同心圆硬化临床及影像学特征

发布时间：2018-07-09 22:31

本文选题：同心圆硬化 + 临床特点　；参考：《安徽医科大学》2014年硕士论文

【摘要】：背景与目的同心圆硬化（Baló,s concentric sclerosis，BCS）是一种少见的中枢神经系统脱髓鞘性疾病，具有特征性病理改变，即病变区域髓鞘脱失带和髓鞘相对保留带相间排列形成明暗相间的条纹。BCS病因和临床表现复杂，既往本病较难诊断，主要依靠脑组织活检和尸检确诊，随着核磁共振成像（Magnetic ResonanceImaging, MRI）技术的发展，越来越多的病例被诊断。此外，有研究认为BCS预后与是否得到早期积极有效治疗相关，因此BCS的早期诊断、早期治疗至关重要。本研究旨在结合既往文献报道，探讨BCS临床表现、影像学特征和病理改变的一般特征及部分少见的不典型表现，以期减少临床误诊、漏诊，改善BCS患者临床预后。方法回顾性分析本院2009年1月~2012年12月收治的经头颅MRI检查证实和（或）核磁共振引导下脑组织活检病理学检查确诊的6例BCS患者，调阅电子病历，记录研究对象的一般情况，如性别、年龄，联系电话等；此次发病的起病形式，病前有无可能相关诱因，临床症状、体征和既往病史；实验室检查如血常规、生化，甲状腺功能组合、多肿瘤标志物的12项检查（C12）及脑脊液（CerebrospinalFluid, CSF）常规、生化、寡克隆区带（Oligoclonal Bands, OB）；调阅并拷贝入组病例影像学资料，由经验丰富的神经内科和影像科主任医师分别阅片，明确每例患者的影像学改变，包括头颅CT（Computer Tomography）平扫病灶的部位，密度改变、有无水肿、邻近脑组织结构受累情况，头颅MRI平扫病灶的部位，信号特征，邻近脑组织结构受累情况，增强扫描有无强化及强化程度和强化方式，病灶区域磁共振波谱分析（magnetic resonance spectroscopy，1H-MRS）；病理科调阅相关病例脑组织病理切片，由经验丰富的神经内科和病理科主任医师分别阅片，明确每例患者组织病理学改变；记录所有患者住院期间接受的治疗方案及疗效；结合既往文献报道，从BCS病因、诱因、起病形式、临床表现、影像学特征、脑组织活检病理学改变、治疗方案、疗效评价和预后等方面进行分析、探讨，总结本病的一般特征和特殊临床表现。结果6例BCS患者中，男性4例，女性2例，年龄23~63岁，平均41.8岁。患者呈急性（4例）或亚急性（2例）起病。首发症状主要为偏侧肢体无力（3例），其他常见临床表现包括共济失调（3例），智能减退（2例），精神淡漠、反应迟钝（2例），中枢性面舌瘫（2例），病理征阳性（3例），时间、地点、人物定向障碍（1例）。头颅MRI平扫除1例单发病灶外，其余均为多发病灶，发病初期病灶呈斑片样或团块状，约2~3周后出现“煎鸡蛋”样双重构造改变（4例），2月后出现同心圆样特征性改变（2例），增强扫描2例出现病灶周围2-3层不等的层状强化，2例患者出现病灶边缘不完全环状强化。3例行脑组织活检病理检查符合同心圆硬化改变。6例中5例经大剂量肾上腺糖激素冲击治疗后患者临床症状均有明显好转，MRI同心圆样病灶逐渐消失。随访6~25个月（平均15个月），1例患者出现癫痫发作，予抗癫痫治疗后控制，1例患者遗留有偏侧肢体麻木，1例患者自觉解小便费力，余2例患者恢复良好。结论BCS多急性和亚急性起病，多以不同程度精神症状、智能减退及运动障碍起病，临床表现复杂。头颅CT平扫无特异性表现，头颅MRI检查早期可出现“煎鸡蛋”样双重构造改变，后期可出现同心圆样特征性改变，具有诊断价值，脑组织活检病理学检查是诊断金标准。早期诊断，并行正规激素冲击治疗，本病多数预后良好，复发率低，，呈良性临床过程。
[Abstract]:Background and purpose Bal s concentric sclerosis (BCS) is a rare demyelinating disease of the central nervous system. It has characteristic pathological changes, that is, the pathogeny and the clinical manifestations of the stripe between the myelin sheath and the myelin sheath and the relative reserving band of the myelin sheath form the dark and dark phase, and the disease is difficult to diagnose in the past. More and more cases are diagnosed with the development of brain tissue biopsy and autopsy. With the development of Magnetic ResonanceImaging (MRI) technology, more and more cases are diagnosed. In addition, there are studies that the prognosis of BCS is related to the early active and effective treatment. Therefore, the early diagnosis and early treatment of BCS are very important. To study the clinical manifestations of BCS, the general features of imaging features and pathological changes and some rare atypical manifestations, in order to reduce the clinical misdiagnosis and missed diagnosis and improve the clinical prognosis of BCS patients.
Methods a retrospective analysis of 6 cases of BCS patients confirmed by MRI examination in January 2009 ~2012 and (or) MRI guided brain biopsy pathology examination confirmed by MRI and (or) MRI guided electronic medical records were used to record the general situation of the subjects, such as sex, age, telephone and so on. Related inducements, clinical symptoms, signs, and past medical history; laboratory tests such as blood routine, biochemistry, thyroid function combination, 12 examination (C12) and cerebrospinal fluid (CerebrospinalFluid, CSF) routine, biochemical, oligoclonal zone (Oligoclonal Bands, OB) of multiple tumor markers. The neurology department and the director of the imaging department read the film to clarify the imaging changes in each case, including