中枢神经细胞瘤和脑室外神经细胞瘤

肿瘤由形态一致伴神经元分化的圆形细胞组成,位于侧脑室室间孔区(中枢神经细胞瘤)或脑实质内(脑室外神经细胞瘤),好发于青年人,预后良好.肿瘤具有良性组织学形态,典型结构为小而成熟的肿瘤细胞,呈单一片状排列,其间被神经毡背景分隔(图1a);肿瘤细胞呈簇状排列于血管周围,形成室管膜瘤样"假菊形团"结构(图1b);肿瘤细胞形态较一致,内有圆形或卵圆形细胞核,染色质呈细斑点状,偶见核仁;某些区域以透明细胞为主,类似少突胶质细胞瘤结构(图1c);少见的组织学形态为"Homer-Wright菊形团"结构和神经节细胞.肿瘤细胞弥漫表达突触素(图2).     

透明细胞型室管膜瘤

<正>2016年世界卫生组织(WHO)中枢神经系统肿瘤分类将透明细胞型室管膜瘤定义为室管膜瘤的一个亚型,以细胞呈少突胶质细胞样,可见核周空晕为特点,好发于青年幕上部位。组织学形态观察,肿瘤细胞呈现少突胶质细胞样表型,胞质透明形成核周空晕(图1),常为幕上富血管肿瘤,偶见于颅后窝和脊髓。免疫组织化学染色可见室管膜和血管周围"菊形团"样结构,     

小脑富脂质髓母细胞瘤临床病理学特征:病例报告及文献综述

目的 探讨小脑富脂质髓母细胞瘤临床病理学特征.方法 对一例小脑富脂质髓母细胞瘤患临临床表现、影像学特点,以及组织病理学和免疫组织化学特征进行回顾性分析,并复习相关文献.结果 男性患者,26岁.临床表现为反复头痛,伴头晕、恶心、呕吐.MRI显示病灶填允第四脑室,T1WI呈均匀低信号、T2WI呈不均匀高信号,增强T1WI扫捕病灶晕明显均匀强化,边界清晰.手术后光学显微镜下观察肿瘤细胞弥漫增生,大小一致,胞质淡染,呈弱酸性或嗜双色,核圆形或卵圆形,染色质细腻,核仁可见,核分裂象易见;间质中含有丰富的薄壁血管;富于脂质的细胞呈灶性分布.免疫组织化学染色肿瘤细胞CD56和突触素弥漫阳性,局灶表达神经微丝,弱表达少突胶质细胞系转录凶子-2;不表达巢蛋白、神经元核抗原、S-100蛋白、胶质纤维酸性蛋白和上皮膜抗原;TP53染色阳性(约10%).Ki-67抗原标记指数约为40%.结论 发生于小脑的富脂质髓母细胞瘤为临床罕见的中枢神经系统肿瘤,影像学呈现发生于小脑蚓部向第四脑室生长的占位性病变;病理学表现肿瘤细胞由高度密集的一致圆形细胞组成,具有灶性脂肪样细胞分化.应注意与小脑脂肪神经细胞瘤和室管膜瘤等相鉴别.     

