断、侵犯宫颈旁组织、侵犯子宫体、侵犯阴道、淋巴结转移的术前检查结果与病理诊断的符合率。结果:两种检查方法在宫颈癌Ⅰb期、Ⅱa期和Ⅱb期诊断符合率比较差异具有高度统计学意义(P<0.01);宫颈癌Ⅲa期和Ⅲb期符合率比较,差异具有统计学意义(P<0.05);宫颈癌Ⅳ期符合率比较,差异无统计学意义(P>0.05);检查提示侵犯宫颈旁组织、侵犯子宫体与侵犯阴道与术后病理诊断符合率比较,差异具有统计学意义(P<0.05)。结论:对于宫颈癌患者,术前应积极采取MRI检查方式,可提高诊断符合率,有利于对患者术后治疗的开展和预后的判断。     

颅内静脉系统血栓形成6 例临床分析

目的:探讨颅内静脉系统血栓形成(CVT)的临床表现、影像学特征以及治疗方法对临床诊断的意义。方法:回顾性分析首都医科大学宣武医院收治的6例CVT患者的临床表现及影像学特征与治疗方法。结果:6例临床表现无特殊,4例经MRI+MRV确诊,3例DSA检查确诊,1例介入治疗,4例抗凝治疗,2例保守,无死亡患者。结论:静脉系统血栓形成临床症状缺乏特异性,临床遇到急性起病的头痛、呕吐,伴或不伴有局灶性神经功能缺损或癫痫发作、意识障碍的青中年人,应高度怀疑CVT。早期应用抗凝、溶栓等治疗方法,对改善预后具有较高的临床应用价值。     

脑发育性静脉畸形9例临床影像特征分析及文献复习

目的:研究脑发育性静脉畸形(Cerebral Developmental Venous Anomalies,CDVA)临床及影像学特征及复习CDVA文献。方法:回顾性收集了自2011年11月至2014年3月我科确诊的9例CDVA的病人,对其临床特征、影像学检查方法包括电子计算机断层扫描(Computed Tomography,CT)、核磁共振成像(Magnetic Resonance Imaging,MRI)、数字减影血管造影(Digital Subtraction Angiography,DSA)及特征进行分析并对相关文献进行复习。结果:(1)临床症状:9例病人的临床症状包括头晕4例(4/9)、头痛4例(4/9)、恶心不适2例(2/9)、站立不稳1例((1/9)、小脑出血史1例(1/9)、眼部症状行眼科检查偶然发现小脑CDVA1例(1/9);(2)病变部位:病变位于幕上4例(4/9);幕下5例(5/9);(3)影像学检查:9例病人中,6例行CT平扫或增强扫描(3例平扫,3例平扫+增强);4例行MRI(1例平扫,3例平扫+增强);3例行DSA检查;(4)影像学特点:CT增强及重建、MRI的T1WI增强、SWI、MRA及DSA静脉期像均可显示出髓静脉及其形成的特征性"海蛇头"征象和其引流静脉。结论:CT、MRI、DSA影像学方法均可用于CDVA的诊断,在临床实践中需根据需要优化选择联合应用。     

眼附属器MALT淋巴瘤临床病例分析

目的:探讨眼附属器MALT淋巴瘤的诊断、治疗、疗效及预后影响因素。方法:回顾性分析1997年1月至2008年1月68例病理确诊的ⅠE期眼附属器MALT淋巴瘤患者的临床表现、影像学结果、治疗经过、放疗疗效及预后情况。结果:本研究68例ⅠE期眼附属器MALT淋巴瘤中男性44例,女性24例,男女比例1.83:1。原发于眼眶40例,眼睑5例,泪腺11例,结膜12例。经过55月(28-135月)的随访,5年总生存率为91.3%,5年的局部控制率为97%。结论:眼附属器MALT淋巴瘤好发于老年男性,以侵及眼眶者居多,B超、CT、MRI仅可提供较好的定性、定位诊断,确诊仍需病理诊断。手术切除联合局部放射治疗对ⅠE期眼附属器MALT淋巴瘤有较好的疗效。眼附属器MALT淋巴瘤有转化为弥漫性大B细胞淋巴瘤的潜在危险,放射治疗后长期的随访仍然是必须的。放疗或化疗也可以作为成功的补救措施。     

