中枢神经系统受累的儿童噬血细胞性淋巴组织增生症临床分析

目的分析儿童噬血细胞性淋巴组织增生症(hemophagocytic lymphohistiocytosis, HLH)患儿中枢神经系统受累的临床特征和预后。方法回顾性分析13例出现中枢神经系统受累的HLH患儿的临床及影像学资料,并应用Kaplan-Meier法分析5年生存率。结果 13例HLH患儿中癫痫发作7例,意识障碍3例,面神经麻痹1例,烦躁1例,吞咽困难和构音障碍1例;1例有恶性血液病家族史;3例MUNC13-4基因突变,1例编码perforin-1基因突变;4例感染EB病毒;13例患儿均行脑CT和MRI检查,7例影像学表现正常,6例异常;7例癫痫发作患者中2例服用抗癫痫药物治疗,5例未服用抗癫痫药物情况下癫痫停止发作;13例患儿死亡4例,5年生存率为69%。结论中枢神经系统受累的HLH患者死亡风险较高,脑CT/MRI影像检查可表现异常,对提高HLH诊断有一定价值。     

8例髓鞘少突胶质细胞糖蛋白抗体相关疾病患者的临床和影像学表现分析并文献复习

目的：分析髓鞘少突胶质细胞糖蛋白抗体相关疾病(anti-myelin oligodendrocyte glycoprotein-IgG associated disorders, MOGAD)患者的临床和影像学表现及预后。方法：对我院2018年1月至2021年12月确诊的8例MOGAD患者的发病诱因、起病形式、临床表现及辅助检查进行分析,观察患者治疗效果及预后,并结合相关文献探讨。结果：8例MOGAD患者中,男性6例,女性2例,平均年龄为27.37岁,均为亚急性、慢性起病,临床表现以肢体无力、肢体抽搐、视物模糊常见。8例患者从出现症状到确诊的时间为2周至4年不等,首诊原因包括脊髓炎、脑炎、多发性硬化、颅内占位性病变。误诊原因与患者临床表现、影像学表现不典型,临床医师对MOGAD认识不足有关。脊髓穿刺检查示,脑脊液压力为95～225 mmH₂O,其中脑脊液压力增高3例,脑脊液中细胞数增高5例,脑脊液蛋白增高4例,而脑脊液中糖、氯化物均正常;血液、脑脊液MOG抗体阳性患者6例;脑诱发电位异常5例,脑电图异常4例;头颅MRI示受累范围较广,包括视神经、脑桥臂、丘脑、侧脑...     

噬血细胞综合征患者桥脑病变MRI表现及病因探讨

目的：探讨噬血细胞综合征(HLH)患者桥脑病变MRI特征及病因。资料与方法：回顾性分析2019年6月—2022年6月北京友谊医院18例HLH患者桥脑病变的MRI特征及临床资料，MRI分析包括病变位置、平扫及增强信号特征、是否有桥脑外病灶、复查变化，并与临床症状、血液生化结果、脑脊液生化结果进行对照分析。结果：18例患者中16例无中枢神经系统症状；15例(83.3%)病变检出前曾发生电解质紊乱(低钠或低钾)，14例行脑脊液检查者3例(21.4%)合并脑脊液生化异常；MRI表现为桥脑中央部病灶，T₁WI低信号，T₂WI及FLAIR高信号，12例(66.7%)表现为DWI高信号但ADC值不低，3例(16.7%)表现为DWI高信号且ADC值减低，6例(33.3%)检出桥脑外病灶；电解质紊乱纠正及鞘内注射化疗药物后，桥脑病变变化不一，与临床预后无确切关联。结论：当HLH患者发生桥脑中央病变时，可能的原因为桥脑中央髓鞘溶解(CPM)或HLH中枢神经系统(CNS-HLH)受累，临床上需关注电解质变化情况，警惕CPM的发生及进展，同时结合脑脊液生化检查判断有...     

