国人Marchiafava-Bignami病的临床特点分析

发布时间：2018-12-31 17:11

【摘要】：目的：Marchiafava-Bignami病(Marchiafava-Bignami disease，MBD)是一种临床罕见的慢性酒精中毒并发症，以胼胝体脱髓鞘和坏死为主要病理特征。该病是于两位病理学家Marchiafava和Bignami在1903年首先描述了3例生前曾长期饮用粗制红酒的患者之后而得以命名。由于该病临床表现缺乏特异性，以往确诊该病需要依靠病理尸检。近年来CT、MRI等影像学技术的发展使得MBD患者的活体诊断成为可能。目前MBD在世界范围内所有种族及男女两性当中均可发病。自张通等于1994年报道首例国人MBD患者后，国内病例报道日渐增多。本研究通过总结国人MBD患者的流行病学、临床表现、影像学以及预后的特征，来提高对国人MBD患者临床特点的认识。方法：首先报道1例我院近年收治的MBD患者。同时，文献检索1994～2012年间中国知网、万方全文数据库、维普全文数据库中的MBD相关中文文献，筛选出资料完整的84例病例。最终纳入国人MBD患者85例，收集并分析其下列临床数据：人口学特征(包括性别、年龄)、临床表现(包括发病时间、首发症状、病程中出现的神经病学征候)、既往史、个人史(包括嗜好)、常规实验室指标、神经影像学特点及预后。结果：在85例国人MBD病例中，男性有82例（96.5%），女性有3例（3.5%）；平均发病年龄为48.2岁；有82例具有大量饮酒史，占96.5%。77例(90.6%)急性、亚急性MBD患者临床主要表现为意识障碍、癫痫伴锥体束征；8例(9.4%)慢性MBD患者则以认知损害表现为主。仅有2例(2.4%)有经典的半球间离断综合征表现。在急性和亚急性患者中，无或轻度意识障碍患者认知损害的发生率高于有较严重意识障碍(昏迷或昏睡)患者(97.5%VS.78.4%，，P0.05)。而有较严重意识障碍患者发病前后出现步态不稳、癫痫发作和肌张力增高的发生率均明显高于无或轻度意识障碍患者(P均0.05)。所有患者均经颅脑CT和/或MRI检查明确诊断。MRI对于胼胝体病变范围的检出率明显优于CT（P0.05）。有74例患者具有完整的胼胝体影像资料，其中46例(62.2%)胼胝体全程受累，28例(37.8%)胼胝体部分受累。85例患者中有36例（42.4%）有胼胝体外部位受累。有无意识障碍与胼胝体全程受累或胼胝体外部位受累无明显相关性(P均0.05)。在57例具有预后资料的患者中，有43例(75.4%)好转；6例(10.5%)恶化；8例(14.0%)死亡，且均为急性和亚急性患者。有较严重意识障碍患者的死亡率要明显高于无或轻度意识障碍患者(P0.05)。结论：(1)国人MBD患者最常见于既往有大量饮酒史的中年男性；(2)大部分国人MBD患者总体预后良好，但急性期出现意识障碍者提示预后不良；(3)影像学早期识别与适当的治疗可能有助于改善MBD患者的预后。
[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.
【学位授予单位】：大连医科大学
【学位级别】：硕士
【学位授予年份】：2013
【分类号】：R749.62

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