本文選題：同心圓硬化 + 臨床特點��； 參考：《安徽醫(yī)科大學》2014年碩士論文

【摘要】：背景與目的同心圓硬化（Baló,s concentric sclerosis，BCS）是一種少見的中樞神經(jīng)系統(tǒng)脫髓鞘性疾病，具有特征性病理改變，即病變區(qū)域髓鞘脫失帶和髓鞘相對保留帶相間排列形成明暗相間的條紋。BCS病因和臨床表現(xiàn)復(fù)雜，既往本病較難診斷，主要依靠腦組織活檢和尸檢確診，隨著核磁共振成像（Magnetic ResonanceImaging, MRI）技術(shù)的發(fā)展，越來越多的病例被診斷。此外，有研究認為BCS預(yù)后與是否得到早期積極有效治療相關(guān)，因此BCS的早期診斷、早期治療至關(guān)重要。本研究旨在結(jié)合既往文獻報道，探討B(tài)CS臨床表現(xiàn)、影像學特征和病理改變的一般特征及部分少見的不典型表現(xiàn)，以期減少臨床誤診、漏診，改善BCS患者臨床預(yù)后。 方法回顧性分析本院2009年1月~2012年12月收治的經(jīng)頭顱MRI檢查證實和（或）核磁共振引導下腦組織活檢病理學檢查確診的6例BCS患者，調(diào)閱電子病歷，記錄研究對象的一般情況，如性別、年齡，聯(lián)系電話等；此次發(fā)病的起病形式，病前有無可能相關(guān)誘因，臨床癥狀、體征和既往病史；實驗室檢查如血常規(guī)、生化，甲狀腺功能組合、多腫瘤標志物的12項檢查（C12）及腦脊液（CerebrospinalFluid, CSF）常規(guī)、生化、寡克隆區(qū)帶（Oligoclonal Bands, OB）；調(diào)閱并拷貝入組病例影像學資料，由經(jīng)驗豐富的神經(jīng)內(nèi)科和影像科主任醫(yī)師分別閱片，明確每例患者的影像學改變，包括頭顱CT（Computer Tomography）平掃病灶的部位，密度改變、有無水腫、鄰近腦組織結(jié)構(gòu)受累情況，頭顱MRI平掃病灶的部位，信號特征，鄰近腦組織結(jié)構(gòu)受累情況，增強掃描有無強化及強化程度和強化方式，病灶區(qū)域磁共振波譜分析（magnetic resonance spectroscopy，1H-MRS）；病理科調(diào)閱相關(guān)病例腦組織病理切片，由經(jīng)驗豐富的神經(jīng)內(nèi)科和病理科主任醫(yī)師分別閱片，明確每例患者組織病理學改變；記錄所有患者住院期間接受的治療方案及療效；結(jié)合既往文獻報道，從BCS病因、誘因、起病形式、臨床表現(xiàn)、影像學特征、腦組織活檢病理學改變、治療方案、療效評價和預(yù)后等方面進行分析、探討，總結(jié)本病的一般特征和特殊臨床表現(xiàn)。 結(jié)果6例BCS患者中，男性4例，女性2例，年齡23~63歲，平均41.8歲�；颊叱始毙裕�4例）或亞急性（2例）起病。首發(fā)癥狀主要為偏側(cè)肢體無力（3例），其他常見臨床表現(xiàn)包括共濟失調(diào)（3例），智能減退（2例），精神淡漠、反應(yīng)遲鈍（2例），中樞性面舌癱（2例），病理征陽性（3例），時間、地點、人物定向障礙（1例）。頭顱MRI平掃除1例單發(fā)病灶外，其余均為多發(fā)病灶，發(fā)病初期病灶呈斑片樣或團塊狀，約2~3周后出現(xiàn)“煎雞蛋”樣雙重構(gòu)造改變（4例），2月后出現(xiàn)同心圓樣特征性改變（2例），增強掃描2例出現(xiàn)病灶周圍2-3層不等的層狀強化，2例患者出現(xiàn)病灶邊緣不完全環(huán)狀強化。3例行腦組織活檢病理檢查符合同心圓硬化改變。6例中5例經(jīng)大劑量腎上腺糖激素沖擊治療后患者臨床癥狀均有明顯好轉(zhuǎn)，MRI同心圓樣病灶逐漸消失。隨訪6~25個月（平均15個月），1例患者出現(xiàn)癲癇發(fā)作，予抗癲癇治療后控制，1例患者遺留有偏側(cè)肢體麻木，1例患者自覺解小便費力，余2例患者恢復(fù)良好。 結(jié)論BCS多急性和亞急性起病，多以不同程度精神癥狀、智能減退及運動障礙起病，臨床表現(xiàn)復(fù)雜。頭顱CT平掃無特異性表現(xiàn)，頭顱MRI檢查早期可出現(xiàn)“煎雞蛋”樣雙重構(gòu)造改變，后期可出現(xiàn)同心圓樣特征性改變，具有診斷價值，腦組織活檢病理學檢查是診斷金標準。早期診斷，并行正規(guī)激素沖擊治療，本病多數(shù)預(yù)后良好，復(fù)發(fā)率低，，呈良性臨床過程。
[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.
【學位授予單位】：安徽醫(yī)科大學
【學位級別】：碩士
【學位授予年份】：2014
【分類號】：R744.5

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