Self-Mutilation in Neurodegeneration with Brain Iron Accumulation

Neurodegeneration with brain iron accumulation (NBIA) is the term applied to a heterogeneous group of disorders resulting in iron deposition in the basal ganglia. Well-known phenotypic features are progressive regression with extra pyramidal involvement and a variable course. A 10-year-old child born to consanguineous parents presented with progressive generalized opisthotonic dystonia, retrocollis, oromandibular dyskinesias, apraxia for swallowing, optic atrophy and severe self-mutilation of lips. MR imaging showed brain iron accumulation. Other causes of self-mutilation were excluded. Early infantile onset, ophisthotonic dystonia with oromandibular dyskinesias and characteristic MR images are suggestive of NBIA. There is only one case reported in the literature of self-mutilation in this condition.     

中风毒邪论与神经保护治疗的研究

中风毒邪论是一种与传统中医中风病理有所不同的理论 ,在中风毒邪论指导下形成解毒通络方是较为理想的神经保护剂 ,可解决目前神经保护治疗的主要障碍 ,有望成为提高中医治疗中风急性期疗效的关键     

Cerebral primitive neuro-ectodermal tumour following treatment of a unilateral retinoblastoma

Summary Cerebral primitive neuro-ectodermal tumour (PNET) occurring as a second primary malignancy in childhood is exceedingly rare. We present a 7-year-old boy who developed a proven supratentorial PNET five years after enucleation and radio-/chemotherapy for a sporadic, unilateral retinoblastoma with optic nerve invasion. The association with this malignant eye disease as well as the effect of irradiation and multi-agent chemotherapy on second tumour induction are evaluated.     

Development of neuropeptide Y (NPY) immunoreactive neurons in the rat occipital cortex: a combined immunohistochemical-autoradiographic study

The postnatal development of neuropeptide Y (NPY)-immunoreactive neurons, previously labeled with [3H]thymidine on embryonic days E14-E21, has been studied in the rat occipital cortex. Immunohistochemistry combined with autoradiography showed evidence of a modified "inside-out" pattern of maturation. NPY-neurons are generated between E14 and E20 and are found in layers II-VI of the cortex and the subcortical white matter. NPY neurons from all these birthdates are overproduced at first, although cells generated at E16 produce the greatest excess, followed by E15 and E17. Some of these transient neurons are found in the "wrong" layer for their birthdates, and their elimination produces a more "correct" alignment at maturity. However, most of the NPY neurons that survive are generated at E17, and these cells are found throughout layers II-VI with a preponderance in layer VI. This evidence is strongly suggestive of cell death rather than merely cessation of production of NPY.     

内皮素—1在慢性低氧高二氧化碳大鼠支气管中的定位表达

目的：观察内皮素－1（ET－1）的慢性缺氧高二氧化碳大鼠支气管中的表达及分布,认识慢性缺高二氧化碳环境与ET－1表达、分布的关系。方法：用原位杂交技术,对慢性缺氧高二氧化碳大鼠支气管中的ET－1的表达进行定位研究,结果：ET－1mRNA在慢性缺氧高二氧化碳大鼠的支气管上皮细胞和平滑肌细胞中广泛表达,特别是在Clara细胞和社会上皮小体的游离面胞浆中的呈强阳性染色。结论：慢性缺氧高二氧化碳大鼠支气管中ET-1mRNA主要定位于Clara细胞和神经上皮小体的游离面胞浆。缺氧高二氧化碳可能是刺激ET－1在肺部合成及释放增加的主要原因。     

神经精神狼疮26例临床分析

目的 了解神经精神狼疮（ＮＰＬＥ）的临床特征及治疗转归。方法 回顾１９９２～１９９８年在本院风湿科诊治的２６例神经精神狼疮的临床资料。结果 ２６例ＮＰＬＥ患者５０％在起病１年内发病,有器质性脑病综合征１１例,脑血管意外２例,癫痫４例,狼疮性头痛２例,精神症状或精神病７例。１９例重症ＮＰＬＥ中,１２例给予甲基强的松龙冲击加免疫抑制剂治疗后１０例病情缓解,２例死亡;７例单用甲基强的松龙冲击治疗者４例缓     

氟桂利嗪和拉莫三嗪对胎鼠缺血缺氧性脑损害的保护作用

目的研究氟桂利嗪(FNZ)、拉莫三嗪(LTG)及两药联合经孕鼠给药,对胎鼠宫内缺血缺氧性脑损害的保护作用。方法将孕20 d Wistar大鼠40只,随机均分为FNZ组、LTG组、FNZ LTG组和对照组。前3组大鼠于脑缺血缺氧术前3 d开始灌胃给药,每天1次,分别为FNZ 0.5 mg/(kg.d),LTG 10 mg/(kg.d),FNZ 0.5 mg/(kg.d) LTG 10 mg/(kg.d)。第3天给药后3 h,每组取8只孕鼠制作宫内缺血缺氧模型,剩余2只孕鼠直接行剖宫产取胎鼠,每胎5只。于缺血缺氧后6、12、24、48 h分别取胎鼠血清0.5 ml,采用ELISA法测定血清神经元特异性烯醇化酶(NSE)和酸性钙结合蛋白(S-100)水平。结果胎鼠缺血缺氧后,血清NSE和S-100水平明显升高。FNZ组、LTG组及FNZ LTG组胎鼠缺血缺氧后各个时间点血清NSE和S-100较对照组明显降低,差异均有极显著性意义(均P<0.01)。联合用药组效果更明显,组间比较差异亦有极显著性意义(P<0.01)。结论产前预防性灌胃FNZ、LTG对胎鼠宫内缺血缺氧性脑损伤具有保护作用,联合用药效果更明显。