可逆性后部白质脑病综合征（RPLS）137例临床诊疗分析

发布时间：2018-06-24 05:28

本文选题：可逆性后部白质脑病综合征 + 神经影像学　；参考：《吉林大学》2014年硕士论文

【摘要】：目的：可逆性后部白质脑病综合征（reversible posteriorleukoencephalopathy syndrome, RPLS）是一组多病因的临床及影像学综合征，其与多种临床医学（神经病学、影像学、妇产学、肿瘤免疫学、器官移植学等）及基础医学关系密切。随着临床医师对该病临床经验的累积和影像学的不断进步，对其发病机理、临床症状、神经影像学特点、诊断和治疗也有了新的发现和了解。该病病因繁多、临床-影像学特征复杂、涉及医学学科较多，临床和影像医师对其缺乏全面了解、易漏诊误诊，从而延误治疗时机。对临床病例的发病率、病因、发病机制、检查手段、诊断标准、治疗预后等临床试验进行探讨，提高对RPLS的诊治和科研水平十分必要。方法：通过对中国学术文献总库（CNKI）、维普中文期刊全文数据库、相关期刊论文、中国生物医学数据库2008年9月到2014年1月的有关RPLS文章进行检索，共46篇，去除综述7篇、会议记录1篇、临床资料不全2篇、重复文章3篇，共126例RPLS患者，同时结合在吉林大学第二医院诊治的11例病例，对137例RPLS患者的临床资料进行统计和分析。结果：本组研究共137例患者，男性36例（27.28%）、女性101例（73.12%），男女比例为1:2.81。发病年龄6~68岁，平均30.43岁。儿童11例（8.02%），，成人126例（91.98%），儿童成人比例为1：11.5。106例急性起病（＜7天），21例亚急性起病（7~30天）。继发于子痫前期/子痫58例，高血压脑病32例，另外还有其他病因，如系统性红斑狼疮、尿毒症、急性肾炎、肾病综合征、服用免疫抑制剂（激素联合环磷酰胺、平顺铂、平阳霉素等）、血液系统疾病化疗后、原发性癫痫、硬皮病、SLE口服激素治疗后血小板减少、肾动脉狭窄、小脑后动脉畸形、肾脏移植等。颅高压症状、痫性发作、意识障碍、视觉异常为常见的临床症状，部分患者还有限局性神经系统症状。经典的影像学特征是病灶多呈对称性、以后循环白质异常信号为主的血管源性水肿。但也可累及脑干、脑室旁、胼胝体、内囊后肢、皮质等不典型部位。CT显示为早期未见异常或低密度病灶；行MRI检查的患者病灶在TI加权成像上呈低或等信号，在T2加权像和FlAIR图像上呈高信号。加做DW1和ADC图像的患者中，DWI像呈略低或等信号，少数DW1像呈稍高信号；而ADC呈高信号。积极治疗后症状及体征恢复至发病前的共135例，复查头部CT或者MRI病灶完全消失或大部分消失。随访3月无临床症状。其中1例治疗不当加重，复查头部MRI形成脑梗塞；1例因脑疝死亡。结论：RPLS是一组复杂的综合征，急性或亚急性起病，发病时大多数患者有血压升高。临床症状主要包括颅高压症状、视觉障碍、痫性发作、意识障碍和精神异常、限局性神经系统症状。女性发病率较男性高，儿童和成年人均可发病。其病因多种多样，且不断被发现，国内患者病因与国外不同，最常见的为子痫/先兆子痫和高血压脑病。病变经典的分布为以大脑后部白质为主的、双侧对称性的、弥漫性的血管源性水肿。但其也可合并或单独出现不典型部位病变，如脑干、基底节、胼胝体、脑室旁、皮层等。信号特点病灶在CT上可呈低密度影或正常；MRI检查：T1WI为低信号或等信号；T2WI为高信号；FLAIR呈高信号；DWI多呈低信号或等信号，少数也可呈稍高信号；ADC呈高信号。结合复习近期文献资料建议采取危险因素+临床表现（治疗前后）+影像学模式（治疗前后MRI）诊断RPLS，指导临床规范诊断。强调早诊断、早治疗，以避免脑组织不可逆性损伤。
[Abstract]:Objective: reversible posteriorleukoencephalopathy syndrome (RPLS) is a group of multiple causes of clinical and imaging syndrome, which is closely related to a variety of clinical medicine (Neurology, imaging, obstetrics, tumor immunology, organ transplantation) and basic medicine. With clinicians on the disease The accumulation of clinical experience and the continuous progress of imaging studies also have new discoveries and understanding of its pathogenesis, clinical symptoms, neuroimaging features, diagnosis and treatment. The etiology of the disease is numerous, the clinical imaging features are complex, many medical subjects are involved, and the clinical and imaging doctors lack a comprehensive understanding of it, and the diagnosis is easy to be misdiagnosed and thus delayed treatment. To improve the diagnosis and treatment of RPLS, it is necessary to explore the clinical trials of the incidence, etiology, pathogenesis, means of examination, diagnostic criteria, and the prognosis of the clinical cases.
Methods: CNKI, full text database of Chinese academic literature (CNKI), full text database of Chinese Journal of VIP, Chinese journal full text database, Chinese biomedical database from September 2008 to January 2014 were retrieved, with a total of 46 articles, 7 articles were removed, 1 were recorded, 2 clinical data were incomplete, 3 articles were repeated, and 126 RPLS patients, Meanwhile, combined with 11 cases diagnosed and treated in the second hospital of Jilin University, the clinical data of 137 patients with RPLS were analyzed statistically.
