1,2-二氯乙烷中毒性脑白质病7例报告

目的 报告7例1,2-二氯乙烷(1,2-DCE)中毒性脑白质病的临床及影像学特征.方法 回顾性分析经广东省职业病防治院确诊的7例1,2-二氯乙烷中毒性脑白质病患者的临床资料.结果 患者中男3例、女4例,年龄17～46岁.为散发性发病,提示发病存在个体易感因素.主要临床表现包括头痛、呕吐、意识改变、癫痫发作等,3例仅有头痛,经CT和/或MR检查诊断脑白质病而后确诊.腰穿检查脑脊液压力增高,头颅CT均表现为两侧大脑半球脑白质对称性"佛指状"密度减低、侧脑室变窄、脑回肿胀和脑沟变浅.头颅MRI均表现两侧大脑半球白质广泛受累,T1WI呈略低信号,T2WI明显高信号,FLAIR亦为高信号,脑回肿胀,脑沟模糊不清,脑沟、脑池变浅,脑室变窄.经脱水降颅压及糖皮质激素等治疗,5例患者症状好转,2例死于脑疝.结论 1,2-二氯乙烷中毒性脑白质病,临床表现以颅高压表现为主,头颅MRI检查T2WI及FLAIR上双侧大脑半球白质高信号是主要的影像学特征,结合接触史调查可做出诊断.治疗以脱水降低颅内压及糖皮质激素为主.     

血管紧张素1-7在中枢神经系统中的研究进展

肾素-血管紧张素系统(RAS)是动脉血压和水电解质平衡的重要生理调节系统。目前已经证明脑组织拥有自己的RAS,并且RAS所有成分在中枢神经系统(CNS)中均有相应的表达。RAS产生具有生物学活性的血管紧张素肽家族,包括血管紧张素1-8(angiotensin-Ⅱ,Ang-Ⅱ)、血管紧张素3-8(angiotensin-Ⅳ,Ang-Ⅳ)和血管紧张素1-7[angiotensin-(1-7),Ang-(1-7)]。其中Ang-(1-7)对于血管主要作为一种舒张血管和抗细胞增殖类激素。越来越多证据表明Ang-(1-7)可能是CNS中重要的血管紧张素肽段,它能引起颅内动脉内皮依赖性的舒张反应。Ang-(1-7)作为一种天然舒血管类激素,可能有对抗Ang-Ⅱ血液动力学效用功能。     

血清Lp-PLA2,BDNF及Ang1-7表达水平与血管性痴呆患者病情严重程度的相关性

目的 探讨血管性痴呆(VD)患者血清脂蛋白相关磷脂酶A2(Lp-PLA2)、脑源性神经营养因子(BDNF)及血管紧张素1-7(Ang1-7)表达水平及其与VD患者病情严重程度的相关性。方法 选取本院2017年10月-2019年10月收治的120例VD患者,依照简易精神状态检查量表(MMSE)分为轻度组、中度组和重度组,各40例,比较各组相关危险因素,颈动脉超声检测颈动脉内膜中层厚度(IMT),检测血清生化指标[低密度脂蛋白胆固醇(LDL-C)、高密度脂蛋白胆固醇(HDL-C)、总胆固醇(TC)、三酰甘油(TG)、糖化血红蛋白(GHb)、空腹血糖(GLU)]、血清指标[Lp-PLA2,BDNF,Ang1-7、神经元特异性烯醇化酶(NSE)、C反应蛋白(CRP)、白细胞介素6(IL-6)、肿瘤坏死因子α(TNF-α)、同型半胱氨酸(Hcy)]水平, 分析Lp-PLA2,BDNF及Ang1-7水平之间的相关性,也分析血清Lp-PLA2,BDNF及Ang1-7水平与MMSE评分的相关性。结果 3组患者年龄、受教育程度、高血压病史、高血脂症史、饮酒、吸烟等临床资料比较均无明显差异(P>0.05),冠心病史、糖尿病史、IMT比较均有明显差异(P<0.05); 3组患者血清LDL-C,HDL-C,TC,TG,GHb,GLU水平比较均无明显差异(P>0.05),血清Lp-PLA2,BDNF,Ang1-7,NSE,CRP,IL-6,TNF-α及Hcy水平比较均有明显差异(P<0.05)。相关性分析显示,血清Lp-PLA2,BDNF及Ang1-7与冠心病史、糖尿病史、血清NSE,CRP,IL-6,TNF-α,LDL-C,TC水平及MMSE评分存在明显相关性。结论 血清Lp-PLA2、BDNF及Ang1-7水平与血管性痴呆患者血清因子水平及VD患者的病情严重程度存在明显相关性,检测血清Lp-PLA2,BDNF及Ang1-7水平有助于VD患者的预防和治疗     

