中枢神经系统脱髓鞘假瘤与胶质瘤的鉴别诊断和临床分析

目的探讨CNS脱髓鞘假瘤(DPT)的临床特点,并与胶质瘤进行鉴别,以提高对DPT的认识。方法收集均经病理证实的13例DPT和17例胶质瘤患者的临床资料,对比二者之间的临床、影像学、实验室检查及病理特点,并分析DPT的治疗与转归。结果 DPT组平均发病年龄高于胶质瘤组(P0.05)。DPT组女性多见,胶质瘤组无明显性别差异。DPT组急性或亚急性起病(76.9%)常见,胶质瘤组慢性起病常见(70.5%)。DPT组首发症状以头痛(38.4%)多见,胶质瘤组以头痛(41.1%)、痫性发作(23.5%)多见。DPT组影像学部分病灶可见"垂直征"及特异性"开环征",DWI高b值呈高信号,胶质瘤DWI高b值多呈低信号。MRS检查出现特异性β,γ-谷氨酸复合物(Glx)峰升高可与胶质瘤进行鉴别。两组CSF压力、蛋白相比差异有统计学意义(均P0.05)。病理活组织检查出现特异性核分裂状的Creutzfeldt细胞及CD68免疫组化染色阳性有助于两者鉴别诊断。经激素治疗后DPT大部分病灶明显缩小或消失,部分可发展为多发性硬化。结论DPT在临床表现及影像学等方面与胶质瘤相似。影像学检查出现"垂直征"、"开环征"及β,γ-Glx峰升高可能具有特异性,DWI/MRS检查有助于鉴别胶质瘤。激素实验性治疗可能有助于鉴别病变性质,但最终确诊仍需病理结果,部分可发展为多发性硬化。     

脑内脱髓鞘性假瘤的诊断与治疗（附3例报告）

目的探讨脑内脱髓鞘性假瘤（DPT）的临床表现、影像学特点、病理特征及治疗效果。方法回顾性分析3例DPT的临床资料,并复习相关文献。结果脑内DPT以急性起病为主;影像学多表现为皮质下单一病灶,其强化呈非闭合性、斑片样或垂直于侧脑室分布的火焰样;病理检查发现病灶呈炎性脱髓鞘样改变;皮质类固醇激素治疗后症状均得到明显改善。结论脑内DPT的临床表现与大多颅内肿瘤类似,易误诊为脑胶质瘤;其影像学具有一定的特征性表现;类固醇激素治疗可获得满意疗效。     

酷似肿瘤的脊髓炎性脱髓鞘假瘤

目的总结脊髓脱髓鞘似瘤的临床、影像学和病理特点。方法回顾分析6例脊髓脱髓鞘假瘤的临床、影像学特征，对其中2例误诊为髓内肿瘤术后病理表现进行分析。结果脊髓脱髓鞘假瘤常以急性或亚急性起病，最常累及颈髓和胸髓，可伴有周围神经受损：脊髓脱髓鞘假瘤单时相起病，症状、体征提示为单病灶；MRI检金病灶多单发（偶可多发），占位水肿效应明显，增强后可呈非闭合性花环样或环肜强化；病理为炎性脱髓鞘改变，白质内髓鞘破坏并水肿，病灶内以小血管周围大量淋巴细胞浸润、巨噬细胞聚集为主要表现；绐激素治疗后好转。结论脊髓炎性脱髓鞘假瘤MRI酷似髓内肿瘤，容易造成误诊。可试用激素治疗或组织活检，不要急于手术。     

炎性脱髓鞘性假瘤1例报告

炎性脱髓鞘性假瘤是中枢神经系统脱髓鞘病变的一种特殊类型.由于其在MRI上多是单一病灶,占位效应及灶周水肿明显,故难与脑脓肿、神经胶质瘤、脑转移瘤等鉴别.现将我院收治的1例术前误诊为星形细胞瘤的炎性脱髓鞘性假瘤的MRI及病理报道如下.     

