白塞病合并骨髓异常增生综合征的临床特点(附6例报告)

目的：为了解白塞病（ＢＤ）与骨髓增生异常的相关性。方法：回顾分析了６例ＢＤ合并骨髓异常增生综合征（ＭＤＳ）患者的临床资料、血象和骨髓象。２例合并难治性贫血（ＭＤＳＲＡ），２例合并难治性贫血伴原始细胞增多（ＭＤＳＲＡＥＢ），１例合并转化型难治性贫血伴原始细胞过多（ＭＤＳＲＡＥＢｔ），１例合并慢性粒—单细胞白血病（ＭＤＳＣＭＭＬ）。结果：６例均示有贫血，５例白细胞低下，４例血小板减少。６例骨髓均增生活跃。４例ＢＤ发病在前，２例ＢＤ与ＭＤＳ同时出现。结论：认为ＢＤ与ＭＤＳ有相关性     

帕金森病痴呆皮质下核病理研究（附七例临床病理分析）

报道了７例帕金森病痴呆患者的临床及病理资料，并与６例非帕金森病，非痴呆作对照。观察其脑黑质致密带，蓝斑核，前脑基底核的Ｍｅｙｎｅｒｔ核，海马的病理改变，并做了细胞计数及形态学检查。结果显示帕金森病痴呆患者皮质下三种神经细胞核明显减少及变性，与对照组比较差异均有显著性。７例中有５例含有Ｌｅｗｙ小体，１例在海马及皮质有Ａｌｚｈｅｉｍｅｒ病的改变；对照组海马改变非常轻微。提示皮质下痴呆与Ａｌｚｈｅｉｍ…     

帕金森病致病机制

帕金森病（ＰＤ）的致病机制研究不断深入，现概述如下。多巴胺与多巴胺受体：目前认为黑质纹状体通路的黑质腹外侧多巴胺（ＤＡ）能神经元退变是ＰＤ的基本病理过程，先累及神经末梢，继而缓慢发展损及胞体。ＤＡ能神经元集中位于中脑，依次为黑质致密部和外侧部（Ａ９）...     

^99mTc—TRODAT—1放射自显影对帕金森病大鼠模型多巴胺？…

目的 探讨＾９９ｍＴｃ－ＴＲＯＤＡＴ－１多巴胺转运蛋白（ＤＡＴ）显像临床应用价值。方法 应用６－羟基多巴胺（６－ＯＨＤＡ）建立完全损毁及部分损毁一侧帕金森病（ＰＤ）大鼠模型，以＾９９ｍＴｃ－ＴＲＯＤＡＴ－１作为配体，采用放射自显影观察一侧ＰＤ大鼠模型ＤＡＴ分布及其密度，高效流体相电化学方法鼠黑质及纹状体ＴＨ阳性细胞及纤维。结果 ６－ＯＨＤＡ损毁侧纹状体放射性浓集明显低于本损毁侧，完全损毁模型的纹状     

急性白血病合并DIC53例临床分析

对５３例ＡＬ合并ＤＩＣ作回顾分析，并发ＤＩＣ的ＡＬ类型为ＡＰＬ２８例，非Ｍ３型ＡＮＬＬ１５例、ＡＬＬ１０例，发生于初发期、诱导化疗和化疗间歇期、难治期的ＤＩＣ分别占２５、１１、１７例。结果显示，ＡＰＬ合并ＤＩＣ６８％发生于初发期、非Ｍ３期ＡＮＬＬ并ＤＩＣ发生于难治期占６０％，而ＡＬＬ并ＤＩＣ５０％与诱导化疗有关。４０例经全程治疗者，ＤＩＣ治愈８例，其中６例为初发期患者（６／１６）。讨论了ＤＩＣ发生     

急性白血病并发糖尿病11例临床分析

急性白血病（ＡＬ）并发糖尿病（ＤＭ）并不罕见，但国内报道不多，其发病机理尚不清。本文观察１１例，对其临床特征及其发病的有关因素和转归报道如下。１临床资料ＡＬ并发ＤＭ１１例中男６例，女５例。年龄为１７～７０岁，平均２９岁。ＡＬ诊断标准按ＦＡＢ协作组的Ａ...     

纹状体黑质变性及其与帕金森病的鉴别

１９６１年Ａｄａｍｓ剖检５０例临床诊断为震颤麻痹的病例，其中大部分病例见黑质及蓝斑神经细胞脱失、变性，并见有胞浆内的Ｌｅｗｙ小体。但有４例除黑质改变外，纹状体、特别是双壳核也见有明显的神经细胞脱失、变性，称之为纹状体黑质变性（ＳＮＤ）。至今国外见有散...     