基质金属蛋白酶-2、-7、-9在人脑星形细胞瘤中的表达及临床意义

目的 探讨基质金属蛋白酶(matrix metalloproteinase，MMP)在人脑星形细胞瘤侵袭、转移中的作用。方法 用免疫组织化学方法(S-P法)检测了45例人脑星形细胞瘤组织中MMP-2、MMP-7、MMP-9的表达情况。结果 Ⅲ、Ⅳ级中MMP-2、MMP-9蛋白表达要明显高于I、Ⅱ级，而MMP-7在部分Ⅳ级星形细胞瘤中过度表达，而在T、Ⅱ、Ⅲ级中无表达。结论 MMP-2、MMP-7、MMP-9蛋白的表达与星形细胞瘤的恶性程度有关，其高表达可能与星形细胞瘤的侵袭、转移等恶性生物学行为有关，并有可能成为星形细胞瘤恶性程度、侵袭能力的判断指标。     

Spinocerebellar Ataxia Type 7: Clinical Course, Phenotype–Genotype Correlations, and Neuropathology

Spinocerebellar ataxia type 7 is a neurodegenerative polyglutamine disease characterized by ataxia and retinal degeneration. The longitudinal course is unknown, and relationships between repeat expansion, clinical manifestations, and neuropathology remain uncertain. We followed 16 affected individuals of a 61-member kindred over 27 years with electroretinograms, neurological examinations including the Brief Ataxia Rating Scale, neuroimaging in five, and autopsy in four cases. We identified four stages of the illness: Stage 0, gene-positive but phenotypically silent; Stage 1, no symptoms, but hyperreflexia and/or abnormal electroretinograms; Stage 2, symptoms and signs progress modestly; and Stage 3, rapid clinical progression. CAG repeat length correlated inversely with age of onset of visual or motor signs (r?=??0.74, p?=?0.002). Stage 3 rate of progression did not differ between cases (p?=?0.18). Electroretinograms correlated with Brief Ataxia Rating Scale score and were a biomarker of disease onset and progression. All symptomatic patients developed gait ataxia, extremity dysmetria, dysarthria, dysrhythmia, and oculomotor abnormalities. Funduscopy revealed pale optic discs and pigmentary disturbances. Visual acuity declined to blindness in those with longer CAG expansions. Hyperreflexia was present from Stage 1 onwards. Restless legs syndrome and sensory impairment were common. Neuropathological hallmarks were neuronal loss in cerebellar cortex, deep cerebellar nuclei, inferior olive, and anterior horns of the spinal cord, and axonal loss in spinocerebellar tracts, dorsal nerve roots, and posterior columns. Retinal pathology included photoreceptor degeneration and disruption of retinal pigment epithelium. Spinocerebellar ataxia type 7 evolves through four clinical stages; neuropathological findings underlie the clinical presentation; electroretinograms are a potential biomarker of disease progression.     