罕见脑实质内菊形团形成型胶质神经元肿瘤临床病理学分析

研究背景菊形团形成型胶质神经元肿瘤(RGNT)为低级别混合性神经元-胶质肿瘤,好发于小脑、第四脑室顶或颅后窝,故也称为第四脑室菊形团形成型胶质神经元肿瘤,以病灶中含神经细胞性"菊形团"和(或)围血管假"菊形团"结构,以及毛细胞型星形细胞瘤成分为特点。目前文献共报道约50例,主要发生于第四脑室及其邻近区域,仅少数发生于第四脑室外,本文报告1例临床罕见的发生于脑实质内的RGNT病例,根据组织学检测方法分析其诊断要点,以期提高临床对该肿瘤的鉴别诊断能力。方法与结果女性患者,12岁。因反复肢体抽搐2年伴轻微间歇性头痛1周入院。MRI显示左侧额叶占位性病变,未累及脑室和硬脑膜,T1WI低信号、T2WI呈不均匀高信号,增强后病灶呈不均匀强化。术中可见病灶位于脑实质内伴囊性变,无包膜,与周围组织分界清楚,手术全切除。肿瘤细胞形成小Homer-Wright样"菊形团"和围血管假"菊形团"结构,并可见典型的毛细胞型星形细胞瘤区域;形成"菊形团"结构的肿瘤细胞突触素和少突胶质细胞转录因子2表达阳性,胶质纤维酸性蛋白表达阴性。结论相对于经典部位,脑实质内RGNT更为罕见,术前难以明确诊断。鉴于发病部位罕见、组织学构象复杂且缺乏特征性影像学表现,临床应提高对脑实质内RGNT的鉴别诊断能力,并注意与其他具有相似组织学构象的中枢神经系统肿瘤相鉴别。     

鞍区和鞍上脑室外神经细胞瘤

目的探讨发生于鞍区和鞍上的脑室外神经细胞瘤的临床病理学特征。方法对1例鞍区和鞍上脑室外神经细胞瘤患者的临床表现、影像学特征、组织学形态、免疫表型和分子遗传学特征进行回顾分析并复习相关文献。结果女性患者,27岁,临床表现为反复头痛伴双眼视物模糊5个月。头部MRI显示鞍区和鞍上占位性病变,T_1WI呈等或低信号,T_2WI呈高或低混杂信号,扩散加权成像呈稍高信号,界限清晰,正常垂体结构显示不清。临床诊断为垂体腺瘤,行经鼻蝶入路垂体腺瘤切除术+脑脊液鼻漏修补术+视神经减压术,手术全切除肿瘤。组织学形态可见肿瘤细胞呈弥漫浸润性生长,部分区域可见神经毡背景;肿瘤细胞大小和形态相对一致,胞核圆形或卵圆形,染色质细腻深染,未见核分裂象。免疫组织化学染色可见肿瘤细胞胞核表达神经元核抗原和甲状腺转录因子-1,胞核和胞质表达钙视网膜蛋白,胞质表达突触素、嗜铬素A、上皮钙黏素和基质金属蛋白酶-9;胞核局灶性表达S-100蛋白,胞质局灶性表达神经微丝蛋白、细胞角蛋白8和波形蛋白;Ki-67抗原标记指数约为3%。网织纤维染色呈阴性。基因检测可见肿瘤细胞无异柠檬酸脱氢酶基因突变,无1p/19q-共缺失。最终病理诊断为脑室外神经细胞瘤(WHOⅡ级)。结论鞍区和鞍上脑室外神经细胞瘤临床极为罕见,组织学形态与发生于脑室的中枢神经细胞瘤相似,表现为肿瘤弥漫浸润性生长,肿瘤细胞形态较一致,胞核圆形,可见神经毡背景和"树枝"状薄壁毛细血管。应注意与垂体腺瘤、少突胶质细胞瘤和透明细胞型室管膜瘤等相鉴别。     

