Rhizomelic Bone Dysplasia with Club-like Femora (Case report and confirmation of a syndrome)

A newborn with rhizomelic bone dysplasia with club-like femora is reported. This is the fourth case Of this easily recognizable, recently reported, congenital bone disease. Maroteaux et al recently reported two patients with rhizomelic bone dysplasia and club-like femora as a distinctive new bone dysplasia. Their observation was confirmed by Gugliantini et al (2) who reported another case. This paper reports a fourth patient with this easily recognisable disorder.     

新生大鼠反复惊厥对NMDA受体表达的长期影响

目的：研究反复惊厥对大鼠脑内N-甲基-D-天门冬氨酸(NMDA)受体表达，以及成年期认知功能和惊厥阈的长期影响。方法：生后6d(用P6表示，下同)的Wistar大鼠随机分成两组，每组6只，惊厥组每日吸入三氟乙醚诱导惊厥发作1次，每次持续30min，连续6d；对照组同样操作但不吸入三氟乙醚。两组大鼠于P60-P65行Morris水迷宫实验，检测大鼠的学习记忆功能。于P75时给予大鼠腹腔注射戊四唑(PTZ)测定大鼠的惊厥阈。随即断头处死大鼠，分离大脑皮质和海马，匀浆提取细胞膜蛋白，应用免疫印记法测定NMDA受体亚基l(NRl)和NR2A-2D表达的变化。结果：从P6l至P65，两组大鼠寻找平台时间均逐渐缩短，惊厥组大鼠在P65的平均寻找平台时间较对照组显著延长。惊厥组大鼠注射PTZ后发生惊厥的潜伏期与对照组比较差异无显著性。在大脑皮层，惊厥组大鼠较对照组NRl和NR2B表达水平明显下调，在海马这两种亚基表达水平无明显改变，在大脑皮层和海马区，惊厥组较对照组NR2A表达水平明显下调，NR2C表达水平明显上调。两组在皮层和海马均无NR2D表达。结论；新生大鼠反复惊厥可导致远期认知障碍，同时伴有NMDA受体的数量和结构上的长期改变，NMDA受体表达的这种改变可能在发育期惊厥导致的脑长期认知功能损害中起重要作用。     

足月新生儿缺氧缺血性脑病的CT临床应用与预后评估

目的:提高对足月新生儿窒息缺氧缺血性脑病(简称HIE)头颅CT的临床应用。方法:时80例有明确围产期窒息史和临床症状的患儿进行头颅CT扫描,其中72例进行复查扫描。作治疗前后CT对照并分析预后。结果:80例均有按CT诊断分度标准的轻(33例、占41.2％)、中(28例、占35.0％)、重度(19例、占23.8％)。CT表现,CT诊断阳性率为100％。结论:头颅CT检查能早期、直观、清楚地对足月新生儿窒息反映出脑缺氧缺血性损害及程度和变化。对指导治疗。评估预后有重要意义。     

高血压脑病的MRI表现

目的 分析高血压脑病的MRI表现。资料与方法 搜集8例经临床诊断高血压脑病患者，行MR T1WI和T2WI检查，其中4例加行扩散加权成像（DWI），分析血压变化、MRI表现及ADC值变化。结果 发病时平均血压199．63／123．5mmHg（1mmHg=0．133kPa），平均升高25．73％／28．71％。MRI上病变主要呈TI稍低、T2稍高信号，以顶枕叶、小脑半球多见。DWI病变呈等及稍高信号，ADC值较正常平均升高58％。仅1个病灶DWI出现明显高信号，ADC值减低。结论 MRI有助于高血压脑病的诊断及鉴别诊断、预后及疗效观察，DWI是将其与急性脑梗死区分开的最佳影像学检查方法。     

An Autopsied Case of Interferon Encephalopathy

Abstract: A 78-year-old male with renal carcinoma was treated with a high dose infusion of interferon-alpha (IFN-alpha) for eight months. The patient had evidence of organlc brain syndrome such 88 : dysfunction of memory, slowing of behavior, and development of mental confusion that appeared eight months after the treatment. MRI at the time of mental confusion revealed difise white matter lesions. Neuropathologic findings were compatible to Binswanger's disease and Senile Dementia of Alzheimer Type (SDAT), Preexisting neurologic abnormalities including intracerebral arteriosclerosis and cerebral atrophy may increase susceptibility to unacceptably severe IFN neurotoxicity.     

围产期不同阶段的缺氧与新生儿缺氧缺血性脑病的关系173例分析

对1989年1月至1993年12月本院分娩的173例新生儿缺血脑病(HIE)及颅内出血(ICH)进行分析,并对其中156例进行头颅CT检查。HIE的发生率为活产儿的2.13％,病死率为7.51％,是同期活产儿围产期死亡率的14.44倍。按围产期不同阶段的缺氧将173例HIE患儿分为四组,探讨其与临床分度、预后的关系以及临床分度与CT分度关系。分析CT诊断HIE及ICH的价值,并对HIE及ICH的早期诊断和预防进行讨论。     

Cystic brain stem necrosis in a premature infant after prolonged bradycardia

Summary A case is described of symmetrical cavitating brain stem necrosis produced by cardiac arrest in a premature infant. Two months after birth this 25-week gestational age infant suffered a prolonged episode of bradycardia. She was resuscitated and then died 3 weeks later. The autopsy revealed striking bilateral cavitation of the brain stem tegmentum extending in a columnar fashion from the upper portion of the spinal cord to the hypothalamus. The findings in this case are identical to the brain stem injury experimentally produced by complete cardiac arrest in the rhesus monkey.     

Multimodal, Multidimensional Models of Mouse Brain

Summary:  Naturally occurring mutants and genetically manipulated strains of mice are widely used to model a variety of human diseases. Atlases are an invaluable aid in understanding the impact of such manipulations by providing a standard for comparison and to facilitate the integration of anatomic, genetic, and physiologic observations from multiple subjects and experiments. We have developed digital atlases of the C57BL/6J mouse brain (adult and neonate) as comprehensive frameworks for storing and accessing the myriad types of information about the mouse brain. Along with raw and annotated images, these contain database management systems and a set of tools for comparing information from different techniques and different animals. Each atlas establishes a canonical representation of the mouse brain and provides the tools for the manipulation and analysis of new data. We describe both these atlases and discuss how they may be put to use in organizing and analyzing data from mouse models of epilepsy.     

儿童缺氧缺血性脑病的影像学和脑电图检查结果的对照及意义

目的：观察影像学和脑电图对儿童缺氧缺血性脑病的应用对照及意义。方法：对65例缺氧缺血性患儿作影像学和脑电图检查。结果：脑电图正常6例（9．23％），轻度异常13例（22．03％），中度异常37例（61．03％），重度异常9例（16．94％），脑电图异常率为90．77％。脑电图异常表现为弥漫性慢波增多和局限性癎性放电。影像学异常44例，异常率为67．69％。结论：影像学和脑电图检查对儿童缺氧缺血性脑病的诊断及治疗有一定的帮助。     

依达拉奉防治缺血性脑损伤研究的新进展

近年来,依达拉奉脑保护作用的研究取得了很大进展,尤以对缺血性脑卒中和新生儿缺血缺氧性脑病的防治研究最为深入,而联合治疗策略也得到了广泛的重视.本文就近5年这些热点研究的新进展进行综述.