[Antemortem diagnosis of Marchiafava-Bignami disease]

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)