不典型性脊髓脱髓鞘疾病的诊断和鉴别诊断（附三例报告）

目的 探讨不典型性脊髓脱髓鞘疾病的诊断和鉴别诊断方法.方法 对3例不典型性脊髓脱髓鞘病变的临床特点、脊髓磁共振(MRI)和手术大体所见以及治疗效果进行分析,并用组织学和免疫组化法对活检组织进行病理学观察.结果 3例患者均为亚急性起病,表现为肢体无力和麻木.MRI提示为占据4～6个椎体范围的髓内病变,临床诊断均为星形细胞瘤;3例均行病变部位活检,3份脊髓标本镜下检查可见大片脱髓鞘坏死,灶内可见格子细胞,血管周围淋巴细胞及浆细胞浸润,病理学诊断为脱髓鞘病变.术后给予激素治疗,复查MRI结果显示病灶好转.结论 不典型性脊髓脱髓鞘疾病临床容易误诊为髓内肿瘤,经试验性激素治疗和病理检查能及时进行诊断和鉴别诊断.     

假瘤型炎性脱髓鞘病的临床及病理

目的 :探讨假瘤型炎性脱髓病的临床病理特点。方法 :分析 5例假瘤型炎性脱髓鞘病的临床、影像及病理特点。结果 :本组临床特点 :1亚急性或慢性起病 ,逐渐进展。 2症状、体征及 MRI均提示为单一病灶。 3MRI所见病灶比 CT大 ,可有占位效应 ,也可呈周边花边样或环形强化 (例 2呈均匀强化 )。病理呈炎性脱髓鞘改变 ;白质内大片脱髓鞘区 ,血管周围淋巴细胞浸润 ,呈袖套样 ,伴有胶质细胞增生。也可主要累及灰质。结论 :1假瘤型炎症性脱髓鞘病可能是多发性硬化 (MS)中的一种特殊类型 ,或者是 MS与急性播散性脑脊髓炎之间的过渡类型。 2假瘤型炎性脱髓鞘病与胶质瘤不易鉴别 ,可试用肾上腺皮质激素治疗或者进行组织活检 ,不可急于手术。     

脑内脱髓鞘假瘤病理、临床与磁共振成像对照分析

目的 探讨脑内脱髓鞘假瘤的MR影像学表现并分析其病理、临床与MRI影像三者之间的相关性. 方法 回顾性分析总结经病理证实的8例病例的病理、临床及MRI表现并加以对照. 结果 临床表现以肢体运动障碍和(或)肢体感觉障碍最常见,亦可见癫痫等少见症状.MRI影像显示病灶均单发、以侵犯皮层下白质为主,"瘤体"多为圆形或类圆形、边界多较清楚,信号多为T1均匀低信号、T2均匀高信号,多明显均一强化,多有明显占位效应及灶周水肿.病理上病变处脑组织脱髓鞘,轴索尚保存.见大量淋巴细胞在血管周围围绕浸润呈套袖现象,此外尚可见大量的巨噬细胞以及胶质细胞增生. 结论 脑内脱髓鞘假瘤病理、临床与MRI影像的相关性较好,MRI是其诊断的重要依据.其发生于幕上时与胶质瘤鉴别诊断相对较易,发生于幕下时与Ⅰ～Ⅱ级胶质瘤不易鉴别,此时应密切结合临床.对于临床与影像学诊断均较困难者,可行肾上腺皮质激素试验性治疗或脑组织活检.     

颅内单发脱髓鞘假瘤的诊治分析

目的 总结颅内单发脱髓鞘假瘤（DPT）的诊治经验。方法 回顾性分析2011年5月至2021年6月收治的19例DPT的临床资料。结果 19例术前均考虑胶质瘤,开颅手术探查;17例术中快速病理结果为脱髓鞘病变或胶质增生,终止手术并给予激素治疗;2例术中快速病理为高级别胶质瘤,行病灶全切除,术后病理结果为胶质增生,病情进展再次手术病理检查确诊为DPT。术后4周复查头部MRI,15例病灶缩小,4例病灶消失;术后2个月复查头部MRI示病灶均完全消失。术后3个月,14例临床症状完全消失,2例遗留肢体偏瘫,3例遗留不同程度失语,1例意识朦胧。结论 DPT是中枢神经系统特有的炎症性疾病,及时诊断并给予相应治疗,预后良好,但易误诊为肿瘤性病变;因此,对于临床表现不典型的颅内炎症性疾病,怀疑肿瘤性病变时,应完善各种检查,降低误诊率,避免不必要的开颅手术。     

