| Objective: Marchiafava-Bignami disease (MBD), characterized pathologically bydemyelination and necrosis of the corpus callosum, is a rare complication of chronicalcoholism. It was named after two pathologists Marchifava and Bignami, who firstdescribed the disease postmortem in three Italian patients with a history ofinexpensively manufactured red wine consumption, in1903. Owing to a lack of specificclinical features, diagnosis of MBD was only made at autopsy in the past; however,advances in neuroimaging techqiques such as computed tomography (CT) and magneticresonance imaging (MRI) have made the diagnosis possible also in the living. MBD isknown to occur worldwide encompassing all races and both genders. Zhang et al firstreported a Chinese patient with MBD in1994. Since then, case reports have increasedgradually in China. The present study aimed to thoroughly understand theepidemiological characteristics, clinical presentations, neuroimaging features andprognosis in patients with MBD in China.Methods: We first present herein a patient with MBD who was recently hospitalizedat our neurology ward in the First Affiliated Hospital of Dalian Medical University. Inaddition, the literatures published in Chinese involving MBD from1994to2012weresearched in China National Knowledge Internet (CNKI), WanFang Full Text Databaseand VIP Full Text Database, and84cases with sufficient data were then identified. Thus,a total of85Chinese cases with MBD were included for further analysis. We collecteddata in each patient regarding the demographic characteristics including gender and age,past history, personal history including special addiction, clinical manifestationsincluding degree of urgency, initial symptoms and neurological findings during thecourse, routine laboratory indicators, neuroimaging features, treatment methods andprognosis.Results: Among85Chinese cases with MBD,82cases (96.5%) were men and3(3.5%) were women. The mean age of patients with MBD was48.2years (rang27-68 yr).82(96.5%) cases had a history of heavy consumption of alcohol. The main clinicalpresentations in77cases (90.6%) of acute and subacute MBD included consciousnessdisturbance, seizures accompanied by the pyramidal tract signs, while8cases (9.4%) ofchronic MBD presented cognitive impairment predominantly. The typicalinterhemispheric disconnection syndromes were observed only in2cases. Thefrequency of cognitive impairment was much higher in cases with no or minorimpairment of consciousness than in those with major impairment of consciousness(coma or stupor)(97.5%VS.78.4%，P<0.05). However, unsteady gait, seizures andhypertonia were more ofen seen in cases with major impairment of consciousness thanin those with no or minor impairment of consciousness (P <0.05, respectively). Allcases were diagnosed by CT and/or MRI. MRI was superior to CT in detecting theextent of corpus callosum injury (P<0.05). Among74cases who had whole imagingdata of the corpus callosum,46cases (62.2%) showed involvement of the entire corpuscallosum, and28cases (37.8%) demostrated patial callosal lesions.36cases (42.4%)revealed extracallosal sites of involvement. There was no correlation betweeninvolvement of the entire corpus callosum or the extracallosal sites and the presence orabsence of consciousness disturbance (P <0.05, respectively). Among57cases who hadprognosis data,43cases (75.4%) showed improvement,6cases (10.5%) showeddeterioration, and8cases (14.0%) who was classified as acute and subacute MBD died.The mortality rate for the cases with major impairment of consciousness was muchhigher when compared to those with no or minor impairment of consciousness (P<0.05).Conclusion: MBD is most commonly seen in male, middle-aged alcoholics inChina. The prognosis in the majority of Chinese patients with MBD seems to befavourable. However, consciousness disturbance occurred in the acute stage of MBDmay indicate poor prognosis. Early recognition with neuroimaging methods andappropriate treatment may improve the prognosis of patients with MBD. |
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