目的 探讨可逆性胼胝体压部病变的临床特征和致病机制。 方法 总结Pubmed和Springer数据库中2000年1月1日－2011年8月1日报道的年龄＞6岁的可逆性胼胝体压部病变患者的临床特征，分析该病可能的致病机制。 结果 14例患者中男7例，女7例；年龄（27.4 ± 15.6）岁，最小7岁，最大58岁；病因为发热、疫苗接种、感染、癫痫发作、接受抗癫痫药物或突然停用抗癫痫药治疗、接受四环素或氟尿嘧啶治疗、营养不良、慢性酒精消耗，临床症状出现率50%，为视幻觉、错觉、定向功能障碍、意识模糊、嗜睡、共济失调步态、急性尿潴留；可逆性胼胝体病变持续时间为（20.6 ± 14.5） d，最短2 d，最长50 d，影像学特征是T1加权成像低或等信号，T2加权成像、弥散加权成像高信号，表观弥散系数成像低信号，水抑制成像常无明显结构或信号异常发现，T1-钆对比剂增强成像无病灶强化。 结论 可逆性胼胝体压部病变病因多样，临床表现复杂，致病机制可能主要为低钠血症、低血糖、精氨酸血管加压素功能紊乱、感染或药物毒性等导致胼胝体压部细胞毒性水肿。
ObjectiveTo evaluate the magnetic resonance imaging (MRI) manifestations, pathological and clinical characteristics, and treatment outcomes of multiple sclerosis (MS) patients with new lesion in medulla oblongata (MO).MethodsPubMed, EBSCO, and Springer databases between January 1st, 2000 and May 1st, 2018 were searched with the combined keywords of " multiple sclerosis” and " medulla oblongata”. Furthermore, the MS patients’ MRI manifestations, pathological and clinical characteristics, and treatment outcomes were summarized.ResultsA total of 18 papers were involved, in which 26 patients were included. The lesions in MO were mainly showed by wedge-shape (9/20), and round or oval-shape (9/20) in axial head MRI. Inflammatory cells infiltration and acute demyelination in the new lesions of MO had been displayed by autopsy reports of two MS patients. The new lesions in MO mainly referred to various types of nystagmus (9/26), left ventricular apical ballooning syndrome (LVABS) (8/26), neurogenic pulmonary edema (NPE) (6/26), and acute heart failure (6/26). Nucleus tracts solitaries (NTS), along with dorsal motor nucleus of the vagus nerve and medial reticular formation (MRF), was related to LVABS and NPE. Intercalatus nucleus, Roller nucleus and/or autonomic nerve structure were related to various types of nystagmus.ConclusionsIn axial head MRI, the new MS lesions in MO were mainly wedge-shape and round or oval-shape. Beyond that, the new MS lesions in MO could involve NTS, dorsal motor nucleus of the vagus nerve, MRF, intercalatus nucleus, Roller nucleus and/or autonomic nerve structure, resulting in special clinical features, such as, nystagmus, LVABS, NPE, and acute heart failure. Corticosteroid is still the main treatment to relieve the clinical manifestations caused by new MS lesions in MO.
【摘要】 目的 探讨佛波酯激活的蛋白激酶C与扭转蛋白A在亚细胞成分中的表达之间的关系。 方法 采用免疫荧光法观察扭转蛋白A在原代培养的神经元和小鼠胚胎成纤维细胞（NIH 3T3细胞）中的分布。运用蛋白质印迹法分析蛋白激酶C和扭转蛋白A在细亚细胞成分中的表达。 结果 扭转蛋白A在NIH 3T3细胞中的表达类似于神经元。扭转蛋白A在细胞质溶质、膜成分中均有分布。佛波酯活化蛋白激酶C后并不引起扭转蛋白A在细胞质成分和膜成分中表达含量的变化。 结论 扭转蛋白A可能是膜相关蛋白，细胞氧化应激中扭转蛋白A表达上调和重分布变化不是由佛波酯诱导的蛋白激酶C活化途径来实现的。鉴于扭转蛋白A表达上调具有潜在的治疗原发性早发扭转性肌张力障碍的前景，影响其分布和表达的分子机制需要进一步研究。【Abstract】 Objective To investigate the relationship between the phorbol 12-myristate 13-acetate (PMA) activated protein kinase C (PKC) and the subcellular expression of TorsinA protein. Methods The expression of TorsinA in the primary cultured neurons and the NIH 3T3 cells was detected by immunofluorescence. The expression of PKC and TorsinA in subcellular fraction was analyzed by the western blotting. Results The expression pattern of TorsinA in NIH 3T3 cells was similar to neuron. PMA, an activator of PKC, did not promote the up-expression of TorsinA or redistribution in the subcellular fraction of NIH 3T3 cells. Conclusions TorsinA may be a membrane-associated protein. The up-regulation and redistribution of TorsinA is not caused by the pathway of the PMA activating PKC after cells insulted by oxidative stress. We should pay more attention on the mechanisms of the expression of TorsinA protein for the potential therapies to early-onset primary torsion dystonia (DYT1).