朗格罕细胞组织细胞增生症颅内垂体影像表现及相关临床表现

目的:探讨朗格罕细胞组织细胞增生症累及垂体的MR表现及相关临床表现。方法:搜集了6例确诊为朗格罕细胞组织细胞增生症并垂体表现异常的患儿,男5例,女1例,年龄2～11岁,平均(6±3)岁,对其影像及临床表现进行回顾性分析。结果:临床患儿主要以头面部包块,多饮、多尿等就诊。头颅MR平扫(T1WI)表现6例患儿神经垂体高信号全部消失,垂体柄增粗5例,垂体柄著征1例,垂体饱满1例,其中3例治疗后复查垂体及垂体柄均有变化。结论:神经垂体高信号消失,垂体柄增粗或著征为朗格罕细胞组织细胞增生症累及垂体的头颅MR表现。累及垂体者临床几乎都有多饮、多尿表现。目前,MRI是诊断朗格罕细胞组织细胞增生症累及垂体的唯一可靠的影像学检查方法,并对治疗后病情随访有重要作用。     

肺部炎性假瘤的CT影像学表现及其鉴别诊断价值

目的:分析肺部炎性假瘤的高分辨率CT影像学表现极其鉴别诊断的价值。方法:收集2012年4月-2015年4月我院收治的59例经手术病理检查证实为肺部炎性假瘤的患者为病例组,并于同一时期随机选取80例周围型肺癌患者为对照组。比较两组患者的高分辨率CT影像学特征,并分析各种影像学特征与肺部炎性假瘤的关系。结果:病例组刀切征、桃尖征及延迟期持续强化的发生率高于对照组,分叶征的发生率低于对照组(均P0.05)。多因素Spearman的秩相关分析发现,刀切征、桃尖征及延迟期持续强化与肺部炎性假瘤诊断呈正相关关系(r=0.738,0.829,0.763,均P0.05),分叶征与肺部炎性假瘤的诊断呈负相关关系(r=-0.791,P0.05)。结论:CT影像学表现中的刀切征、分叶征、桃尖征及延迟期持续强化对肺部炎性假瘤具有较高的鉴别诊断价值,可作为临床辅助诊断肺部炎性假瘤的重要影像学资料。     

Differential diagnostic relevance of high resolution magnetic resonance in patients with possible multiple system atrophy (MSA) - A case report

Multiple system atrophy (MSA) is sporadic, progressive neurodegenerative disorder characterized clinically by autonomic dysfunction, Parkinsonism (MSA-P), and cerebellar ataxia (MSA-C) in any combination. Parkinsonism is present in the majority of patients (80%). Early in the course of the disease autonomic dysfunctions are present in approximately 40% of patients, while the domination of cerebellar symptoms is present in 20% of all patients. According to second consensus statement on diagnosis of MSA, to make the diagnosis of possible MSA, except Parkinsonism or a cerebellar syndrome, there must be one feature involving autonomic dysfunction plus one other additional that can include findings on history, clinical examination or changes in structural or functional imaging. We present a case of 60-year old male with Parkinsonism and cerebellar symptoms accompanied with signs of autonomic nervous system involvement. Level of autonomic dysfunction was not the level required for the diagnosis of probable MSA. On initially performed 1.5T MRI, the most prominent neurodegenerative feature of brain stem, cerebellum and basal ganglia was atrophy, however features like "hot-cross bun" sign, "slit-like" putaminal rim and middle cerebellar peduncle hyperintensities were detected only after MR imaging on higher resolution (3T) device. Our case points to the possibility that some typical structural changes that can help in diagnostic process may not be clearly visible on 1.5 T MRI devices. In such cases we suggest using 3T MRI device, if feasible, in order to demonstrate findings that may help in establishing the diagnosis of possible MSA.     

Hyperintense putaminal rim at 1.5 T: prevalence in normal subjects and distinguishing features from multiple system atrophy

ABSTRACT: BACKGROUND: Hyperintense putaminal rim (HPR) is an important magnetic resonance imaging (MRI) sign for multiple system atrophy (MSA). Recent studies have suggested that it can also be observed in normal subjects at 3 T. Whether it can be observed in normal subjects at 1.5 T is not known. This study aimed to determine whether HPR could be observed in normal subjects at 1.5 T; and if so, to establish its prevalence, the MRI characteristics, and the features which distinguish from HPR in MSA patients. METHODS: Axial T2-weighted images of 130 normal subjects were evaluated for the prevalence of HPR, its age and gender distribution, laterality, maximum dimension, association with hypointensity of nearby putamen, and presence of discontinuity. To distinguish from that observed in MSA, axial T2-weighted images of 6 MSA patients with predominant parkinsonism (MSA-P) and 15 MSA patients with predominant cerebellar symptoms (MSA-C) were also evaluated. The characteristics of HPR were compared between these patients and age-matched normal subjects. The mean diffusivity (MD) values of putamen were also compared. Fisher's exact test, t-test, and one way analysis of variance were used to determine significance at corrected p < 0.05. RESULTS: HPR was observed in 38.5% of normal subjects. Age and gender predilection and laterality were not observed. In most cases, it occupied the full length or anterior half of the lateral margin of putamen, and was continuous throughout its length. Maximum transverse dimension was 2 mm. There was no association with hypointensity of nearby putamen. However, in MSA-P, HPR was located predominantly at the posterolateral aspect of putamen, and associated with putaminal atrophy. Discontinuity of HPR was more frequently observed in MSA-P. On visual analysis, the characteristics of HPR were similar between MSA-C patients and normal subjects. Patients with MSA of either type had significantly higher MD values of putamen than normal subjects. CONCLUSIONS: HPR can be observed in 38.5% of normal subjects at 1.5 T. Thin linear hyperintensity without discontinuity, occupying the full length or anterior half of the lateral margin of the putamen, is suggestive of "normal." In doubtful cases, measurement of the MD values of nearby putamen may be valuable.     