the location of the head CT (Computer Tomography), the density change, the edema, the involvement of the adjacent brain tissue, the location of the head MRI, the signal features, the involvement of the adjacent brain tissue, and the enhanced scan. No strengthening and strengthening degree and strengthening method, magnetic resonance spectroscopy (magnetic resonance spectroscopy, 1H-MRS) in the focus area; pathological section read the pathological section of brain tissue in the related cases, read the film by the experienced neurology department and the chief physician of the pathology department respectively, to make clear the histopathological changes in each case; record all the patients in hospital. The treatment scheme and curative effect were received during the period, and combined with previous literature reports, the general characteristics and special clinical manifestations of BCS were analyzed from the causes, causes, causes, forms of onset, clinical manifestations, imaging features, pathological changes of brain biopsy, treatment plan, therapeutic evaluation and prognosis.
Results among the 6 BCS patients, there were 4 males and 2 females, with an average age of 41.8 years old. The patients were acute (4 cases) or subacute (2 cases). The first symptoms were mainly lateral limb weakness (3 cases), other common clinical manifestations including ataxia (3 cases), intelligent hypothyroidism (2 cases), mental indifference, slow reaction (2 cases), central facial palsy (2 cases), pathology, and pathology. Positive (3 cases), time, place, and character orientation disorder (1 cases). The head MRI was removed from 1 cases of single focus, and the rest were multiple lesions. The lesion was spotted or lump in the initial stage. After about 2~3 weeks, the "fried egg" double structural change (4 cases) appeared. After February, the concentric circular pattern was changed (2 cases), and the enhanced scan appeared in 2 cases. 2-3 layers of stratiform enhancement around 2-3 layers and 2 patients with incomplete circumferential enhancement on the edge of the lesion. The pathological examination of brain tissue biopsy was consistent with the change of concentric rounded sclerosis in the.6 cases, 5 cases of the patients were obviously improved after the high dose of adrenocorticosteroid shock treatment, and the MRI concentric circular lesion gradually disappeared. The follow-up of 6~25 months (mean 15 months) 1 patients had epileptic seizures, controlled after antiepileptic treatment, 1 patients left side limb numbness, 1 patients consciously solved urination, and the remaining 2 patients recovered well.
Conclusion BCS is multiple acute and subacute onset, with different degrees of mental symptoms, intelligent hypomania and dyskinesia. The clinical manifestations are complicated. CT plain scan of head has no specific manifestation. The double structural changes of "fried eggs" can appear in the early cranial MRI examination, and the characteristic changes of concentric circles can appear in the later period, with diagnostic value and biopsy of brain tissue. Pathological examination is the diagnostic standard of gold. Early diagnosis and regular hormonal shock therapy are most favorable for the prognosis of this disease. The recurrence rate is low, showing a benign clinical course.
【学位授予单位】：安徽医科大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R744.5

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