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[马基亚法瓦-比尼亚米病的生前诊断]

[Antemortem diagnosis of Marchiafava-Bignami disease].

作者信息

Shiota J, Kawamura M, Hirayama K, Isono O, Maki T

出版信息

Rinsho Shinkeigaku. 1989 Jun;29(6):701-6.

PMID:2684469
Abstract

Marchiafava-Bignami disease (M-B disease) is a peculiar disease, observed in hardened drinkers and resulting in demyelinization and necrosis of the corpus callosum. The clinical symptoms are diverse, and it is difficult to diagnose accurately while the patient is alive. Almost all reports in the past have been based on necropsy. Only three cases of M-B disease have been reported in Japan so far, none of which was based on a diagnosis while the patient was alive. We have been able to perform diagnosis of M-B disease while the patients were still alive by means of X-ray computed tomography (CT) and magnetic resonance imaging (MRI), and to study the clinical symptoms from the acute stage. In addition to the study on our own patients, we also conducted a neurosymptomatic study on the clinical symptoms of 6 cases, for which details of the symptomatology have been described-out of about 150 cases reported in the past. In the acute stage of M-B disease, we observed psychic symptoms, such as clouding of consciousness, mnemonic disorders and disorders and disorientation, or various frontal lobe symptoms, such as change in personality, raptus nervorum, dysphemia, pyramidal signs and astasia-abasia. These are non-specific symptoms and it is necessary to differentiate from encephalitis and other encephalopathy. In the chronic stage, dysphemia becomes sustained and, in addition, symptoms of transection of the corpus callosum are noted among the disorders of higher cerebral functions. If M-B disease is suggested from clinical symptoms, it is advisable to confirm the lesions in the corpus callosum by X-ray CT and MRI.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

马基亚法瓦-比尼亚米病(M-B病)是一种特殊的疾病,见于酗酒者,可导致胼胝体脱髓鞘和坏死。其临床症状多样,患者在世时难以准确诊断。过去几乎所有报告均基于尸检。迄今为止,日本仅报告了3例M-B病,均非生前诊断。我们已能够通过X线计算机断层扫描(CT)和磁共振成像(MRI)在患者生前对M-B病进行诊断,并从急性期开始研究临床症状。除了对我们自己的患者进行研究外,我们还对6例患者的临床症状进行了神经症状学研究,在过去报告的约150例病例中,已有对这些病例症状学细节的描述。在M-B病的急性期,我们观察到精神症状,如意识模糊、记忆障碍、定向障碍,或各种额叶症状,如人格改变、神经发作、言语困难、锥体束征和站立行走不能。这些都是非特异性症状,有必要与脑炎和其他脑病相鉴别。在慢性期,言语困难持续存在,此外,在高级脑功能障碍中可注意到胼胝体横断的症状。如果根据临床症状怀疑为M-B病,建议通过X线CT和MRI确认胼胝体的病变。(摘要截选于250字)

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