Can prion disease suspicion be supported earlier?: Clinical,radiological and laboratory findings in a series of cases

The subacute spongiform encephalopathies are prion diseases characterized by acute and rapid neurodegeneration that lead to the death of the patient within months to a few years. The epidemiology of CJD is complicated and the frequency in Mexico is unknown. We aim to describe the cases of prion disease in Mexico. Consecutive patients who met the diagnostic criteria by the WHO were enrolled. We describe 26 patients with clinical manifestations, imaging and laboratory studies compatible with prion disease. The mean age at onset was 52 years old. The main clinical manifestations were cognitive alterations (69%) followed by extrapyramidal movements (50%), abnormal cerebellar function (46%), behavioral alterations (46%), myoclonus (46%) and mood depression (23%), among other features. Half of the patients progressed rapidly to a state of akinetic mutism (53%). Only 2 (7.6%) patients had a family history of a similar disease. Time interval between onset and diagnosis varied between 71 days to 24 months, with a median of 6 months. The classical bilateral basal ganglia hyperintensities were present in the very early stage of the disease. Protein 14-3-3 immuneassay in the CSF was positive in all measured cases. Bilateral basal ganglia hyperintensities was the most important early finding, while protein 14-3-3 was a late finding and the results were usually obtained after the patient was discharged. Around 1.5 cases of CJD cases per year are reported in our country. When suspected, MRI can support the diagnosis earlier than other studies.Key words: Creutzfeldt-Jacob disease, prions     

糖尿病酮症酸中毒合并吉兰-巴雷综合征2 例临床分析

目的:研究糖尿病酮症酸中毒(diabetic ketoacidosis,DKA)合并吉兰-巴雷综合征(Guillain-Barrésyndrome,GBS)的临床特点,以探讨其临床表现、治疗、预后及发病机制。方法:回顾性分析2例DKA合并GBS患者的病例资料,对其病史、临床表现、电生理学、脑脊液改变、治疗进行总结。结果:2例DKA患者均急性起病,两例患者发病前1周均有呼吸道感染史,尽管DKA得到了纠正,患者出现呼吸衰竭后被发现四肢力弱。肌电图提示周围神经损害。脑脊液示细胞蛋白分离。给予免疫球蛋白后,例1在出现肢体瘫痪后18天恢复至正常,遗留有四肢末端麻木感,例2在出现肢体瘫痪后1年肌力恢复正常。结论:DKA合并GBS临床少见,多发生在DKA纠正后一周左右出现四肢迟缓性瘫痪,早期给予免疫球蛋白治疗,预后相对良好,目前发病机制尚不清楚。     

CT和MRI在宫颈癌分期诊断中的临床对比分析

目的：比较CT和MRI在宫颈癌分期诊断中的临床应用价值。方法：回顾性分析宫颈癌患者96例,上述患者均有完整临床资料及术前CT和MRI检查结果,根据术后病理检查结果,比较两种检查方法影像学诊断、侵犯宫颈旁组织、侵犯子宫体、侵犯阴道、淋巴结转移的术前检查结果与病理诊断的符合率。结果：两种检查方法在宫颈癌Ⅰb期、Ⅱa期和Ⅱb期诊断符合率比较差异具有高度统计学意义（P〈0．01）;宫颈癌Ⅲa期和Ⅲb期符合率比较,差异具有统计学意义（P〈0．05）;宫颈癌Ⅳ期符合率比较,差异无统计学意义（P〉O．05）;检查提示侵犯宫颈旁组织、侵犯子宫体与侵犯阴道与术后病理诊断符合率比较,差异具有统计学意义（P〈0．05）。结论：对于宫颈癌患者,术前应积极采取MRI检查方式,可提高诊断符合率,有利于对患者术后治疗的开展和预后的判断。     