室管膜下巨细胞型星形细胞瘤

研究背景室管膜下巨细胞型星形细胞瘤为中枢神经系统少见肿瘤,多发生于20岁以下的儿童或青少年,好发于侧脑室室间孔区,大多数患者具有独特的组织病理学和免疫组织化学特征。方法与结果女性患者,13岁。临床表现为头晕、头痛伴呕吐;MRI显示左侧侧脑室室间孔区异常信号,增强后病灶呈不均匀明显强化。组织学观察肿瘤细胞大小不一、形态多样,大片束状排列的梭形细胞背景中可见成片的胖细胞样肿瘤细胞,胞质丰富呈毛玻璃样,以及少量节细胞样巨细胞和多核瘤巨细胞;肿瘤细胞胞核呈空泡状、核仁明显,无明显血管内皮增生或坏死区域;肿瘤细胞弥漫表达波形蛋白,部分表达S-100蛋白、上皮膜抗原和胶质纤维酸性蛋白,少量胖细胞样肿瘤细胞表达突触素,不表达神经微丝蛋白、神经元核抗原和细胞角蛋白,Ki-67抗原标记指数1%。结论室管膜下巨细胞型星形细胞瘤为中枢神经系统良性肿瘤(WHOⅠ级),具有独特的临床表现和组织学特征,需注意与肥胖细胞型星形细胞瘤、室管膜瘤、神经节细胞胶质瘤和巨细胞型胶质母细胞瘤等相鉴别。     

脑室内伸长细胞型室管膜瘤的临床病理学特征:病例报告并文献综述

目的 探讨脑室内伸长细胞型室管膜瘤的临床病理学特征.方法 对1例右侧侧脑室内伸长细胞型室管膜瘤患者的临床、影像学、病理学和免疫组织化学特征进行回顾性分析,并复习相关文献.结果 患者临床表现为无明显诱因的头痛,呈进行性加重;MRI检查提示右侧侧脑室内囊实性病灶.经手术完全切除,大体组织呈鱼肉样,约2 cm×3 cm×1 cm大小,部分区域脑组织呈沙砾感.组织病理学检查长梭形肿瘤细胞呈单极或双极伸长,由簇状梭形细胞形成的细胞核密集区及细胞突起呈束状排列的纤维样无核区构成,未见核分裂象及坏死灶,围绕血管分布形成血管周围假菊形团;部分区域可见血管壁玻璃样变和钙化小体.免疫组织化学染色肿瘤细胞胶质纤维酸性蛋白、S-100蛋白、波形蛋白、巢蛋白和神经微丝表达阳性;上皮膜抗原呈局灶性核旁点状阳性反应;突触素和神经元核抗原表达阴性;MIB-1标记指数约为0.50%.文献报道国外29例伸长细胞型室管膜瘤,其中发生于脑室内6例,组织病理学和免疫组织化学特征与本文病例基本一致.电子显微镜观察肿瘤细胞呈室管膜细胞的典型特征,包括细胞突起内可见大量的中间纤维丝、明显的细胞间连接、大量位于细胞表面的纤细的微绒毛以及微绒毛构成的小管状结构.结论 伸长细胞型室管膜瘤是一种较少见的起源于室管膜伸长细胞的室管膜瘤亚型,组织病理学上应与其他由梭形细胞组成的神经系统肿瘤相鉴别,如毛细胞型星形细胞瘤、纤维型星形细胞瘤、神经鞘瘤和孤立性纤维性肿瘤等,免疫组织化学染色及电子显微镜下的特征性改变有助于诊断与鉴别诊断.     

第四脑室形成菊形团的胶质神经元肿瘤

目的探讨第四脑室形成菊形团的胶质神经元肿瘤的临床病理学特征。方法与结果女性患者,24岁。临床表现为渐进性加重的头晕、运动障碍如行走不稳等症状。MRI显示第四脑室扩张,其内可见长T1、长T2信号影,边界清晰,病灶内少许强化。手术切除第四脑室和菱形窝上方肿瘤,术中可见肿瘤分界清楚,分块切除,显微镜下未见肿瘤残留。光学显微镜观察肿瘤组织分别由神经元成分("菊形团"或血管周围假"菊形团"样结构,中心呈均质淡染伴微囊形成)和神经胶质成分(毛细胞型星形细胞瘤样结构)构成,"菊形团"中心和血管周围假"菊形团"样结构表达突触素和少突胶质细胞转录因子2,毛细胞型星形细胞瘤样区域表达胶质纤维酸性蛋白,二者散在表达S-100蛋白和神经元特异性烯醇化酶,Ki-67抗原标记指数为1.50%~2.00%。结论第四脑室形成菊形团的胶质神经元肿瘤为临床罕见的良性肿瘤,影像学呈边界清晰的实性或囊实性占位性病变;具有两种特征性组织学成分,免疫组织化学染色两种成分均表达突触素和胶质纤维酸性蛋白可提示诊断。     

