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.
目的 探討可逆性胼胝體壓部病變的臨床特征和致病機制。 方法 總結Pubmed和Springer數據庫中2000年1月1日－2011年8月1日報道的年齡＞6歲的可逆性胼胝體壓部病變患者的臨床特征，分析該病可能的致病機制。 結果 14例患者中男7例，女7例；年齡（27.4 ± 15.6）歲，最小7歲，最大58歲；病因為發熱、疫苗接種、感染、癲癇發作、接受抗癲癇藥物或突然停用抗癲癇藥治療、接受四環素或氟尿嘧啶治療、營養不良、慢性酒精消耗，臨床癥狀出現率50%，為視幻覺、錯覺、定向功能障礙、意識模糊、嗜睡、共濟失調步態、急性尿潴留；可逆性胼胝體病變持續時間為（20.6 ± 14.5） d，最短2 d，最長50 d，影像學特征是T1加權成像低或等信號，T2加權成像、彌散加權成像高信號，表觀彌散系數成像低信號，水抑制成像常無明顯結構或信號異常發現，T1-釓對比劑增強成像無病灶強化。 結論 可逆性胼胝體壓部病變病因多樣，臨床表現復雜，致病機制可能主要為低鈉血癥、低血糖、精氨酸血管加壓素功能紊亂、感染或藥物毒性等導致胼胝體壓部細胞毒性水腫。
【摘要】 目的 探討佛波酯激活的蛋白激酶C與扭轉蛋白A在亞細胞成分中的表達之間的關系。 方法 采用免疫熒光法觀察扭轉蛋白A在原代培養的神經元和小鼠胚胎成纖維細胞（NIH 3T3細胞）中的分布。運用蛋白質印跡法分析蛋白激酶C和扭轉蛋白A在細亞細胞成分中的表達。 結果 扭轉蛋白A在NIH 3T3細胞中的表達類似于神經元。扭轉蛋白A在細胞質溶質、膜成分中均有分布。佛波酯活化蛋白激酶C后并不引起扭轉蛋白A在細胞質成分和膜成分中表達含量的變化。 結論 扭轉蛋白A可能是膜相關蛋白，細胞氧化應激中扭轉蛋白A表達上調和重分布變化不是由佛波酯誘導的蛋白激酶C活化途徑來實現的。鑒于扭轉蛋白A表達上調具有潛在的治療原發性早發扭轉性肌張力障礙的前景，影響其分布和表達的分子機制需要進一步研究。【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).