CT、MRI引导下立体定向颅内病变活检

目的探讨立体定向脑活检方法的可靠性和安全性,研究手术方法及技术要点。方法回顾性分析我院1999年6月至2005年11月,CT或MRI引导下立体定向活检47例。其中男32例,女15例。年龄18~83岁,平均年龄36.4±14.8岁。病灶位于:大脑半球25例,基底节及丘脑13例,鞍区6例,多发病变3例。结果活检病理诊断:星形细胞瘤I级22(46.8%),星形细胞瘤II级5例(10.6%),星形细胞瘤III级1例(2.1%),胶质母细胞瘤(IV级)3例(6.4%),炎性病变、转移瘤各3例(6.4%),颅咽管瘤、脑囊虫病各2例(4.3%),脑胶质瘤病、假瘤型炎性脱髓鞘病、结核瘤各1例(2.1%),胶质增生或未明确诊断3例(6.4%)。活检确诊率94.6%,无并发症发生。结论立体定向脑深部病灶活检技术安全、可靠,对临床诊断、鉴别诊断、治疗的选择及手术有重要的意义。     

脑内脱髓鞘假瘤

目的探讨脑内脱髓鞘假瘤的病理特点。方法回顾性分析11例经手术证实的脑内脱髓鞘假瘤的临床与影像学表现、病理特点、治疗效果及随访情况。结果脱髓鞘假瘤以急性起病为主，病程呈单时相；影像学多表现为皮质下单一病灶、其强化呈非闭合性或与脑室垂直：病理学发现病灶呈炎性脱髓鞘样改变。结论脱髓鞘假瘤临床表现各异，典型的临床及影像学表现有助于诊断；组织病理学检查是确诊的金标准。脱髓鞘假瘤不宜手术治疗，肾上腺皮质激素对此病治疗效果好。     

Effects of genistein on neuronal apoptosis, and expression of Bcl-2 and Bax proteins in the hippocampus of ovariectomized rats

Genistein is one of several isoflavones that has a structure similar to 17β-estradiol, has a strong antioxidant effect, and a high affinity to estrogen receptors. At 15 weeks after ovariectomy, the expression of Bcl-2 in the hippocampus of rats decreased and Bax expression increased, with an obvious upregulation of apoptosis. However, intraperitoneal injection of genistein or 17β-estradiol for 15 consecutive weeks from the second day after operation upregulated Bcl-2 protein expression downregulated Bax protein expression, and attenuated hippocampal neuron apoptosis. Our experimental findings indicate that long-term intervention with genistein can lead to a decrease in apoptosis in hippocampal neurons following ovariectomy, upregulate the expression of Bcl-2, and downregulate the expression of Bax. In addition, genistein and 17β-estradiol play equal anti-apoptotic and neuroprotective roles.     

Ultrastructure of non-myelinated neurons during energy deprivation

Summary This paper examines the neuropathology of oxygen-glucose deprivation uncomplicated by stagnant conditions. Rabbit vagus nerves were pulled into asmulti-compartment perfusion chamber, stimulated five times per second and deprived of energy by substituting nitrogen and deoxyglucose for oxygen and glucose in the Locke's perfusate. After incubation the compartments were perfused with gluteraldehyde solution, and the nerves were prepared for electron microscopy. Fixation in the compartments ensured precise cross and longitudinal sections which permitted quantitative comparisons. Although the action potentials ceased in 45 min, 1 h of energy deprivation did not significantly affect the ultrastructure. After 2 h of deprivation the axons were smaller and flattened and microtubules appeared packed together. In the smallest axons the microtubules were gone, the neurofilaments were compacted and the few mitochondria had a dense, homogenous appearance. By 4 h the shrinking was extreme, yet 8% were swollen much larger than any of the controls. Longitudinal views showed these balloned areas were greatly expanded regions of the smallest axons. Both tiny and huge regions were devoid of microtubules and the swollen axons contained expanded mitochondria.Calcium is indirectly implicated in the pathogenesis by the concurrence of mitochondrial alteration as the microtubules disappear coupled with the known role of mitochondria in calcium regulation and the reported effect of high calcium on microtubual dissociation. In is suggested that axons first shrink as osmotially active molecules are used or washed out. After a time without energy the mitochondria can no longer regulate the intracellular calcium, microtubules dissociate, and calcium-activated phospholipases create osmotically active molecules. Finally, high-amplitude, disruptive swelling occurs.Supported, in part, by a Grant-in-aid from the American Heart Association with funds contributed by the American Heart Association, Kansas Affiliate and by the University of Kansas Biomedical Sciences Support Grant RR0737     