脑室内少突胶质细胞瘤与中枢神经细胞瘤的临床病理分析

目的 总结发生于脑室内的少突胶质细胞瘤与中枢神经细胞瘤的临床病理特点。方法 选取同期发生于侧脑室内的6例少突胶质细胞瘤和5例中枢神经细胞瘤进行回顾性研究,对各自的临床病理特点进行分析,探讨二者的鉴别诊断要点。结果。发生于脑室内的少突胶质细胞瘤与中枢神经细胞瘤在临床表现、神经影像学和光镜下的病理学表现均十分相似。结论 目前认为光镜下出现无核原纤维岛、免疫组织化学显示突触素阳性表达、电镜超微结构检查证实肿瘤细胞有神经元分化证据为诊断中枢神经细胞瘤的金标准,也是鉴别少突胶质细胞瘤与中枢神经细胞瘤的重要依据。     

胚胎发育不良性神经上皮瘤的临床病理特征(附1例报告)

目的 探讨胚胎发育不良性神经上皮瘤(DNT)的临床病理特征.方法 回顾性分析1例DNT患者的临床资料.结果 本例为青年男性,首发症状为发作性肢体抽搐伴意识障碍.MRI显示颞叶T1WI低信号,T2WI高信号,无水肿及占位效应.病变由多结节特异性胶质神经元和微囊细纤维黏液性基质等成分组成,肿瘤细胞主要有神经元、少突胶质样细胞(OLC)和星形细胞3种成分,伴有皮质发育不良.免疫组织化学结果为神经元及部分OLC呈神经核抗原、神经突触素、神经元特异性烯醇化酶阳性表达;OLC呈S-100蛋白阳性表达;星形细胞呈胶质纤维酸性蛋白阳性表达;瘤细胞Ki-67阳性率为1％.结论 DNT好发于儿童和年轻人,部分患者有慢性癫痫病史.DNT的组织学特征是神经胶质及神经元成分.     

The effect of age of onset of PD on risk of dementia

Background Dementia occurs in the majority of patients with Parkinson’s disease (PD). Late onset of PD has been reported to be associated with a higher risk for dementia. However, age at onset (AAO) and age at baseline assessment are often correlated. The aim of this study was to explore whether AAO of PD symptoms is a risk factor for dementia independent of the general effect of age. Methods Two community-based studies of PD in New York (n = 281) and Rogaland county, Norway (n = 227) and two population-based groups of healthy elderly from New York (n = 180) and Odense, Denmark (n = 2414) were followed prospectively for 3–4 years and assessed for dementia according to DSM-IIIR. All PD and control cases underwent neurological examination and were followed with neurological and neuropsychological assessments. We used Cox proportional hazards regression based on three different time scales to explore the effect of AAO of PD on risk of dementia, adjusting for age at baseline and other demographic and clinical variables. Findings In both PD groups and in the pooled analyses, there was a significant effect of age at baseline assessment on the time to develop dementia, but there was no effect of AAO independent of age itself. Consistent with these results, there was no increased relative effect of age on the time to develop dementia in PD cases compared with controls. Interpretation This study shows that it is the general effect of age, rather than AAO that is associated with incident dementia in subjects with PD. Received in revised form: 22 December 2005     

