Marchiafava–Bignami disease is a progressive neurological disease of alcoholism, characterized by corpus callosum demyelination and necrosis and subsequent atrophy. The disease was first described in 1903 by the Italian pathologists Amico Bignami and Ettore Marchiafava in an Italian Chianti drinker. In this autopsy, Marchiafava and Bignami noticed that the middle two-thirds of the corpus callosum were necrotic. It is very difficult to diagnose and there is no specific treatment. 

Symptoms

Symptoms can include, but are not limited to lack of consciousness, aggression, seizures, depression, hemiparesis, ataxia, apraxia, coma, etc. There will also be lesions in the corpus callosum.

Causes

It is classically associated with chronic alcoholism especially with red wine consumption and sometimes associated nutritional deficiencies.Alcoholism can also cause thiamine deficiency, which is also observed to cause MBD.

Mechanism

Individuals with MBD usually have a history of alcohol abuse, but this is not always the case. The mechanism of the disease is not completely understood, but it is believed to be caused by a Vitamin B deficiency, malnutrition, or alcohol abuse.The damage to the brain can extend into neighboring white matter and sometimes go out as far as subcortical regions.

Diagnosis

Marchiafava–Bignami disease is routinely diagnosed with the use of an MRI because the majority of clinical symptoms are non-specific. Before the use of such imaging equipment, it was unable to be diagnosed until autopsy. The patient usually has a history of alcoholism or malnutrition and neurological symptoms are sometimes present and can help lead to a diagnosis. MBD can be told apart from other neural diseases due to the symmetry of the lesions in the corpus callosum as well as the fact that these lesions don't affect the upper and lower edges.

There are two clinical subtypes of MBD

Type A- Stupor and coma predominate. Radiological imaging shows involvement of the entire corpus callosum. This type is also associated with symptoms of the upper motor neurons.

Type B- This type has normal or only mildly impaired mental status and radiologic imaging shows partial lesions in the corpus callosum.

Treatment

Treatment is variable depending on individuals. Some treatments work extremely well with some patients and not at all with others. Some treatments include Therapy with thiamine and vitamin B complex. Alcohol consumption should be stopped. Some patients survive, but with residual brain damage and dementia. Others remain in comas that eventually lead to death. Nutritional counseling is also recommended. Treatment is often similar to those administered for Wenicke-Korsakoff syndrome or for alcoholism.Type A has 21% mortality rate and an 81% long-term disability rate. Type B has a 0% mortality rate and a 19% long-term disability rate.