Results: in this study, there were 137 patients, 36 males (27.28%) and 101 women (73.12%). The male and female age were 1:2.81. age 6~68, average 30.43 years, 11 children (8.02%) and 126 adults (91.98%). The proportion of children and adults was 1:11.5.106 cases (< 7 days) and subacute onset (7~30 days), secondary to pre eclampsia / eclampsia. 32 cases of hypertensive encephalopathy, and other causes, such as systemic lupus erythematosus, uremia, acute nephritis, nephrotic syndrome, use of immunosuppressive agents (hormone combined cyclophosphamide, smooth platinum, Pingyang mycin, etc.), after chemotherapy of the blood system disease, primary epilepsy, scleroderma, SLE oral hormone treatment after thrombocytopenia, renal artery stenosis, small The malformation of the posterior cerebral artery, the kidney transplantation, the symptoms of craniofacial, epileptic seizures, the disturbance of consciousness, the visual abnormalities are common clinical symptoms, and some of the patients also have the symptoms of the sexual nervous system in the limited Bureau. The classic imaging features are that the focus is mostly symmetrical, and the abnormal signal of the circulating white matter is the main angiogenic edema. The atypical sites, such as the corpus callosum, the posterior limb of the inner capsule, and the cortex, showed no abnormal or low density lesions at the early stage, and the MRI examination showed a low or equal signal on the TI weighted imaging, and a high signal on the T2 weighted image and the FlAIR image. In the patients with the DW1 and ADC images, the DWI images were slightly lower or equal signals, and a few DW1 images were slightly higher signals. And ADC showed high signal. The symptoms and signs of the patients recovered to 135 cases before the onset of the disease. The reexamination of the head CT or MRI lesions disappeared completely or most. No clinical symptoms were observed in the follow-up period in March. Among them, 1 cases were treated unproperly, the head MRI formed cerebral infarction, and 1 cases died of cerebral hernia.
Conclusion: RPLS is a complex syndrome with acute or subacute onset. Most patients have elevated blood pressure during the onset of the disease. Clinical symptoms mainly include craniofacial symptoms, visual disorders, epileptic seizures, disturbance of consciousness and psychosis, and limited local nervous system symptoms. The incidence of women is higher than that of men. The etiology of domestic patients is different from that of foreign countries. The most common types are eclampsia / preeclampsia and hypertensive encephalopathy. The classical distribution is mainly the white matter in the posterior part of the brain, bilateral symmetrical and diffuse angioedema. But it can also be combined or alone with atypical lesions, such as the brainstem, basal ganglia, and callus. The signal characteristic focus on CT can be low density shadow or normal; MRI examination: T1WI is low signal or equal signal; T2WI is high signal; FLAIR is high signal; DWI is low signal or equal signal, and a few also show slightly high signal; ADC is high signal. Combined with review of recent literature, risk factors suggest taking risk factors + + Clinical manifestation (before and after treatment) + imaging model (MRI before and after treatment) diagnosis of RPLS, guiding clinical standard diagnosis, emphasizing early diagnosis and early treatment in order to avoid irreversible brain damage.
【学位授予单位】：吉林大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R742

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