散发型克雅病4例的临床、脑电图及影像学分析

目的:探讨散发型克雅病(sCJD)的临床、脑电图(EEG)及影像学特点。方法:回顾性分析4例sCJD患者的临床表现、EEG、影像学特点。结果:4例患者均有认知功能障碍,其中2例伴有锥体束损害,2例伴有锥体外系损害,1例伴有典型肌阵挛及癫痫发作。3例行脑脊液14-3-3蛋白检测均为阳性。EEG检查均有弥漫性异常,其中1例行动态脑电图(A-EEG)检查示典型的周期性三相波发放。3例头颅MRI检查,T2加权序列(T2WI)、液体衰减反转恢复序列(FLAIR)及弥散加权成像(DWI)在皮质、基底节区等发现异常高信号。结论:临床上对迅速进展型痴呆,并伴多系统受累等症状的疑似CJD患者,尽早行EEG、头颅MRI以及脑脊液14-3-3蛋白的检测有助于临床早期诊断。     

原发性低颅压综合征13例临床分析

目的 探讨原发性低颅压综合征的临床表现、脑脊液和影像学特点.方法 回顾分析13例临床诊断明确的原发性低颅压综合征患者的临床表现、脑脊液及影像资料.结果 所有患者均有体位性头痛,并可伴有恶心、呕吐、头晕、复视及颈强直;CSF压力均<60 mm H2O,蛋白升高5例、白细胞增多8例、红细胞增多9例;均行头CT检查,4例分别出现硬膜下积液和脑室变小;2例头颅MRI增强检查示弥漫性硬脑膜强化;均采用保守治疗,均痊愈.结论 体位性头痛是原发性低颅压综合征最典型的临床症状,硬脑膜弥漫性强化是最常见的影像学表现,CSF容量减少是原发性低颅压综合征临床及影像学表现的病理生理基础.     

成人轻度脑炎或脑病伴可逆性胼胝体压部病变6例临床分析

目的总结成人轻度脑炎或脑病伴可逆性胼胝体压部病变(mild encephalitis/encephalopathy with a reversible splenial lesion,MERS)患者的临床特点。方法回顾性分析6例MERS患者的临床资料,总结MERS的临床特征。结果 6例MERS患者中,5例以精神行为异常为首发症状,5例伴发热,出现反应迟钝、记忆力下降、一过性意识障碍、谵妄各2例,合并头痛、嗜睡各1例;3例存在定向障碍,2例脑膜刺激征阳性;脑脊液检查均无明显异常;头颅MRI均可见胼胝体压部异常信号;经治疗后,1个月内患者症状及影像学改变消失。结论当患者有脑炎或脑病症状且头颅MRI示胼胝体压部有异常信号时,需高度警惕MERS,定期复查头颅MRI有助于MERS的诊断;治疗后颅内病变可逆者预后良好。     

系统性红斑狼疮的中枢神经系统表现特征

目的 :分析系统性红斑狼疮(systemic lupus erythematosus,SLE)患者的中枢神经系统病变特征,以期早期识别这些特征,为指导诊治、改善患者预后提供帮助。方法:收集1996年至2010年,上海交通大学医学院附属瑞金医院风湿科及神经科收治的共53例中枢神经系统狼疮患者的临床资料,进行临床症状和体征、实验室检查、神经影像学检查、治疗和预后的总结和相关分析。结果:SLE患者的系统性症状以皮肤黏膜病变最为常见,而中枢神经系统症状则以头痛和癫痫间最为常见,此外脊髓也较常受累(15%),且多为长节段性病变,可能合并视神经脊髓炎。SLE伴缺血性脑卒中的发病与抗心磷脂抗体并不相关。多数SLE患者有血清学指标的异常,但这些指标的检测结果为阴性亦不能排除中枢神经系统病变的可能。患者脑脊液多有异常改变,但与其疗效及预后并不相关。患者头颅和脊髓的MRI表现多异常(60%),部分可表现为缺血灶、非特异性白质改变、钙化或长节段的脊髓病灶。约79%的SLE患者中枢神经系统症状经糖皮质激素、免疫抑制剂和(或)免疫球蛋白等治疗后得到改善。讨论:除头痛和癫痫间外,脊髓症状在中枢神经系统狼疮患者中较常见,实验室和脑脊液检查多异常,但结果正常亦不能排除中枢神经系统狼疮可能。中枢神经系统狼疮患者的神经影像学变化多样。     

