Marchiafava Bignami Disease: A Rare Neurological Complication of Long-Term Alcohol Abuse

Abstract

Marchiafava Bignami disease is a demyelinating and necrotic disease of the central nervous system in chronic alcohol users and malnourished patients. The primary characteristic lesion of this disease is present in the corpus callosum in the form of its necrosis, but plenty of evidence suggests that it can also affect other parts of the brain. The main pathophysiology revolves around the consumption of alcohol and its ability to cause thiamine depletion in the body and hinder various metabolic pathways. There is also a hindrance in myelin synthesis, which further damages the signal transmission leading to an array of symptoms and signs. It is present in different degrees in patients in the form of different stages, namely acute, subacute, and chronic. The diagnosis of the disease becomes tough as the presenting symptoms are very generic and vague. Before the innovation of advanced imaging techniques, it was mainly a finding during an autopsy, but presently it can be diagnosed by a well-taken history and imaging techniques which can help to rule out other diseases having a similar clinical presentation. The gold standard for the diagnosis of the disease is using magnetic resonance imaging (MRI) techniques to visualize the lesions present in the corpus callosum and other areas, but other methods like computed tomography (CT) are also used. The prognosis of the disease is influenced by many factors, and it varies greatly. Some factors such as broad involvement of the cerebral cortex and severe disturbances in consciousness are indicative of a poor prognosis. The differential diagnosis consists of other alcohol use disorders like Wernicke's encephalopathy, neoplastic conditions, and multiple sclerosis, to mention a few. Each one should be carefully eliminated before finalizing the diagnosis. The treatment of the disease is not concrete, but evidence shows improvement with specific interventions.

Keywords: alcoholism; demyelination; diagnosis; marchiafava–bignami disease; neurological disorder; treatment.

Figure 1. MRI T1-weighted sagittal view showing the sandwich sign - hypointense center of corpus callosum but normal area around it.

Reference [30] is licensed under CC BY-NC 4.0

Figure 2. MRI T2 weighted

Reference [30] is licensed under CC BY-NC 4.0

Figure 3. FLAIR showing abnormal hypointensity in corpus callosum

Reference [30] is licensed under CC BY-NC 4.0