11例肝豆状核变性的磁共振影像学观察

１１例肝豆状核变性的磁共振影像学观察刘吉良，李义召，张凤芝，张守瑞自１９９０年以来，我们观察了１１例肝豆状核变性（ＨＬＤ）患者的磁共振（ＭＲＩ）影像学改变，目的是研究ＨＬＤ的ＭＲＩ表现与临床症状、病程进展、治疗效果之间的相互关系。并对ＨＬＤ脑病灶的Ｍ...     

多发性硬化三例标本MRI长T2信号与病理对照研究

采用磁共振成像（ＭＲＩ）与病理对照方法探讨多发性硬化（ＭＳ）ＭＲＩ异常长Ｔ２信号与病理改变的关系。对３例经１０％福尔马林固定的全脑标本（其中１例含脊髓）行自旋回波（ＳＥ）序列扫描，对照ＭＲＩ图像所示的长Ｔ２信号行标本大体及镜下观察。３例ＭＳ均显示有长Ｔ２信号，其病理基础除ＭＳ斑块外还可见坏死软化灶及空洞形成。斑块状长Ｔ２信号具有特征性的ＭＲＩ表现：直角脱髓鞘征，见于病程长者（例１）。坏死软化灶及空洞形成引起的非斑块状长Ｔ２信号形态分布不一，以病程短、起病急者为著（例２、３），后者脊髓受累程度亦严重。ＭＲＩ是诊断ＭＳ最有价值的影像学检查方法。ＭＳ长Ｔ２信号的病理基础除典型硬化斑外还有坏死软化灶及空洞形成。当临床拟诊为ＭＳ，而ＭＲＩ仅显示非斑块状长Ｔ２信号时，应警惕为急性发病的ＭＳ。     

肝豆状核变性离体培养细胞内金属硫蛋白代射的研究

测定了一般培养条件下以及经各种浓度铜孵育后肝豆状核变性（ＨＬＤ）患者，杂合子的离体培养皮肤成纤维细胞内金属硫蛋白（ＭＴ）含量的变化。发现，一般培养条件下，三组间ＭＴ含量无显著性差异，但经高浓度铜孵育后，患者ＭＴ的含量明显比另二组高。讨论了ＭＴ异常在ＨＬＤ发病机理中的重要作用。     

铜孵育对肝豆状核变性培养细胞内铜在不同分子量蛋白上分布的影响

研究不同浓度铜孵育后，肝豆状核变性（ＨＬＤ）患者和杂合子离体培养皮肤成纤维细胞内铜在不同分子量蛋白上分布的变化规律，提出了在ＨＬＤ患者细胞内，可能存在铜进入细胞后从大分子蛋白向金属硫蛋白（ＭＴ）转移的过程，并对ＭＴ异常在ＨＬＤ发病机理中的作用进行了讨论。     

铜孵育对肝豆状核变性培养细胞内铜在不同分子量蛋白上分布…

研究不同浓度铜孵育后，肝豆状核变性（ＨＬＤ）患者和杂合子离体培养皮肤成纤维细胞内铜在不同分子量蛋白上分布的变化规律，提出了在ＨＬＤ患者细胞内，可能存在铜进入细胞后从大分子蛋白向金属硫蛋白（ＭＴ）转移的过程，并对ＭＴ异常在ＨＬＤ发病机理中的作用进行了讨论。     

急性脊髓炎的MRI与临床

目的探讨急性脊髓炎的脊髓ＭＲＩ表现特点，以及ＭＲＩ是否有助于本病的诊断。方法对２１例急性脊髓炎患者进行了脊髓ＭＲＩ矢状面和横轴面扫描。结果本病以上胸段及下颈段受累多见，部分病例可无异常，大部分病例病变区范围大，ＭＲＩ显示的病变上界高于临床感觉障碍平面的上界，脊髓呈均匀一致肿胀，Ｔ２加权像呈斑片状或条索状高信号，增强扫描可无强化或轻度斑点状强化。结论认为ＭＲＩ对本病的诊断与鉴别诊断有其特殊的价值，是其它检查不能替代的。     

亚急性联合变性的磁共振表现

目的评价磁共振成像（ＭＲＩ）对亚急性联合变性（ＳＣＤ）的诊断价值。方法对临床和实验室检查确诊的７例ＳＣＤ患者进行ＭＲＩ检查。结果有３例异常，均位于胸段脊髓，２例Ｔ２加权为片状或条状高信号区，１例胸髓变细。结论ＭＲＩ对ＳＣＤ有一定的诊断价值。     