伴进行性失聪及失明脑膜癌1例

<正>脑膜癌病(MC)是由中枢神经系统的原发肿瘤或其他系统的肿瘤经血液淋巴或脑脊液转移而发生的脑膜病变,该病病程进展快,平均生存期短,临床表现与其他神经系统疾病有类似的地方,且影像学诊断困难,极易误诊。本研究通过回顾1例伴进行性失聪及失明脑膜癌患者的临床表现和实验室检查,探讨脑脊液脱落细胞学检查在脑膜癌疾病诊断中的应用价值。     

成人噬血细胞综合征基因型特点及家系调查

目的对存在噬血细胞综合征(HLH)相关基因缺陷的成人患者进行基因特点的分析和家系调查,为研究HLH的发病机制进一步提供基础。方法以2016年1月至2018年12月北京HLH协作组在册登记的272例成人HLH患者为研究对象,对存在HLH相关基因缺陷的成人患者及其家属成员的基因特点、细胞毒功能进行检测和分析。结果成人原发性HLH的遗传学特点表现为错义突变和不典型位点突变。具有纯合突变或复杂杂合突变的成人原发性HLH患者NK细胞活性较存在单等位基因SNP的患者略低,但携带单等位基因SNP的健康家属成员的NK细胞活性明显高于原发性HLH患者以及HLH发病的单等位基因杂合突变患者。部分患者尽管在HLH相关致病基因上存在突变,但在缺少强烈外界刺激条件的情况下可以不出现HLH的临床表现。结论成人原发性HLH患者的突变位点、突变方式多见于错义突变和不典型位点,残存的细胞毒功能可能在一定程度维持基本的机体免疫功能,当外界刺激因素达到一定强度后导致HLH的临床表现。     

13例神经白塞病患者的临床及影像学特点分析

目的:探讨13例神经白塞病患者的临床表现及影像学特点。方法:收集符合神经白塞病诊断标准的13例住院患者的临床及影像学资料进行回顾性分析,总结其特点。结果:13例患者中头痛5例,肢体偏瘫5例,头晕4例,视物模糊3例、合并癫痫发作3例;血液学检查示血沉、CRP等炎性指标升高,脑脊液检查示脑脊液压力、白细胞计数、蛋白升高;头颅MRI可见双侧额叶、颞叶、基底节、放射冠区可见多发斑点状或斑片状长T1、长T2信号影,FLAIR及DWI呈高信号,脑室脑池稍大,脑沟增宽,颅内血管检查可见非动脉粥样硬化性狭窄甚至闭塞。结论:临床上疑似白塞病的患者,若同时伴有神经系统损害,需高度怀疑神经白塞病,需进一步行相关血液学检查、头颅MRI平扫及增强、脑脊液、针刺试验等检查以确诊。     

Determination of creatine kinase isoenzyme MB activity in serum using immunological inhibition of creatine kinase M subunit activity. Activity kinetics and diagnostic significance in myocardial infarction.

This is a new method for the determination of creatine kinase isoenzyme MB activity in serum. The method uses direct activity measurement of creatine kinase B subunit activity after blocking of CK-M subunit activity by inhibiting antibodies. The test takes no longer than 15 min. The method yields an intra-serial C.V. of 2.0-12.9%, and a C.V. from day to day of 5.5%. The detection limit is 3.4 U/l creatine kinase MB. In the 95 cases with proven myocardial infarction several types of creatine kinase MB activity kinetics could be determined. The percentage of creatine kinase MB of peak CK-total is 6-25%, with a mean of 11.1%. The amount of creatine kinase MB with respect to total CK activity after reinfarction is higher than the amount after initial infarction.     