腺垂体功能减退症临床特征变化分析

目的探讨腺垂体功能减退症患者的病因结构变化及临床表现。方法回顾性分析我院2013-01—2016-12住院及门诊78例腺垂体功能减退症患者的临床资料。结果男32例(41.03%),女46例(58.97%);诊断时年龄11~89岁,平均62.5岁;鞍区占位(包括术前及术后)52例(66.67%),席汉综合征8例(10.26%),空泡蝶鞍9例(11.65%),病因不明8例(10.26%),垂体-下丘脑发育不良1例(1.28%)。首次就诊科室:纳差厌食、恶心呕吐就诊于消化内科36例(46.15%)最常见。ACTH+TSH+Gn+G激素缺乏为19例最多,占24.36%,ACTH+TSH+Gn缺乏15例,占19.23%。结论腺垂体功能减退症病因结构发生变化,发病人群、首发症状及受累激素也不同,患者女性多于男性,发病年龄偏高,症状不典型,分布于临床多个科室,其中以低钠血症为首发临床表现就诊消化内科最多。     

Standards of instrumentation of EMG

Standardization of Electromyography (EMG) instrumentation is of particular importance to ensure high quality recordings. This consensus report on “Standards of Instrumentation of EMG” is an update and extension of the earlier IFCN Guidelines published in 1999. First, a panel of experts in different fields from different geographical distributions was invited to submit a section on their particular interest and expertise. Then, the merged document was circulated for comments and edits until a consensus emerged.The first sections in this document cover technical aspects such as instrumentation, EMG hardware and software including amplifiers and filters, digital signal analysis and instrumentation settings. Other sections cover the topics such as temporary storage, trigger and delay line, averaging, electrode types, stimulation techniques for optimal and standardised EMG examinations, and the artefacts electromyographers may face and safety rules they should follow. Finally, storage of data and databases, report generators and external communication are summarized.     

Release of endogenous catecholamines from slices of hypothalamus of rats

The release of endogenous catecholamines from superfused slices of rat hypothalamus was studied under basal conditions and during release evoked by 40 mM K⁺. Catecholamines in superfusates, and in extracts of the tissue after stimulation, were isolated by column chromatography and quantitated by liquid chromatography with electrochemical detection. Norepinephrine (NE) was not consistently demonstrable in superfusate collected under basal conditions, but 40 mM K⁺ caused the release of from 2 to 4 ng/g of tissue per min. The addition of cocaine to the superfusate caused increases in basal and evoked release of NE. Epinephrine (E) could be measured in superfusates of slices from male but not female rats and then only when cocaine was added to the superfusate. Accordingly, the concentration of E in hypothalamus was greater in male rats than in female rats. Dopamine (DA) was not consistently measurable in the spontaneous overflow from slices either in the presence or absence of cocaine. K⁺-evoked release of DA could be demonstrated in slices from female rats. The addition of cocaine increased the evoked release of DA from slices from both sexes. Corticosterone, added to cocaine, had no effects on the efflux of any of the catecholamines. The experiments suggest that neuronal reuptake of all catecholamines is very efficient in the hypothalamus both under basal conditions and during evoked release.     

帕金森病运动症状进展分析

目的分析帕金森病(PD)患者运动症状进展特点。方法采用PD统一评分量表(UPDRS)Ⅲ对912例PD患者进行评估。结果与病程1年的患者比较,除病程1~2年的患者外,其他病程患者的UPDRSⅢ评分、强直分、姿势或步态异常分、轴性症状总分、言语分、步态分显著升高(均P0.05),病程5~6年及14年患者的震颤分,病程5~6年、7~8年、9~13年、14年患者的运动迟缓分、姿势分显著升高(P0.05~0.01)。轴性症状进展速度高于UPDRSⅢ评分。结论 PD患者病程早期UPDRSⅢ评分进展快,震颤症状进展独立于其他症状,轴性症状评分较UPDRSⅢ更敏感地反映疾病加重趋势。     

Effects of prevention of afferentation of the development of the chick optic lobe