星形胶质细胞对天冬氨酸特异性半胱氨酸蛋白酶介导β淀粉样蛋白早期突触毒性作用的影响

目的探讨星形胶质细胞(astrocyte,AS)对天冬氨酸特异性半胱氨酸蛋白酶(cysteinyl aspartate specific proteinase,caspase)介导β淀粉样蛋白(β-amyloid,Aβ)早期突触毒性作用的影响,以期为进一步研究与血管性痴呆(vascular dementia,Va D)的发病机制奠定基础。方法以原代培养大鼠海马纯神经元体系(NE-S)及混合培养体系(MIX-S,主要包含神经元及AS)为研究对象,各体系分为6组:对照组、caspase-8抑制剂组、caspase-9抑制剂组、Aβ处理组、caspase-8抑制剂预处理加Aβ组和caspase-9抑制剂预处理加Aβ组。免疫荧光检测各组近胞体10μm段树突中突触后密度蛋白(postsynaptic density-95,PSD95)表达量的变化。结果 1在NE-S与MIX-S中,与对照组相比,caspase-8抑制剂组、caspase-9抑制剂组PSD95的表达量均无明显差异,Aβ处理组PSD95的表达量均显著降低(P均0.001)。2在NE-S中,与Aβ处理组相比,caspase-9抑制剂预处理加Aβ组PSD95的表达量显著回升至对照组水平,caspase-8抑制剂预处理加Aβ组则无显著改变;在MIX-S中的结果则相反,即caspase-8抑制剂预处理加Aβ组PSD95的表达量显著回升至对照组水平,而caspase-9抑制剂预处理加Aβ组则无显著改变。3MIX-S与NE-S两种培养系统间相比较,对照组间及Aβ处理组间PSD95的表达量均无显著差异,而caspase-8抑制剂预处理加Aβ组间及caspase-9抑制剂预处理加Aβ组间PSD95的表达量差异有显著性。结论在Aβ早期突触毒性作用中,AS参与caspase-8介导的死亡受体通路激活过程,且参与抑制神经元的线粒体通路。     

Development of 18F-labeled radiotracers for neuroreceptor imaging with positron emission tomography

Positron emission tomography (PET) is an in vivo molecular imaging tool which is widely used in nuclear medicine for early diagnosis and treatment follow-up of many brain diseases. PET uses biomolecules as probes which are labeled with radionuclides of short half-lives, synthesized prior to the imaging studies. These probes are called radiotracers. Fluorine-18 is a radionuclide routinely used in the radiolabeling of neuroreceptor ligands for PET because of its favorable half-life of 109.8 min. The delivery of such radiotracers into the brain provides images of transport, metabolic, and neurotransmission processes on the molecular level. After a short introduction into the principles of PET, this review mainly focuses on the strategy of radiotracer development bridging from basic science to biomedical application. Successful radiotracer design as described here provides molecular probes which not only are useful for imaging of human brain diseases, but also allow molecular neuroreceptor imaging studies in various small-animal models of disease, including genetically-engineered animals. Furthermore, they provide a powerful tool for in vivo pharmacology during the process of pre-clinical drug development to identify new drug targets, to investigate pathophysiology, to discover potential drug candidates, and to evaluate the pharmacokinetics and pharmacodynamics of drugs in vivo.     

Differential expression of carboxyl terminal derivatives of amyloid precursor protein among cell lines.

Understanding the pathway for amyloid percursor protein (APP) catabolism has become an important line of investigation. APP is a ubiquitous membrane bound protein that is rapidly cleaved at the membrane, yielding a secreted protein identical to protease nexin II and an internalized 11.5 kDa 100 residue C terminal derivative (CTD). The levels of CTDs in a variety of cell lines have been examined and were found to differ. Cell types associated with the pathology of Alzheimer's disease (AD), such as olfactory neuroblasts (ON) and cortical vascular endothelial cells, have higher levels of CTDs than lymphoblasts and melanoma cells. The mechanism of CTD catabolism appears to involve the lysosome because blockade of lysosomal but not endosomal or mitochondrial function results in increased levels of CTDs. Under these conditions, production of larger, amyloidogenic CTDs is also seen. In cells possessing higher levels of CTDs we find that the mechanism for production of amyloidogenic CTDs may involve the internalization of intact full-length APP. Thus, inhibition of the lysosomal system appears capable of generating amyloidogenic peptides. The amount of amyloidogenic peptides appears to vary among cell lines. Such variation may shed light on why amyloid accumulates around specific cell types such as vascular endothelial cells, neurons, and glia. Finally, disfunction of the lysosomal system may play a role in the pathogenesis of Alzheimer's disease.     