Dissociable correlates of two classes of retrieval processing in prefrontal cortex

Although substantial evidence suggests that the prefrontal cortex (PFC) implements processes that are critical for accurate episodic memory judgments, the specific roles of different PFC subregions remain unclear. Here, we used event-related functional magnetic resonance imaging to distinguish between prefrontal activity related to operations that (1) influence processing of retrieval cues based on current task demands, or (2) are involved in monitoring the outputs of retrieval. Fourteen participants studied auditory words spoken by a male or female speaker and completed memory tests in which the stimuli were unstudied foil words and studied words spoken by either the same speaker at study, or the alternate speaker. On "general" test trials, participants were to determine whether each word was studied, regardless of the voice of the speaker, whereas on "specific" test trials, participants were to additionally distinguish between studied words that were spoken in the same voice or a different voice at study. Thus, on specific test trials, participants were explicitly required to attend to voice information in order to evaluate each test item. Anterior (right BA 10), dorsolateral prefrontal (right BA 46), and inferior frontal (bilateral BA 47/12) regions were more active during specific than during general trials. Activation in anterior and dorsolateral PFC was enhanced during specific test trials even in response to unstudied items, suggesting that activation in these regions was related to the differential processing of retrieval cues in the two tasks. In contrast, differences between specific and general test trials in inferior frontal regions (bilateral BA 47/12) were seen only for studied items, suggesting a role for these regions in post-retrieval monitoring processes. Results from this study are consistent with the idea that different PFC subregions implement distinct, but complementary processes that collectively support accurate episodic memory judgments.     

俯卧位通气对高海拔地区肺复张治疗无效急性呼吸窘迫综合征患者氧合的影响

目的 探讨俯卧位通气对高海拔地区肺复张术(RM)治疗无效急性呼吸窘迫综合征(ARDS)患者的治疗作用.方法 从海拔2260m的地区医院筛选RM治疗无效的41例ARDS患者[平均氧合指数( PaO2/FiO2)较RM前升高＜20％视为RM无效],依不同病因分为肺内源性ARDS组(ARDSp组)和肺外源性ARDS组(ARDSexp组),每组再按信封法随机分为俯卧位组和仰卧位组,即ARDSp俯卧位组(11例)、ARDSp仰卧位组(9例)、ARDSexp俯卧位组(10例)、ARDSexp仰卧位组(11例).在通气前及通气1、2、3、4h监测动脉血氧分压( PaO2)、PaO2/FiO2、静态顺应性(Cst)、气道阻力(Raw)的变化.结果 通气lh时,ARDSexp俯卧位组PaO2/FiO2( mm Hg,l mm Hg=0.133 kPa)即较通气前显著升高(157.4±40.6比129.3±48.7,P＜0.05),并随通气时间延长呈持续增高趋势,4h达峰值(219.1 ±41.1);且ARDSexp俯卧位组通气3h内PaO2/FiO2较其他3组显著增高,另3组间则差异无统计学意义.ARDSp俯卧位组、ARDSexp俯卧位组通气4h时PaO2/FiO2均较相应仰卧位组显著增高(208.8±39.7比127.4±47.1,219.1±41.1比124.9±50.8,均P＜0.05).4组通气前后Cst无显著改变,各组间差异也无统计学意义.ARDSp俯卧位组通气4h时Raw(cmH2O·L-1·s-1)较通气前显著降低(6.8±1.7比10.7±1.8,P＜0.05),且明显低于其他3组;其他3组各时间点Raw组内及组间比较差异均无统计学意义.结论 俯卧位通气作为ARDS机械通气重要策略之一,可以改善RM无效高原ARDS患者的氧合,为抢救患者赢得宝贵的时间.     