BONDY, S. C., M. E. HARRINGTON AND C. L. ANDERSON. Effects of prevention of afferentation on the developmentof the chick optic lobe. BRAIN RES. BULL. 3(5) 411–413, 1978.—The effects of unilateral extirpation of the right optic cup of the three-day incubated chick embryo upon the rate of synthesis and the stability of DNA in the non-innervated optic lobe, have been studied. This surgical procedure prevents innervation of the optic lobe contralateral to the removed eye, while the other optic lobe is normally innervated by retinal ganglion cells of the remaining eye. At the 20th day of incubation, the DNA content of the non-innervated lobe was below that of the paired lobe receiving normal innervation. This deficiency of cell number was caused by two events; death of an excess number of neurons formed early in embryogenesis and a reduced rate of glial proliferation in the later stages of incubation.     

Frequency of accumulation of filaments in adaxonal cytoplasm of Schwann cells of myelinated fibers of rat spinal roots

Summary The frequency of accumulation of 6-nm filaments in the adaxonal cytoplasm of Schwann cells in the 6th lumbar dorsal and ventral roots was evaluated in 4-, 8-, 26- and 45-week-old Sprague-Dawley rats. The frequency was higher in 4- and 8-week-old (growing) rats than in 26- and 45-week old (mature) rats, and also higher in ventral than in dorsal roots in 4-, 8- and 26-week old rats. There were no clusters on certain groups of myelinated fibers according to the size of transverse axonal area, in both the ventral and dorsal roots. Therefore, this accumulation may reflect certain functions of the adaxonal cytoplasm of Schwann cell during natural growth and maturation of the axon and myelin sheath.     

《绵阳市社会心理服务工作管理办法》解读

2018年,国家卫生健康委员会等10部委联合发布《关于印发全国社会心理服务体系建设试点工作方案的通知》,四川省绵阳市被列为全国第一批试点地区。绵阳市人民政府依据《中华人民共和国精神卫生法》等相关法律法规和文件精神,结合前期调查研究和社会心理服务工作的试点实际,编制出台了《绵阳市社会心理服务工作管理办法》,并于2021年12月25日起施行。本文围绕社会心理服务的相关概念、办法总则、重点内容、保障措施等方面进行解读,以期为社会心理服务工作的规范、持续和有效开展提供参考。     

Function of circle of Willis

Nearly 400 years ago, Thomas Willis described the arterial ring at the base of the brain (the circle of Willis, CW) and recognized it as a compensatory system in the case of arterial occlusion. This theory is still accepted. We present several arguments that via negativa should discard the compensatory theory. (1) Current theory is anthropocentric; it ignores other species and their analog structures. (2) Arterial pathologies are diseases of old age, appearing after gene propagation. (3) According to the current theory, evolution has foresight. (4) Its commonness among animals indicates that it is probably a convergent evolutionary structure. (5) It was observed that communicating arteries are too small for effective blood flow, and (6) missing or hypoplastic in the majority of the population. We infer that CW, under physiologic conditions, serves as a passive pressure dissipating system; without considerable blood flow, pressure is transferred from the high to low pressure end, the latter being another arterial component of CW. Pressure gradient exists because pulse wave and blood flow arrive into the skull through different cerebral arteries asynchronously, due to arterial tree asymmetry. Therefore, CW and its communicating arteries protect cerebral artery and blood–brain barrier from hemodynamic stress.     

阿立哌唑对精神分裂症患者生活质量的影响

目的:比较阿立哌唑与利培酮对精神分裂症患者生活质量的影响。方法:60例精神分裂患者随机平分为两组各30例,分别给予阿立哌唑和利培酮治疗。疗程8周。用生活质量综合评定问卷-74(GQOLI-74)、阳性与阴性症状量表(PANSS)及副反应量表(TESS)评定疗效及不良反应。结果:阿立哌唑与利培酮均能显著提高精神分裂症患者生活质量,但阿立哌唑在改善GQOLI-74总分、躯体健康及社会功能维度优于利培酮。结论:阿立哌唑治疗有利于提高精神分裂症患者生活质量。