國人Marchiafava-Bignami病的臨床特點分析
發(fā)布時間：2018-12-31 17:11
【摘要】：目的：Marchiafava-Bignami病(Marchiafava-Bignami disease，MBD)是一種臨床罕見的慢性酒精中毒并發(fā)癥，以胼胝體脫髓鞘和壞死為主要病理特征。該病是于兩位病理學家Marchiafava和Bignami在1903年首先描述了3例生前曾長期飲用粗制紅酒的患者之后而得以命名。由于該病臨床表現(xiàn)缺乏特異性，以往確診該病需要依靠病理尸檢。近年來CT、MRI等影像學技術的發(fā)展使得MBD患者的活體診斷成為可能。目前MBD在世界范圍內所有種族及男女兩性當中均可發(fā)病。自張通等于1994年報道首例國人MBD患者后，國內病例報道日漸增多。本研究通過總結國人MBD患者的流行病學、臨床表現(xiàn)、影像學以及預后的特征，來提高對國人MBD患者臨床特點的認識。 方法：首先報道1例我院近年收治的MBD患者。同時，文獻檢索1994～2012年間中國知網、萬方全文數(shù)據(jù)庫、維普全文數(shù)據(jù)庫中的MBD相關中文文獻，篩選出資料完整的84例病例。最終納入國人MBD患者85例，收集并分析其下列臨床數(shù)據(jù)：人口學特征(包括性別、年齡)、臨床表現(xiàn)(包括發(fā)病時間、首發(fā)癥狀、病程中出現(xiàn)的神經病學征候)、既往史、個人史(包括嗜好)、常規(guī)實驗室指標、神經影像學特點及預后。 結果：在85例國人MBD病例中，男性有82例（96.5%），女性有3例（3.5%）；平均發(fā)病年齡為48.2歲；有82例具有大量飲酒史，占96.5%。77例(90.6%)急性、亞急性MBD患者臨床主要表現(xiàn)為意識障礙、癲癇伴錐體束征；8例(9.4%)慢性MBD患者則以認知損害表現(xiàn)為主。僅有2例(2.4%)有經典的半球間離斷綜合征表現(xiàn)。在急性和亞急性患者中，無或輕度意識障礙患者認知損害的發(fā)生率高于有較嚴重意識障礙(昏迷或昏睡)患者(97.5%VS.78.4%，，P0.05)。而有較嚴重意識障礙患者發(fā)病前后出現(xiàn)步態(tài)不穩(wěn)、癲癇發(fā)作和肌張力增高的發(fā)生率均明顯高于無或輕度意識障礙患者(P均0.05)。所有患者均經顱腦CT和/或MRI檢查明確診斷。MRI對于胼胝體病變范圍的檢出率明顯優(yōu)于CT（P0.05）。有74例患者具有完整的胼胝體影像資料，其中46例(62.2%)胼胝體全程受累，28例(37.8%)胼胝體部分受累。85例患者中有36例（42.4%）有胼胝體外部位受累。有無意識障礙與胼胝體全程受累或胼胝體外部位受累無明顯相關性(P均0.05)。在57例具有預后資料的患者中，有43例(75.4%)好轉；6例(10.5%)惡化；8例(14.0%)死亡，且均為急性和亞急性患者。有較嚴重意識障礙患者的死亡率要明顯高于無或輕度意識障礙患者(P0.05)。 結論：(1)國人MBD患者最常見于既往有大量飲酒史的中年男性；(2)大部分國人MBD患者總體預后良好，但急性期出現(xiàn)意識障礙者提示預后不良；(3)影像學早期識別與適當?shù)闹委熆赡苡兄诟纳芃BD患者的預后。
[Abstract]:Objective: Marchiafava-Bignami 's disease (Marchiafava-Bignami disease,MBD) is a rare complication of chronic alcoholism, with demyelination and necrosis of corpus callosum as the main pathological feature. The disease was named after two pathologists, Marchiafava and Bignami, first described three patients who had long consumed crude red wine in 1903. Due to the lack of specificity in clinical manifestations, the diagnosis of the disease in the past depends on pathological autopsy. In recent years, with the development of imaging techniques such as CT,MRI, it is possible to diagnose MBD patients in vivo. MBD is present in all races and sexes around the world. Since Zhang Tong reported the first case of MBD in 1994, the number of cases in China has been increasing. In this study, we summarized the epidemiology, clinical manifestations, imaging and prognosis of Chinese patients with MBD to improve the understanding of the clinical characteristics of Chinese patients with MBD. Methods: a case of MBD in our hospital in recent years was reported. At the same time, 84 cases with complete data were selected by searching the Chinese literature related to MBD from 1994 to 2012 in China Zhiwang, Wanfang Full-text Database and Weipu Full-text Database. The following clinical data were collected and analyzed: demographic characteristics (including sex, age), clinical manifestations (including onset time, initial symptoms, neurologic symptoms in the course of the disease), past history. Personal history (including hobbies), routine laboratory indicators, neuroimaging features and prognosis. Results: among 85 cases of MBD in Chinese, 82 cases were male (96.5%), 3 cases were female (3.5%), the average age was 48.2 years old. 82 cases had a history of heavy