Magnetic resonance morphometry of the loss of gray matter volume in Parkinson’s disease patients

Voxel-based morphometry can be used to quantitatively compare structural differences and functional changes of gray matter in subjects.In the present study,we compared gray matter images of 32 patients with Parkinson’s disease and 25 healthy controls using voxel-based morphometry based on 3.0 T high-field magnetic resonance T1-weighted imaging and clinical neurological scale scores.Results showed that the scores in Mini-Mental State Examination and Montreal Cognitive Assessment were lower in patients compared with controls.In particular,the scores of visuospatial/executive function items in Montreal Cognitive Assessment were significantly reduced,but mean scores of non-motor symptoms significantly increased,in patients with Parkinson’s disease.In addition,gray matter volume was significantly diminished in Parkinson’s disease patients compared with normal controls,including bilateral temporal lobe,bilateral occipital lobe,bilateral parietal lobe,bilateral frontal lobe,bilateral insular lobe,bilateral parahippocampal gyrus,bilateral amygdale,right uncus,and right posterior lobe of the cerebellum.These findings indicate that voxel-based morphometry can accurately and quantitatively assess the loss of gray matter volume in patients with Parkinson’s disease,and provide essential neuroimaging evidence for multisystem pathological mechanisms involved in Parkinson’s disease.     

Neuroprotective role of fibronectin in neuron-glial extrasynaptic transmission

Most hypotheses concerning the mechanisms underlying Parkinson’s disease are based on altered synaptic transmission of the nigrostriatal system.However,extrasynaptic transmission was recently found to affect dopamine neurotransmitter delivery by anisotropic diffusion in the extracellular matrix,which is modulated by various extracellular matrix components such as fibronectin.The present study reviewed the neuroprotective effect of fibronectin in extrasynaptic transmission.Fibronectin can regulate neuroactive substance diffusion and receptor activation,and exert antineuroinflammatory,adhesive and neuroprotective roles.Fibronectin can bind to integrin and growth factor receptors to transactivate intracellular signaling events such as the phosphatidylinositol 3-kinase/protein kinase B pathway to regulate or amplify growth factor-like neuroprotective actions.Fibronectin is assembled into a fibrillar network around cells to facilitate cell migration,molecule and ion diffusion,and even drug delivery and treatment.In addition,the present study analyzed the neuroprotective mechanism of fibronectin in the pathogenesis of Parkinson’s disease,involving integrin and growth factor receptor interactions,and discussed the possible therapeutic and diagnostic significance of fibronectin in Parkinson’s disease.     

Clinical and molecular research of neuroacanthocytosis

Neuroacanthocytosis is an autosomal recessive or dominant inherited disease characterized by widespread, non-specific nervous system symptoms, or spiculated "acanthocytic" red blood cells. The clinical manifestations typically involve chorea and dystonia, or a range of other movement disorders. Psychiatric and cognitive symptoms may also be present. The two core neuroacanthocytosis syndromes, in which acanthocytosis is atypical, are autosomal recessive chorea-acanthocytosis and X-linked McLeod syndrome. Acanthocytes are found in a smaller proportion of patients with Huntington’s disease-like 2 and pantothenate kinase-associated neurodegeneration. Because the clinical manifestations are diverse and complicated, in this review we present features of inheritance, age of onset, neuroimaging and laboratory findings, as well as the spectrum of central and peripheral neurological abnormalities and extraneuronal involvement to help distinguish the four specific syndromes.     