西比灵治疗CO中毒后迟发性脑病35例

急性ＣＯ中毒后迟发性脑病（ＤＥＡＣＭＰ）系指急性ＣＯ中毒患者经过抢救意识恢复后，在数天至数周的“假愈期”后再度出现以急性痴呆、精神障碍和帕金森综合征等为主要表现的中枢神经系统损害。我们应用西比灵治疗ＤＥＡＣＭＰ３５例，疗效明显。报告如下。临床资料：Ｄ...     

帕金森病诊断与鉴别诊断

帕金森综合征（Ｐａｒｋｉｎｓｏｎｉｓｍ,ＰＤ综合征）包括原因不明的ＰＤ（原发性或特发性ＰＤ综合征）以及各种原因（感染、中毒、外伤、药物及遗传等）造成的继发性（症状性）ＰＤ综合证,包括以黑质纹状体多巴胺能神经元变性导致纹状体多巴胺（ＤＡ）含量下降为基础...     

CD44V6和CD44S表达与膀胱癌转移的相关性研究

采用免疫组化ＳＡＢＣ法对８１例膀胱移行细胞癌患者癌组织中细胞粘附分子ＣＤ４４ｖ６和ＣＤ４４ｓ的表达进行检测。结果显示，ＣＤ４４ｖ６的阳性表达与膀胱癌临床分期、病理分级有显著关系，伴淋巴结转移组的ＣＤ４４ｖ６阳性表达率显著低于未转移组。     

皮质基底节变性的临床和病理研究

目的：阐述皮质基底节变性的临床和病理特征。方法：报告2例皮质基底节变性的临床资料和病理检查结果。结果：2例病人早期表现为单侧肢体运动障碍如肌张力增高,跌倒发作,病程中出现四肢强直少动,伴肌阵挛和不同程度的皮层认知功能障碍;影像学表现为额顶叶不对称性萎缩或脑代谢减低。大体见普遍脑萎缩,但以额、顶叶皮层为著,镜下检查见皮层气球样神经元,基底节和黑质神经细胞变性脱失。Gallyas-Braak 银染色和Tan免疫组织化学染色显示基底节、黑质等神经元胞质内Tan阳性球形包涵体,额叶、顶叶皮层及基底节大量Tan阳性簇状星形胶质细胞和星形胶质细胞斑。结论：老年人慢性进展性不对称肢体运动障碍伴痴呆时,临床应用考虑到皮质基底节变性。如病理上发现特异性神经元及星形胶质细胞改变,则有助于本病的确诊。     

Explant cultures of catecholamine-containing neurons from rat brain: Biochemical, histofluorescence, and electron microscopic studies

Norepinephrine (NE)-producing cells of the nucleus locus ceruleus and dopamine (DA)-producing cells of the substantia nigra were dissected microscopically from embryonic rat brain, explanted, and maintained in culture for up to 5 weeks. The cultured neurons of both brain regions showed normal maturation of axons and dendrites and formed ultrastructurally defined synaptic contacts. Fluorescence microscopy of cultured neurons from both brain regions showed typical in situ cytological features: long axonal processes with multiple varicosities for locus ceruleus cultures, and smooth, wispy nonvaricose processes in the substantia nigra cultures. All cultures processed for fluorescence microscopy contained specific catecholamine-fluorescent cells. By radioenzyme assay for catecholamines, more than half of the locus ceruleus cultures contained measurable (>10 pg) quantities of NE and DA, but, unlike results on intact brains, DA content exceeded NE content. Cultures of substantia nigra neurons retained no NE and very little DA. Media from substantia nigra and locus ceruleus cultures contained substantial quantities of DA. Addition of reserpine (10 muM) to the medium depleted locus ceruleus neurons of both amines.The long survival time in culture of locus ceruleus cells, the normal appearance of fluorescent cell bodies and processes, the apparent development of morphologically specialized interneuronal connections, and the ability to synthesize and store NE make these cultures ideally suited for neurophysiological recording as well as morphological, biochemical, and pharmacological experiments.     

