Uchiyama Masanobu, Kasai Hideyo, Kurokawa Shinji, Sakae Yoshiki, Kinno Ryuta
Nihon Arukoru Yakubutsu Igakkai Zasshi. 2014 Oct;49(5):238-48.
Marchiafava-Bignami disease is a rare alcohol-associated disorder. Clinical features include not only disturbed consciousness, dysarthria, tetraparesis, and astasia-abasia as initial symptom but also cognitive deficits and symptoms of interhemispheric disconnection as clinical outcomes. The clinical significance of cerebral microhemorrhage has been recognized in patients with cognitive deficits. We have recently examined the clinical significance of cerebral microhemorrhage in Marchiafava-Bignami disease and demonstrated that demented patients showed higher severity of cerebral microhemorrhage than patients with normal cognitive function. However, the relationship between callosal lesions and cerebral microhemorrhage in Marchiafava-Bignami disease has not been fully examined. The aim of the present study was to clarify the relationship between callosal lesions and cerebral microhemorrhage in Marchiafava-Bignami disease. For this purpose, we report four patients with Marchiafava-Bignami disease. All cases had a history of chronic alcohol abuse and symmetrical lesions in the corpus callosum. Clinical symptoms include not only coma, dysarthria, and astasia-abasia as initial symptom but also dementia as clinical outcomes. Susceptibility-weighted imaging showed asymmetrical hypointense areas in the multiple cortico-subcortical regions, indicating the presence of cerebral microhemorrhage. There were no apparent relationships between the extension of callosal lesion and the severity of cognitive deficits or cerebral microhemorrhage. Our present report indicates that cerebral microhemorrhage, an important. factor for the severity of dementia in Marchiafava-Bignami disease as clinical outcomes, is independent of the callosal lesion.
马基亚法瓦-比尼亚米病是一种罕见的与酒精相关的疾病。临床特征不仅包括意识障碍、构音障碍、四肢轻瘫和站立不能-步行不能作为初始症状,还包括认知缺陷和大脑半球间联系障碍的症状作为临床结局。脑微出血在认知缺陷患者中的临床意义已得到认可。我们最近研究了马基亚法瓦-比尼亚米病中脑微出血的临床意义,结果表明痴呆患者的脑微出血严重程度高于认知功能正常的患者。然而,马基亚法瓦-比尼亚米病中胼胝体病变与脑微出血之间的关系尚未得到充分研究。本研究的目的是阐明马基亚法瓦-比尼亚米病中胼胝体病变与脑微出血之间的关系。为此,我们报告了4例马基亚法瓦-比尼亚米病患者。所有病例均有长期酗酒史,胼胝体出现对称性病变。临床症状不仅包括昏迷、构音障碍和站立不能-步行不能作为初始症状,还包括痴呆作为临床结局。磁敏感加权成像显示多个皮质-皮质下区域存在不对称的低信号区,提示存在脑微出血。胼胝体病变的范围与认知缺陷或脑微出血的严重程度之间没有明显关系。我们目前的报告表明,脑微出血作为马基亚法瓦-比尼亚米病临床结局中痴呆严重程度的一个重要因素,与胼胝体病变无关。