本文選題：線粒體腦肌病 + MELAS綜合征��； 參考：《中國全科醫(yī)學(xué)》2017年15期

【摘要】：背景線粒體腦肌病影像學(xué)診斷存在一定困難,目前尚缺乏針對該病疾病進(jìn)展中影像學(xué)動(dòng)態(tài)變化特點(diǎn)的研究。目的探討線粒體腦肌病,尤其是線粒體腦肌病伴高乳酸血癥和卒中樣發(fā)作(MELAS)綜合征的臨床特點(diǎn)與顱腦CT及MRI影像學(xué)動(dòng)態(tài)變化特點(diǎn)。方法回顧性分析溫州醫(yī)科大學(xué)附屬第一醫(yī)院2010—2015年確診的9例線粒體腦肌病患者共19次發(fā)作時(shí)的臨床特點(diǎn)及顱腦影像學(xué)表現(xiàn),動(dòng)態(tài)觀察病情演變過程中相應(yīng)病灶彌散加權(quán)成像(DWI)及表觀彌散系數(shù)(ADC)的變化等情況。結(jié)果 9例患者中5例確診為MELAS綜合征。9例患者以年輕男性(6例)為主,臨床主要表現(xiàn)為癇性發(fā)作、腦卒中樣發(fā)作、認(rèn)知功能減退、頭痛或頭暈、聽力或視力下降、明顯肌肉萎縮。9例患者共計(jì)23個(gè)病灶累及大腦各腦葉,顱腦CT檢查顯示病灶部位呈低密度改變,MRI檢查顯示病灶部位T1低信號(hào)T2高信號(hào)。病變初期以血管源性水腫為主,腦回部位DWI高信號(hào)ADC低信號(hào),鄰近皮質(zhì)下出現(xiàn)云絮狀高信號(hào)改變并持續(xù)存在,腦回部位DWI信號(hào)隨時(shí)間緩慢減低,而ADC隨之升高;病程中部分組織壞死,出現(xiàn)鄰近腦室擴(kuò)大或腦溝增寬、蜂窩狀液化等不可逆改變。4例CT檢查顯示雙側(cè)基底核區(qū)高密度影(鐵沉積/鈣化)。結(jié)論線粒體腦肌病臨床表現(xiàn)復(fù)雜多樣,以皮質(zhì)受累為主,顱腦CT檢查可發(fā)現(xiàn)鐵沉積/鈣化,而顱腦MRI檢查中病灶部位DWI和ADC變化能夠在一定程度上動(dòng)態(tài)反映線粒體腦肌病的疾病演變特點(diǎn)。
[Abstract]:Background there are some difficulties in imaging diagnosis of mitochondrial encephalomyopathy. Objective to investigate the clinical features of mitochondrial encephalomyopathy, especially mitochondrial encephalomyopathy with hyperlactic acidemia and stroke like attack syndrome (Mel ASS), and to investigate the dynamic changes of CT and MRI imaging in patients with mitochondrial encephalomyopathy. Methods the clinical features and craniocerebral imaging findings of 9 patients with mitochondrial encephalomyopathy diagnosed in the first affiliated Hospital of Wenzhou Medical University from 2010 to 2015 were retrospectively analyzed. The changes of diffusion weighted imaging (DWI) and apparent diffusion coefficient (ADCC) were observed dynamically. Results among the 9 cases, 5 cases were diagnosed as MELAS syndrome. The main clinical manifestations were epileptic seizure, stroke like attack, cognitive impairment, headache or dizziness, hearing loss or visual acuity, and the main clinical manifestations were epileptic seizure, stroke like attack, headache or dizziness, hearing or vision loss. There were 23 lesions involved in all cerebral lobes in 9 patients with obvious muscular atrophy. Brain CT showed low density changes in the lesions. MRI showed T 1 low signal T 2 high signal intensity. At the beginning of the lesion, vasogenic edema was dominant, DWI in gyrus was high signal ADC low signal, cloud flocculant high signal was appeared in adjacent cortex, DWI signal in gyrus decreased slowly with time, and ADC increased. In the course of the disease, some of the tissues were necrotic, the adjacent ventricles were enlarged or the sulcus widened, and the honeycomb liquefaction and other irreversible changes were found in 4 cases. Ct examination showed high density (iron deposition / calcification) in the bilateral basal nuclei. Conclusion the clinical manifestations of mitochondrial encephalomyopathy are complex and diverse, mainly involving the cortex, and the iron deposit / calcification can be found by brain CT examination. The changes of DWI and ADC in brain MRI can reflect the disease evolution of mitochondrial encephalomyopathy to some extent.
【作者單位】： 溫州醫(yī)科大學(xué)附屬第一醫(yī)院神經(jīng)內(nèi)科;溫州醫(yī)科大學(xué)附屬第一醫(yī)院影像科;
【分類號(hào)】：R445.2;R741

【相似文獻(xiàn)】

相關(guān)期刊論文 前10條

1 陳文輝;;1例線粒體腦肌病病人的治療與護(hù)理[J];護(hù)理研究;2010年29期

2 沈利平;管雪芬;歐小凌;熊小妮;;3例線粒體腦肌病的護(hù)理[J];當(dāng)代護(hù)士(學(xué)術(shù)版);2011年05期

3 周曾蓉;馮靈;;1例線粒體腦肌病患者的護(hù)理[J];護(hù)理實(shí)踐與研究;2013年01期

4 王穎莉;楊蓉;;線粒體腦肌病的護(hù)理體會(huì)一例[J];華西醫(yī)學(xué);2011年10期

5 趙麗華;況小紅;;1例線粒體腦肌病的觀察與護(hù)理[J];內(nèi)蒙古中醫(yī)藥;2011年03期

6 黃赤;;3例線粒體腦肌病的護(hù)理干預(yù)[J];大家健康(學(xué)術(shù)版);2014年14期

7 吳維平,耿榮,陳賢楠,吳滬生,呂丹云;以睡眠呼吸暫停為突出癥狀的線粒體腦肌病一例[J];中華兒科雜志;1998年12期

8 徐建國;;線粒體腦肌病患者的臨床診斷及神經(jīng)影像學(xué)分析[J];影像技術(shù);2014年02期

9 劉金平;王宏;孫艷;沈鉞;;一例睡眠呼吸機(jī)依賴線粒體腦肌病患者的護(hù)理[J];天津護(hù)理;2011年06期

10 袁哲星;劉文;蔡宗堯;肖朝勇;;線粒體腦肌病綜合影像表現(xiàn)[J];放射學(xué)實(shí)踐;2007年11期

相關(guān)會(huì)議論文 前1條

1 高力頻;;線粒體腦肌病的護(hù)理問題探討[A];第十一屆全國神經(jīng)病學(xué)學(xué)術(shù)會(huì)議論文匯編[C];2008年

，

本文編號(hào)：1960272