An autopsy case of Alzheimer's disease associated with Parkinson's disease, compared to 2 autopsy cases of diffuse Lewy body disease

A 68 year old male, diagnosed as Alzheimer's disease clinically, pathologically showed both findings of Alzheimer's disease and Parkinson's disease. The brain weight was 940 g. Macroscopically, severe cortical brain atrophy and depigmentation of the substantia nigra was noted. Microscopic examination showed marked appearance of senile plaque and a large number of neurofibrillary tangle with sever neuronal loss of the cerebral cortex. Additionally, the loss of neuron with many Lewy bodies was found in the substantia nigra. Lewy bodies were also found in the locus ceruleus and the dorsal vagal nucleus, but few in the cerebral cortical neurons. We compared this case neuropathologically with two autopsy cases of diffuse Lewy body disease (DLBD). There was no distinction concerning the lesions of the brain stem between this case and the cases of DLBD. In all three cases, the nucleus of basalis of Meynert showed marked neuronal loss. However, the brain was lighter than those of the cases of DLBD. Senile changes such as senile plaque and neurofibrillary tangles were more marked in this cases than in the cases of DLBD. Furthermore a large number of cortical Lewy bodies were found in the cases of DLBD, but few in this case. The distribution and number of Lewy bodies did not correspond with those of senile changes in the cases of DLBD. Also the cerebral cortical structure was better preserved in the cases of DLBD than in this case. In conclusion, from the clinicopathological findings, we considered that this case is Alzheimer's disease associated with Parkinson's disease. According to Kosaka's study, this case seemed to correspond with a transitional type of the Lewy body disease.     

An autopsy case of dementia with Lewy bodies who showed the typical parkinsonism in the initial five years

A 76-year-old man with parkinsonism and dementia was reported. He developed resting tremor at age 69 followed by hypokinesia, rigidity and small step gait. L-dopa ameliorated his symptoms with no hallucinations for the initial 5 years. His mental level did not decrease during that period. He was admitted to our hospital because of dehydration and fever at age 74. Subsequently, his cognitive function deteriorated, with visual hallucination. Serial brain CT studies displayed a progressive cerebral cortical atrophy without focal lesions. He died of respiratory distress syndrome and disseminated coagulopathy resulting from pneumonia, dehydration and syndrome malin. Postmortem examination revealed a marked bilateral loss of melanin-containing neurons with Lewy bodies in the substantia nigra and locus ceruleus. Lewy bodies were also in the basal nucleus of Meynert, with moderate neuronal cell loss. The distribution of Lewy bodies was widespread in the cerebral cortical areas, corresponding to the neocortical subtype according to the consensus guideline for the pathologic diagnosis of dementia with Lewy bodies. According to the criteria of the Consortium to Establish a Registry for Alzheimer's Disease, the age-related plaque score in the present case suggested Alzheimer's disease, although cortical neurofibrillary changes corresponded to stage II by the criteria of Braak and Braak. These pathological findings established the diagnosis of dementia with Lewy bodies from the quantitative and distributional viewpoints. Based on recent neuropathological evidence, a spectral theory, which presents idiopathic Alzheimer's disease and Parkinson's disease as the two extremes of a spectrum of neurodegeneration, has been proposed. Dementia with Lewy bodies is located in the middle of this spectrum. Pathological evaluation based on quantitative consensus guidelines is important to establish the diagnosis in patients with parkinsonism and dementia, since neuropathological changes of Alzheimer's disease, Parkinson's disease and dementia with Lewy bodies are often observed in a mixed manner in these patients.     

Loss of locus coeruleus neurons and reduced startle in parkin null mice

Parkinson's disease (PD) is the most common neurodegenerative movement disorder and is characterized pathologically by degeneration of catecholaminergic neurons of the substantia nigra pars compacta and locus coeruleus, among other regions. Autosomal-recessive juvenile Parkinsonism (ARJP) is caused by mutations in the PARK2 gene coding for parkin and constitutes the most common familial form of PD. The majority of ARJP-associated parkin mutations are thought to be loss of function-mutations; however, the pathogenesis of ARJP remains poorly understood. Here, we report the generation of parkin null mice by targeted deletion of parkin exon 7. These mice show a loss of catecholaminergic neurons in the locus coeruleus and an accompanying loss of norepinephrine in discrete regions of the central nervous system. Moreover, there is a dramatic reduction of the norepinephrine-dependent startle response. The nigrostriatal dopaminergic system does not show any impairment. This mouse model will help gain a better understanding of parkin function and the mechanisms underlying parkin-associated PD.     

Parkinsonism-inducing neurotoxin, N-methyl-4-phenyl-1,2,3,6 -tetrahydropyridine: characterization and localization of receptor binding sites in rat and human brain.