drinking, accounting for 96.5% (90.6%) of acute. The clinical manifestations of subacute MBD patients were mainly consciousness disorder, epilepsy with pyramidal beam sign. Cognitive impairment was predominant in 8 patients (9.4%) with chronic MBD. Only 2 cases (2.4%) had classic hemispheric disconnection syndrome. In both acute and subacute patients, the incidence of cognitive impairment in patients without or with mild consciousness disorders was higher than that in patients with more severe consciousness disorders (coma or drowsiness) (97.5VS.78.4% P0.05). However, the incidence of gait instability, seizure and muscle tension in patients with severe consciousness disorder was significantly higher than that in patients without or with mild consciousness disorder (P 0.05). All patients were diagnosed by craniocerebral CT and / or MRI. MRI was superior to CT in detecting the extent of corpus callosum (P0.05). The corpus callosum was involved in 46 cases (62.2%), the corpus callosum in 28 cases (37.8%) and the external position of corpus callosum in 36 cases (42.4%). There was no significant correlation between the involvement of corpus callosum and the external position of corpus callosum (P 0.05). Of the 57 patients with prognostic data, 43 (75.4%) improved, 6 (10.5%) deteriorated, and 8 (14.0%) died, all of them were acute and subacute. The mortality rate of patients with severe disturbance of consciousness was significantly higher than that of patients without or with mild disturbance of consciousness (P0.05). Conclusion: (1) Chinese patients with MBD are most common in middle-aged men who have a history of heavy drinking. (2) the majority of Chinese patients with MBD have a good prognosis, but the patients with acute disturbance of consciousness suggest poor prognosis. (3) early imaging identification and appropriate treatment may help to improve the prognosis of MBD patients.
【學位授予單位】：大連醫(yī)科大學
【學位級別】：碩士
【學位授予年份】：2013
【分類號】：R749.62
本文編號：2396876
[Abstract]:Objective: Marchiafava-Bignami 's disease (Marchiafava-Bignami disease,MBD) is a rare complication of chronic alcoholism, with demyelination and necrosis of corpus callosum as the main pathological feature. The disease was named after two pathologists, Marchiafava and Bignami, first described three patients who had long consumed crude red wine in 1903. Due to the lack of specificity in clinical manifestations, the diagnosis of the disease in the past depends on pathological autopsy. In recent years, with the development of imaging techniques such as CT,MRI, it is possible to diagnose MBD patients in vivo. MBD is present in all races and sexes around the world. Since Zhang Tong reported the first case of MBD in 1994, the number of cases in China has been increasing. In this study, we summarized the epidemiology, clinical manifestations, imaging and prognosis of Chinese patients with MBD to improve the understanding of the clinical characteristics of Chinese patients with MBD. Methods: a case of MBD in our hospital in recent years was reported. At the same time, 84 cases with complete data were selected by searching the Chinese literature related to MBD from 1994 to 2012 in China Zhiwang, Wanfang