脑积水脑室-腹腔分流术后脑室内出血7例处理分析

目的 总结脑积水脑室-腹腔分流术（VPS）后脑室内出血（IVH）的处理经验。方法 回顾性分析2015年9月1日至2018年9月1日VPS治疗的266例脑积水的临床资料。结果 266例中，7例VPS后发生IVH。采用脑室外引流术治疗2例，分流管调整术治疗1例，腰大池引流术3例，尿激酶局部注射1例；治疗后脑室内积血清除，脑室腹腔分流管均通畅。结论 IVH是脑积水VPS后常见并发症，造成IVH的原因除病人本身因素以外，手术是重要因素之一。对于VPS后IVH，可根据术后病人情况，灵活选择治疗方式     

成人人类疱疹病毒-7型相关脑炎1例报告

<正>人类疱疹病毒7型(human herpes virus-7,HHV-7)是1990年被Frankel等在健康人外周血单个核细胞的T细胞中分离出的一种双链DNA嗜淋巴病毒,属于疱疹病毒亚科^[1]。原发感染主要发生在免疫功能正常的5岁以下儿童,感染后可无症状或出现轻微及非特异性症状,给予抗病毒或对症治疗预后相对较好。成人HHV-7型相关脑炎罕见,特别是免疫功能正常的HHV-7型相关脑炎的临床表现、流行病学特点及发病机制仍有待研究。     

新型冠状病毒相关吉兰-巴雷综合征7例临床分析

目的 探讨新型冠状病毒（severe acute respiratory syndromes coronavirus 2,SARS-CoV-2）相关吉兰-巴雷综合征（Guillain-Barre syndrome,GBS）的临床特征、辅助检查及治疗，以提高对该病的认识。方法 纳入2022年12月至2023年2月在河北医科大学第二医院神经内科确诊的7例SARS-CoV-2相关GBS患者，查阅病历收集其临床特征、辅助检查及治疗资料，进行回顾性分析。结果 7例SARS-CoV-2相关GBS患者，男6例，女1例，年龄31～73岁，平均（57.4±16.2）岁，以肢体运动功能障碍或肢体运动感觉功能障碍伴受累肢体反射减弱或消失为主要临床特征，均伴脑脊液蛋白细胞分离现象，使用免疫调节治疗效果好。结论 SARS-CoV-2相关GBS和经典GBS的临床表现、病程、对治疗的反应相似。     

Expression of α-, β-, and γ-synuclein in glial tumors and medulloblastomas

alpha-, beta- and gamma-synuclein are highly homologous proteins that are found predominantly in neurons. Abnormal accumulation of synucleins has been associated with diseases of the central nervous system particularly Parkinson's disease. Immunoreactivity of alpha-synuclein is demonstrated in brain tumors with neuronal differentiation and in schwannomas, whereas gamma-synuclein has been demonstrated in breast and ovarian carcinomas. The immunoreactivity of synucleins has not been described in glial tumors. Immunoreactivity of synucleins in glial cells in culture and in pathological conditions, however, suggests that synucleins may be expressed by glial tumors. We studied the expression of alpha-, beta-, and gamma-synuclein in 84 human brain tumors (24 ependymomas, 31 astrocytomas, 8 oligodendrogliomas, and 21 medulloblastomas) by immunohistochemistry. Our study demonstrates immunoreactivity for gamma-synuclein in high-grade glial tumors; immunoreactivity is found in all anaplastic ependymomas but in only 33% of ependymomas and 16% of myxopapillary ependymomas. Immunoreactivity for gamma-synuclein is noted in 63% of glioblastomas but not in other astrocytic tumors. Of medulloblastomas, 76% have immunoreactivity for either alpha- or beta-synuclein or both; no immunoreactivity for gamma-synuclein is seen in medulloblastomas.     

Cholinergic receptor,nicotinic, alpha 7 as a target molecule of Arctic mutant amyloid β

正Alzheimer’s disease(AD)is a progressive cognitive disorder that develops predominantly in elderly patients and is characterized by cognitive impairments affecting memory,learning,and attention(Selkoe,2002).As the prevalence of AD is increasing concurrently with an increase in the aging