Digital imaging among medical facilities

The Department of Veterans Affairs (VA) in the USA operates a network of 172 medical centres which all utilize a hospital information system (HIS) which has been developed and is currently maintained by the VA. During the past several years, an image management and communication module has been developed, installed and clinically utilized at the Washington DC and Maryland VA Medical Centres. This image management and communication system, referred to as the decentralized hospital computer program (DHCP) imaging system, is fully integrated with a commercial picture archiving and communication system (PACS). The system is utilized to capture, archive, and display all images generated within the hospital including radiology, nuclear medicine, pathology, endoscopy, bronchoscopy, and dermatology, intraoperative photographs, ECG data, and a limited number of paper documents. The ultimate goal of the project is to have all patient text and image data available at any clinical workstation to any authorized user anywhere within the network of medical centres. Clinical requirements for an imaging workstation include ease of use, rapid and reliable access to the complete set of patient information, and images which are of acceptable quality to meet the requirements of the user and the subspecialty. Patient confidentiality and data security must be safeguarded at all times. Integration of the images with the remainder of the patient's database was found to be critical to the success of the project. The experience at the Washington and Maryland facilities suggests that an imaging system that is successfully integrated with a hospital information system can provide substantial clinical and economic benefits both within and among medical centres. Clinical acceptance and utilization of the system has been excellent, particularly in diagnostic radiology where DHCP Imaging has been interfaced to a commercial PAC system. Based upon this initial experience, the VA has begun to deploy the system throughout its large network of medical centres.     

Myocardial elastography at both high temporal and spatial resolution for the detection of infarcts

Myocardial elastography is a novel method for noninvasively assessing regional myocardial function, with the advantages of high spatial and temporal resolution and high signal-to-noise ratio (SNR). In this paper, in-vivo experiments were performed in anesthetized normal and infarcted mice (one day after left anterior descending coronary artery [LAD] ligation) using a high-resolution (30 MHz) ultrasound system (Vevo 770, VisualSonics Inc., Toronto, ON, Canada). Radiofrequency (RF) signals of the left ventricle (LV) in longitudinal (long-axis) view and the associated electrocardiogram (ECG) were simultaneously acquired. Using a retrospective ECG gating technique, 2-D full field-of-view RF frames were acquired at an extremely high frame rate (8 kHz) that resulted in high-quality incremental displacement and strain estimation of the myocardium. The incremental results were further accumulated to obtain the cumulative displacements and strains. Two-dimensional and M-mode displacement images and strain images (elastograms), as well as displacement and strain profiles as a function of time, were compared between normal and infarcted mice. Incremental results clearly depicted cardiac events including LV contraction, LV relaxation and isovolumetric phases in both normal and infarcted mice, and also evidently indicated reduced motion and deformation in the infarcted myocardium. The elastograms indicated that the infarcted regions underwent thinning during systole rather than thickening, as in the normal case. The cumulative elastograms were found to have higher elastographic SNR (SNR(e)) than the incremental elastograms (e.g., 10.6 vs. 4.7 in a normal myocardium, and 6.0 vs. 2.4 in an infarcted myocardium). Finally, preliminary statistical results from nine normal (m = 9) and seven infarcted (n = 7) mice indicated the capability of the cumulative strain in differentiating infracted from normal myocardia. In conclusion, myocardial elastography could provide regional strain information at simultaneously high temporal (>/=0.125 ms) and spatial ( approximately 55 microm) resolution as well as high precision ( approximately 0.05 microm displacement). This technique was thus capable of accurately characterizing normal myocardial function throughout an entire cardiac cycle, at the same high resolution, and detecting and localizing myocardial infarction in vivo.     

Clinical Pharmacokinetics of Morphine

Morphine, the most widely used mu-opioid analgesic for acute and chronic pain, is the standard against which new analgesics are measured. A thorough understanding of the pharmacokinetics of morphine is required in order to safely and effectively use this analgesic in a wide variety of patients with different levels of organ function. A MEDLINE search was conducted to identify literature published between 1966 and January 2002 relevant to the pharmacokinetics of morphine. These publications were reviewed and the literature summarized regarding unique and clinically important elements of morphine disposition relative to its parenteral administration (including intravenous, intramuscular, subcutaneous, epidural and intrathecal administration), absorption profile (immediate release, controlled release, and sublingual/buccal, and rectal administration), distribution, and its metabolism/ excretion. Special populations, including infants, elderly, and those with renal/liver failure, have a unique morphine pharmacokinetic profile that must be taken into account in order to maximize analgesic efficacy and reduce the risk of adverse events.