磁共振成像对肾上腺脑白质营养不良的诊断价值

报告４例肾上腺脑白质营养不良（ＡＬＤ）患者的磁共振成像（ＭＲＩ）表现。ＭＲＩ表现为双侧对称的白质异常信号，主要位于枕、顶、颞叶，视放射和胼胝体压部，Ｔ１加权为低信号，Ｔ２加权为高信号，有增强效应。多维成像和高对比强度使ＭＲＩ比ＣＴ有更强的敏感性，能明确病变的部位及其严重程度，故ＭＲＩ是诊断ＡＬＤ的有效手段。     

多发性硬化的MRI增强的临床意义研究

目的探讨在ＭＲＩ增强中多发性硬化（ＭＳ）病灶强化的机制与临床的关系。方法对２３例ＭＳ患者均作ＭＲＩ平扫与增强扫描,其中９例作ＭＲＩ平扫与增强复查,使用德国西门子公司Ｐ８０．２Ｔ永磁型ＭＲ成像仪,采用ＳＥ序列。造影剂使用ＧＤ－ＤＴＰＡ,剂量为０．１ｍｌ／ｋｇ。结果ＭＲＩ平扫,ＭＳ病灶呈长Ｔ１长Ｔ２的斑片状异常信号,静脉注入ＧＤ－ＤＴＰＡ后产生的强化。用以分析ＭＳ的ＭＲＩ强化特征与临床分型的关系。结论不同临床类型的ＭＳ患者,在ＭＲＩ平扫及增强上均有不同的表现,这可能与ＭＳ斑的形成有不同的病理机制有关     

32例肝豆状核变性患者的脑电图研究

对３２例肝豆状核变性（ＨＬＤ）患者的脑电图（ＥＥＧ）、颅脑ＣＴ、颅脑磁共振（ＭＲＩ）和血清铜、铜蓝蛋白、铜氧化酶、尿铜及肝功能进行了比较研究。结果表明，ＥＥＧ的异常率略高于ＣＴ、ＭＲＩ、血清铜、铜蓝蛋白、铜氧化酶的异常率，但无显著性差异；ＥＥＧ的异常率显著低于尿酮的异常率和显著高于ＳＧＰＴ的异常率，其间有显著性差异。这提示，ＨＬＤ患者的ＥＥＧ异常不但直接反映大脑功能状况，而且还可间接地反映患者体内     

Mechanisms of the suppression of the nociceptive reflex of the opening of the mouth during stimulation of the structures of the limbic brain

The comparative effectiveness of the inhibitory influence of tetanic stimulation of hypothalamus, amygdala and limbic cortex on EMG-response of m. digastricus evoked by electrical stimulation of tooth pulp nociceptive afferents was studied in cats anesthetized with a mixture of chloralose and nembutal. It was found that inhibition of the EMG-component of the jaw-opening reflex is most pronounced in case of stimulation of medial and lateral region of the hypothalamus, the inhibitory effect of central and medial nuclei of the amygdala is less pronounced and the effect of the limbic cortex is the weakest. It was shown that the mechanism of the antinociceptive effect of tetanic stimulation of the hypothalamus is not related to the concomitant increase of the blood pressure. After stabilization of the blood pressure the suppressive effect of the hypothalamus remains without changes, that points out to a direct, primary, not baro-afferent mechanism of the inhibition of the activity of nociceptive neurons of the trigeminal sensory nuclei. Noradrenaline, injected intravenously, induced a large increase of the blood pressure accompanied by a pronounced inhibition of the pain reflex. Angiotensin causes the same degree of blood pressure elevation without changes in the amplitude of the EMG-response of the pain reflex. Hypothalamic and noradrenergic mechanisms for control of pain sensitivity are discussed.     