轻型缺血性卒中静脉溶栓后双重抗血小板疗效观察

目的 观察rt-PA静脉溶栓联合双重抗血小板治疗轻型缺血性卒中的有效性及安全性。 方法 以2013年12月-2016年12月在石家庄市第一医院连续住院治疗的轻型缺血性卒中患者为研究 对象,将其随机分为对照组、溶栓+单抗组和溶栓+双抗组。对照组不进行静脉溶栓,长期口服阿 司匹林（100 mg/d）抗血小板治疗;溶栓+单抗组在rt-PA静脉溶栓（0.9 mg/kg,最大剂量90 mg）基 础上长期单用阿司匹林（100 mg/d）抗血小板治疗;溶栓+双抗组在溶栓后单抗基础上加用氯吡格雷 （75 mg/d）双重抗血小板治疗,双抗治疗21 d后改为阿司匹林长期单抗治疗。随访3个月,有效性指标 为3个月时NIHSS 0～1分、Barthel指数（Barthel index,BI）95～100分和mRS 0～1分的比例,3个月时缺 血性卒中的复发率;安全性指标为治疗24 h出血转化和症状性出血转化的发生率。另外比较三组间 基线和3个月时血清hs-CRP和IL-6的水平差异。 结果 研究共纳入85例患者,对照组28例,溶栓+单抗组28例,溶栓+双抗组29例,全部患者均完 成3个月随访,无死亡患者。对照组、溶栓+单抗组和溶栓+双抗组3个月随访时NIHSS 0～1分比例分 别为46.43%、78.57%和93.10%,BI 95～100分比例分别为53.57%、82.14%和89.66%,mRS 0～1分 的比例分别为50.00%、82.14%和93.10%,三组上述有效性指标差异均有统计学意义,两两比较显 示,溶栓+双抗组高于溶栓+单抗组和对照组,溶栓+单抗组高于对照组,差异均有统计学意义;对 照组、溶栓+单抗组和溶栓+双抗组3个月时缺血性卒中复发率分别为32.14%、7.14%和3.45%,差异 有统计学意义。安全性指标方面,三组均无出血转化事件。对照组、溶栓+单抗组和溶栓+双抗组3 个月时的hs-CRP水平分别为11.92±3.58 mg/L、9.04±2.85 mg/L和6.04±2.65 mg/L,IL-6水平分别为 26.18±4.65 ng/L、16.11±6.93 ng/L和12.84±2.57 ng/L,三组上述炎症因子水平差异均有统计学意 义,其中溶栓+双抗组低于溶栓+单抗组和对照组,溶栓+单抗组低于对照组。 结论 对于急性轻型缺血性卒中患者,rt-PA静脉溶栓治疗后短期双重抗血小板治疗可显著改善患 者神经功能,降低炎症因子水平,降低复发率,且不增加出血风险。     

血浆甘油三酯水平与缺血性卒中出院结局关系研究

目的   探讨急性缺血性卒中患者入院时血浆甘油三酯（triglyceride,TG）水平与出院结局不良的关系。 方法  采用回顾性队列研究的方法,连续纳入内蒙古兴安盟人民医院2009年6月1日～2012年5月31日急性缺血性卒中患者,共计3351例。结局不良组定义为患者出院时改良Rankin量表（modified Rankin Scale,mRS）评分≥3分,对结局不良组和结局良好组患者间基线资料进行比较。用四分位数法将患者入院时血浆TG水平分为4组,用非条件Logistic回归分析入院时TG水平与急性缺血性卒中出院结局不良的关系,计算比值比（odds ratio,OR）及95%可信区间（confidence interval,CI）。 结果  研究对象中发生结局不良的共341例,发生率为10.2%。单因素非条件Logistic回归分析结果显示,TG相对最高分位数组（TG＞2.12?mmol/L）,第1、2、3分位数组（TG分别为≤1.06?mmol/L、1.06～1.46?mmol/L、1.46～2.12?mmol/L）的结局不良发生率差异有显著性（P＜0.001）。在调整了年龄、住院天数、发病到入院时间、缺血性卒中首发、吸烟、饮酒、心脏病史、心房颤动史、高血压、高血糖和心率后,相对于最高分位数组,第3分位数组的结局不良发生率差异无显著性（P=0.0758）,而第1、2分位数组结局不良发生率升高（均P＜0.0001）,其OR（95%CI）分别为11.883（1.307～2.714）和2.063（1.436～2.963）。 结论  急性缺血性卒中患者入院时低水平TG可能独立地增加出院结局不良的风险。