Full-text Database and Weipu Full-text Database. The following clinical data were collected and analyzed: demographic characteristics (including sex, age), clinical manifestations (including onset time, initial symptoms, neurologic symptoms in the course of the disease), past history. Personal history (including hobbies), routine laboratory indicators, neuroimaging features and prognosis. Results: among 85 cases of MBD in Chinese, 82 cases were male (96.5%), 3 cases were female (3.5%), the average age was 48.2 years old. 82 cases had a history of heavy drinking, accounting for 96.5% (90.6%) of acute. The clinical manifestations of subacute MBD patients were mainly consciousness disorder, epilepsy with pyramidal beam sign. Cognitive impairment was predominant in 8 patients (9.4%) with chronic MBD. Only 2 cases (2.4%) had classic hemispheric disconnection syndrome. In both acute and subacute patients, the incidence of cognitive impairment in patients without or with mild consciousness disorders was higher than that in patients with more severe consciousness disorders (coma or drowsiness) (97.5VS.78.4% P0.05). However, the incidence of gait instability, seizure and muscle tension in patients with severe consciousness disorder was significantly higher than that in patients without or with mild consciousness disorder (P 0.05). All patients were diagnosed by craniocerebral CT and / or MRI. MRI was superior to CT in detecting the extent of corpus callosum (P0.05). The corpus callosum was involved in 46 cases (62.2%), the corpus callosum in 28 cases (37.8%) and the external position of corpus callosum in 36 cases (42.4%). There was no significant correlation between the involvement of corpus callosum and the external position of corpus callosum (P 0.05). Of the 57 patients with prognostic data, 43 (75.4%) improved, 6 (10.5%) deteriorated, and 8 (14.0%) died, all of them were acute and subacute. The mortality rate of patients with severe disturbance of consciousness was significantly higher than that of patients without or with mild disturbance of consciousness (P0.05). Conclusion: (1) Chinese patients with MBD are most common in middle-aged men who have a history of heavy drinking. (2) the majority of Chinese patients with MBD have a good prognosis, but the patients with acute disturbance of consciousness suggest poor prognosis. (3) early imaging identification and appropriate treatment may help to improve the prognosis of MBD patients.
【學位授予單位】：大連醫(yī)科大學
【學位級別】：碩士
【學位授予年份】：2013
【分類號】：R749.62
【參考文獻】
相關期刊論文 前10條
1 陸景紅;周穎;李宗友;王幼萌;許靜;尚志紅;;Marchiafava-Bignami病的臨床及影像學特征[J];安徽醫(yī)學;2007年06期
2 楊華,黃顯龍;胼胝體變性的MRI和CT影像表現(xiàn)及鑒別[J];重慶醫(yī)學;2004年08期
3 王玉波;于云莉;吳珊;劉琦;;慢性乙醇中毒致胼胝體變性1例報道[J];重慶醫(yī)學;2011年04期
4 馬強;馬大慶;靳二虎;賀文;陳步東;;胼胝體變性一例[J];放射學實踐;2008年07期
5 陳麗麗;曹亦賓;;原發(fā)性胼胝體變性1例報告及文獻復習[J];國際神經病學神經外科學雜志;2007年05期
6 王雷;王志敏;周和平;;合并皮質受累的胼胝體變性1例報道及國內報道薈萃分析[J];國際神經病學神經外科學雜志;2009年01期
7 晏華,段廣軍,李繼亮;1例原發(fā)性胼胝體變性[J];貴陽醫(yī)學院學報;2005年05期
8 劉君鳳,郎兆會,譚紅艷;胼胝體變性的CT分析(2例報道并文獻復習)[J];罕少疾病雜志;2001年01期
9 肖衛(wèi)軍,徐嚴明,劉凌,席靜;胼胝體變性2例[J];華西醫(yī)學;2005年03期
10 張榮國,袁清民,王英姿,金光鎮(zhèn),王欣玲,鳳志嵐;胼胝體變性2例報告[J];吉林醫(yī)學;2001年04期
本文編號：2396876
本文鏈接：http://sikaile.net/yixuelunwen/jsb/2396876.html
最近更新
教材專著