药物治疗与合并认知行为治疗对强迫症疗效的比较

目的探讨认知行为心理治疗(CBT)在强迫症(OCD)患者各亚型治疗中的有效性和规律性。方法本研究为临床对照研究。符合入组标准的强迫症患者按患者自愿原则分为两组,治疗观察3、6、12个月。疗效评定分别运用Yale-Brown强迫量表,自拟的自评好转程度量表和临床疗效评定。结果认知行为心理治疗合并药物治疗组31例,临床有效率70.9%,其中治愈率1.8%。单纯药物治疗组24例,临床有效率33.3%。Yale-Brown强迫量表和自评量表得分在6个月和12个月两组有显著差异(P<0.05)。其中强迫症亚型(怕脏型、反复检查型和反复担心型)的疗效比较,怕脏型在治疗3个月末两组间自评量表评分有显著性差异(P<0.05);反复担心型在治疗6个月末两组间Yale-Brown强迫量表总分有显著性差异(P<0.05);反复检查型两组间无统计学差异。结论认知行为心理治疗合并药物治疗强迫症的疗效明显优于单纯药物治疗。强迫症的亚型在治疗中的有效性次序为:反复担心型>怕脏型>反复检查型。     

Characteristics of the sympathetic innervation of the nictitating membrane and of the vasculature of the nose and tongue of the cat

Summary Vasomotor responses from the nasal mucosa and tongue, and contractions of the nictitating membrane, were recorded on stimulation of the cervical sympathetic or internal carotid nerves.Preganglionic sympathetic nerve fibres which elicited a membrane response possessed a lower threshold than those which evoked nasal vasoconstriction, while the latter displayed a lower threshold than fibres which evoked tongue vasoconstriction. The sympathetic vasodilator fibres to the tongue, whose activity was revealed after-receptor blockade, had a similar threshold to the vasoconstrictor fibres.Membrane contraction, nasal vasoconstriction and occasionally tongue vasoconstriction could be evoked by stimulating the internal carotid nerve. The postganglionic fibres innervating the nasal mucosa had a similar threshold to those of the nictitating membrane, which may indicate that there are small myelinated fibres innervating the mucosa.The preganglionic compound nerve action potential had four major components, S₁–S₄. S₁, S₂ and usually S₃ fibres were associated with membrane contraction; S₂, S₃ and sometimes S₁ fibres were associated with nasal vasoconstriction; and S₃, usually S₂ and occasionally S₁ fibres were associated with vasoconstriction in the tongue. It is concluded that each of these three groups of nerve fibres, but not S₄ fibres, may include fibres associated functionally with the three effectors.There was a considerable difference between the relative amplitude of the responses of the three effectors elicited by stimulation of the cervical sympathetic nerve at frequencies between 0.2 and 2 Hz. Vasoconstrictor responses were relatively larger than membrane contractions suggesting differences in the mechanisms of neurotransmission at the neuroeffector junctions.     

Development of the projection from the nucleus of the brachium of the inferior colliculus to the superior colliculus in the ferret

Neurons in the deeper layers of the superior colliculus (SC) have spatially tuned receptive fields that are arranged to form a map of auditory space. The spatial tuning of these neurons emerges gradually in an experience-dependent manner after the onset of hearing, but the relative contributions of peripheral and central factors in this process of maturation are unknown. We have studied the postnatal development of the projection to the ferret SC from the nucleus of the brachium of the inferior colliculus (nBIC), its main source of auditory input, to determine whether the emergence of auditory map topography can be attributed to anatomical rewiring of this projection. The pattern of retrograde labeling produced by injections of fluorescent microspheres in the SC on postnatal day (P) 0 and just after the age of hearing onset (P29), showed that the nBIC-SC projection is topographically organized in the rostrocaudal axis, along which sound azimuth is represented, from birth. Injections of biotinylated dextran amine-fluorescein into the nBIC at different ages (P30, 60, and 90) labeled axons with numerous terminals and en passant boutons throughout the deeper layers of the SC. This labeling covered the entire mediolateral extent of the SC, but, in keeping with the pattern of retrograde labeling following microsphere injections in the SC, was more restricted rostrocaudally. No systematic changes were observed with age. The stability of the nBIC-SC projection over this period suggests that developmental changes in auditory spatial tuning involve other processes, rather than a gross refinement of the projection from the nBIC.     

A contribution to the study of the architecture of the autonomic nervous system of the digestive tract of the rat

Summary The distribution of aminergic and non-aminergic nerve fibres to the different constituents of the wall of the digestive tract in various regions is described. Aminergic fibres synapse with all nervous perikarya. Densely interlacing networks of nerve fibres are found in both layers of the tunica muscularis and in the lamina muscularis mucosae. A finely meshed plexus is observed in relation to the wall of the blood vessels in the wall of the gut. There are many fibres connecting the muscular and the vascular plexus. No nerve fibres have been observed in direct relation to the epithelium.The functional implications of these findings are discussed.