A Chinese patient of P102L Gerstmann-Sträussler-Scheinker disease contains three other disease-associated mutations in SYNE1

Gerstmann-Sträussler-Scheinker disease (GSS) with the P102L mutation in PRNP gene is characterized with progressive cerebellar dysfunction clinically and PrP^Sc plaques neurologically. Due to the cerebellar ataxia in the early stage, GSS P102L is often misdiagnosed as other neurodegenerative disorders. We presented here a 49-year-old female patient with proven P102L PRNP mutation, and three heterologous mutations in hereditary ataxias associated gene SYNE1, including p.V3643L, p.M3376V and p.T2860A. The patient appeared progressive unsteady gait in early stage and developed the Creutzfeldt-Jacob disease (CJD) – associated clinical manifestations, including progressive dementia, myoclonus, pyramidal and extrapyramidal signs. She is still alive but with akinetic mutism 21 months after onset. Observation of intense signal changes in cortical regions (cortical ribboning) in diffusion weighted imaging (DWI) MRI scanning and positive protein 14-3-3 in cerebrospinal fluid (CSF) proposed the diagnosis of sporadic CJD. The final diagnosis of P102L GSS was made after PRNP sequencing.     

Diagnostic Accuracy of Apparent Diffusion Coefficient and 123I-Metaiodobenzylguanidine for Differentiation of Multiple System Atrophy and Parkinson’s Disease

Background

It is often hard to differentiate Parkinson’s disease (PD) and parkinsonian variant of multiple system atrophy (MSA-P), especially in the early stages. Cardiac sympathetic denervation and putaminal rarefaction are specific findings for PD and MSA-P, respectively.

Purpose

We investigated diagnostic accuracy of putaminal apparent diffusion coefficient (ADC) test for MSA-P and ¹²³I-metaiodobenzylguanidine (MIBG) scintigram for PD, especially in early-stage patients.

Methods

The referral standard diagnosis of PD and MSA-P were the diagnostic criteria of the United Kingdom Parkinson’s Disease Society Brain Bank Criteria and the second consensus criteria, respectively. Based on the referral standard criteria, diagnostic accuracy [area under the receiver-operator characteristic curve (AUC), sensitivity and specificity] of the ADC and MIBG tests was estimated retrospectively. Diagnostic accuracy of these tests performed within 3 years of symptom onset was also investigated.

Results

ADC and MIBG tests were performed on 138 patients (20 MSA and 118 PD). AUC was 0.95 and 0.83 for the ADC and MIBG tests, respectively. Sensitivity and specificity were 85.0% and 89.0% for MSA-P diagnosis by ADC test and 67.0% and 80.0% for PD diagnosis by MIBG test. When these tests were restricted to patients with disease duration ≤3 years, the sensitivity and specificity were 75.0% and 91.4% for the ADC test (MSA-P diagnosis) and 47.7% and 92.3% for the MIBG test (PD diagnosis).

Conclusions

Both tests were useful in differentiating between PD and MSA-P, even in the early stages. In early-stage patients, elevated putaminal ADC was a diagnostic marker for MSA-P. Despite high specificity of the MIBG test, careful neurological history and examinations were required for PD diagnosis because of possible false-negative results.     

妊娠相关脑卒中39 例临床分析

目的:探讨妊娠相关脑卒中的发病原因、临床表现、母婴结局、治疗及预防措施。方法:回顾性分析2001年1月-2013年12月我院共收治的妊娠相关脑卒中39例患者的临床资料。结果:39例患者中,发生在妊娠期21例,产褥期18例。经电子计算机断层扫描(CT)、磁共振成像(MRI)、磁共振动脉血管造影(MRA)、磁共振脑静脉血管成像(MRV)、数字减影血管造影术(DSA)和腰椎穿刺等检查明确诊断,诊断出血性脑卒中7例,脑梗死3例,脑静脉窦血栓(CVST)29例,均给予相应的抢救及治疗。16例早期、中期妊娠患者行人工流产术或利凡诺羊膜腔内注射穿刺引产终止妊娠,5例患者行剖宫产终止妊娠。患者治愈出院15例,6例死亡,14例遗留不同程度的肢体活动障碍或语言障碍,家属放弃治疗出院4例。结论:妊娠相关脑卒中危险因素主要包括子痫前期、心源性栓塞、脑血管畸形、脑动脉瘤、水电解质紊乱等代谢障碍性疾病及产褥感染等。其发病急、病死率高,故需提高对本病的认识,定期产前检查,及时发现高危因素,早诊断及时治疗,选择适当的时机及方式终止妊娠是改善妊娠相关脑卒中患者预后的关键。