N-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) produces neuropathologic and clinical abnormalities in humans and animals that closely resemble idiopathic Parkinson disease. [3H]MPTP binds with high affinity (Kd = 28 X 10(-9) M) to brain membranes. The chemical specificity of the binding sites corresponds to structure-activity requirements for neurotoxicity. Autoradiographic studies in human brain show very high receptor densities in the caudate, substantia nigra, and locus coeruleus, which may explain the neurotoxic and neurochemical sequelae of MPTP administration. In contrast to the human, rat substantia nigra and caudate display only moderate receptor concentrations. This species difference may explain the difficulty in producing selective nigrostriatal degeneration in rats. Sites densely labeled in rat brain include the locus coeruleus, interpeduncular nucleus, arcuate and periventricular hypothalamic nuclei, and the subfornical organ.     

1-甲基-4-苯基-1，2，3，6-四氢吡啶致黑质多巴胺能神经元变性的分子机制的研究

目的：初步研究1－甲基4－苯基－1,2,3,6－四氢吡啶（MPTP）致黑质多巴胺能神经元变性的分子机制,方法：采用MPTP腹腔注射制备帕金森病褐鼠模型,利用mRNA差异显示技术获得在实验组及正常对照组黑质中差异表达的基因片段,应用反Northern杂交去除假阳性,对真阳性的表达序列标签（EST）进行克隆,测序及同源性比较,结果：用1个随机引物和锚定引物对进行差示反应,在实验组获得2个黑质特异表达的EST,其中1个被克隆,测序,为121bp,与人基因abl外显子1b有低的同源性,结论：MPTP致黑质社经元变性可能是通过改变促神经元凋亡基因表达而起作用。     

Parkinsonism in cirrhosis: pathogenesis and current therapeutic options

Acquired hepatolenticular degeneration, also known as “Parkinsonism in cirrhosis” is characterized by extrapyramidal symptoms including hypokinesia, dystonia and rigidity that are rapidly progressive and may be independent of the severity of cognitive dysfunction. Magnetic resonance imaging reveals T1-weighted hyperintense signals in both globus pallidus and substantia nigra. Estimates of the prevalence of Parkinsonism in cirrhosis have been reported as high as 21 %. The cause of Parkinsonism in cirrhosis has been attributed to manganese deposition in basal ganglia structures, leading to the dysfunction of the dopaminergic neurotransmitter system. In particular, there is evidence from both spectroscopic and biochemical investigations for damage to (or dysfunction of) presynaptic dopamine transporters together with a loss of post-synaptic dopamine receptors in basal ganglia of affected patients. Therapeutic options are limited; ammonia-lowering strategies are without substantial benefit, and an effective manganese chelator is not available. In many patients, L-Dopa replacement therapy and the dopamine receptor agonist bromocriptine are beneficial, and liver transplantation is generally effective. However, reports of post-transplant residual extrapyramidal symptoms suggest an element of irreversibility in some cases.     

Dementia with Lewy bodies

The presence of a high number of Lewy bodies--the morphological marker of Parkinson's disease--in the cerebral cortex of some cases of dementia has been frequently observed in association to Alzheimer type lesions (mainly senile plaques) and changes in the substantia nigra, that may be held responsible for the frequently associated symptoms of parkinsonism. The term "dementia with Lewy body" (DLB) has recently been suggested by a consensus conference and indicates that the pathogenetic mechanism of the dementia remains poorly understood. Marked fluctuations of alertness and of the cognitive performances, moderate parkinsonism and episodes of visual hallucinations may lead to suspect this diagnosis in cases of dementia. Unexplained falls, syncopes, delirium or alterations of consciousness may also be observed, and the patients may then be admitted in departments of internal medicine or geriatrics. The Lewy body is an intraneuronal spherical inclusion, present in Parkinson's disease. It is observed in the brainstem (substantia nigra, locus coeruleus, dorsal nucleus of the Xth nerve) and in the nucleus basalis of Meynert. The cortical Lewy bodies have a different aspect, but retain their antigenic characteristics: they are, in particular, stained by the antiubiquitin antibodies. Recently, they were found to be also labeled by antisynuclein antibodies. A mutation of the synuclein gene was recently identified in cases of familial Parkinson's disease. Clinically as well as pathologically, DLB may thus be difficult to distinguish from Alzheimer's disease on the one hand, and from Parkinson's disease, on the other. That diagnosis, however, is associated with a poor prognosis and should lead to specific therapeutic measures.