Vázquez C, Salamano R, Legnani C, Cardinal P
Instituto de Neurología, Hospital de Clínicas, Facultad de Medicina, Universidad de la República, Montevideo, Uruguay.
Neurologia. 2008 Jun;23(5):322-8.
Marchiafava-Bignami disease (MBD) is a rare complication of chronic alcoholism characterized by demyelination and necrosis of the corpus callosum that usually has a poor prognosis. It has an extensive clinical spectrum presentation and can presently be diagnosed in vivo with the computed tomography (CT) and magnetic resonance imaging (MRI).
We describe two cases of MBD disease with fatal outcome that presented with acute onset coma. Impairment of consciousness, hypertonia, and frontal release signs were the dominant clinical signs. One of the patients had optic neuropathy and the other had neuropsychiatric symptoms before onset of coma and pellagra-like skin lesion. The CT scan and MRI showed lesions of the white matter that were fundamentally in the corpus callosum.
The possibility of the existence of MBD should be investigated in all patients with chronic alcoholism who have prolonged confusional syndrome, stupor or coma. A prodromal stage with neuropsychiatric symptoms should be thoroughly investigated with a neuropsychological exploration including interhemispheric disconnection signs. Careful attention should be given to the corpus callosum in the image for the diagnosis of this probably underdiagnosed disease.
马基亚法瓦-比尼亚米病（MBD）是慢性酒精中毒的一种罕见并发症，其特征为胼胝体脱髓鞘和坏死，通常预后较差。它有广泛的临床症状表现，目前可通过计算机断层扫描（CT）和磁共振成像（MRI）进行体内诊断。
我们描述了两例MBD病导致死亡的病例，均表现为急性起病的昏迷。意识障碍、肌张力亢进和额叶释放征是主要临床体征。其中一名患者在昏迷和糙皮病样皮肤病变发作前有视神经病变，另一名患者有神经精神症状。CT扫描和MRI显示白质病变主要位于胼胝体。
对于所有患有长期意识模糊综合征、昏迷或昏睡的慢性酒精中毒患者，均应调查是否存在MBD的可能性。对于出现神经精神症状的前驱期，应通过包括半球间分离征在内的神经心理学检查进行全面调查。在诊断这种可能诊断不足的疾病时，应仔细观察图像中的胼胝体。
