What Is Marchiafava-Bignami Disease?

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Marchiafava-Bignami disease (MBD) is a rare condition in which the nerves of the corpus callosum—the area that connects the brain's two hemispheres—become eroded. This disease is most commonly seen in people with severe alcohol use disorder but can also appear in severely malnourished people. MBD is associated with several symptoms, such as paranoia, psychosis, seizures, and dementia.

This article reviews Marchafava-Bignami disease symptoms, causes, diagnosis, and treatment.

human brain with highlighted corpus callosum

KATERYNA KON / SCIENCE PHOTO LIBRARY / Getty Images

Marchiafava-Bignami Disease Symptoms

MBD symptoms can vary by person. Subtle signs might include:

  • Decreased consciousness
  • Psychotic symptoms
  • Depression
  • Apathy
  • Aggressiveness
  • Difficulty speaking

The disease is often divided into three categories: chronic, subacute, or acute.

The chronic form of MBD is less common but generally includes mild dementia. However, this symptom is often correlated with conditions other than MBD.

Subacute forms of MBD may include the following symptoms:

  • Mild to severe confusion
  • Slurred or slow speech
  • Behavioral abnormalities
  • Inability to perform purposeful movements
  • Difficulty walking

Acute symptoms may include:

  • Seizures
  • Decreased consciousness
  • Rapid death within weeks or months

Causes

MBD occurs when nerves of the corpus callosum—the area between the brain's hemispheres—become demyelinated, or eroded. The erosion damages the nerves and eventually leads to nerve cell death and necrosis of the corpus callosum.

This condition is primarily associated with extreme alcohol consumption. However, it has also been observed in patients with severe malnutrition. MBD is thought to result from a deficiency in thiamine, a B vitamin.

Diagnosis

Diagnosing MBD requires magnetic resonance imaging (MRI). An MRI is a noninvasive diagnostic test that uses radiofrequency and magnets to produce images inside the body. MRIs are commonly used for patients who present with neurological symptoms to help establish a diagnosis.

MBD is impossible to diagnose without an MRI. Patients often have a history of alcohol use or malnutrition that may also be used to help establish a diagnosis.

Treatment

Because MBD is closely associated with decreased B vitamins, intravenous (IV) thiamine may be given to treat patients. Recovery also includes neurological and psychological examinations and follow-up MRIs.

Stopping Alcohol Use

People with MBD should stop drinking alcohol immediately. It may even be necessary for some patients to be admitted to the hospital for observation and medications to manage alcohol withdrawal.

Outlook

The outlook for patients with MBD is highly variable. Generally speaking, the outlook is more positive with an early diagnosis and prompt treatment. However, this can also vary from person to person.

Some patients survive for years with symptoms of MBD and then deteriorate suddenly. Repeat MRIs have also shown that MBD lesions can disappear, although this is more likely with early diagnosis and treatment.

Some evidence shows that the appearance and location of lesions on the nerves can help determine the overall prognosis. Research also shows that cerebral lobe impairment, severe loss of consciousness, and heavy alcohol usage are correlated with a poorer prognosis.

If you or a loved one is diagnosed with MBD, talk with your healthcare provider about your specific prognosis and treatment options that may help.

Summary

MBD is a rare condition in which the nerves of the corpus callosum—the area that connects the brain's two hemispheres—erode. MBD is most commonly seen in people with severe alcohol use disorder or in people with severe malnourishment.

MBD is associated with several symptoms such as paranoia, psychosis, seizures, and dementia. Midler symptoms may include difficulty walking, talking, depression, aggression, and decreased consciousness.

MRIs are the only way to establish a diagnosis of MBD. Intravenous thiamine is often given to treat patients with MBD. Recovery also includes neurological and psychological examinations and follow-up MRIs.

A Word From Verywell

Decreasing or eliminating your alcohol intake can lower your chances of developing alcohol-related health issues, such as Marchiafava-Bignami disease. If you are concerned about your alcohol consumption, know that you are not alone, and there are many resources available to help you. Fortunately, with early detection and treatment, many patients with MBD have a favorable prognosis.

Frequently Asked Questions

  • How common is Marchiafava-Bignami disease?

    Marchiafava-Bignami disease is extremely rare. Up-to-date prevalence figures are limited, but a 2001 case study found a total of just 250 reported cases.

  • What are the risk factors for Marchiafava-Bignami disease?

    This condition is primarily associated with extreme alcohol consumption. However, it has also been observed in patients with severe malnutrition. MBD is thought the result from a thiamine deficiency.

  • What are the early signs of Marchiafava-Bignami disease?

    MBD symptoms can vary per patient. Early and more subtle signs might include decreased consciousness, psychotic symptoms, depression, apathy, aggressiveness, and difficulty speaking.

4 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
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  2. Carrilho PE, Santos MB, Piasecki L, Jorge AC. Marchiafava-Bignami disease: a rare entity with a poor outcome. Rev Bras Ter Intensiva. 2013;25(1):68-72. doi:10.1590/s0103-507x2013000100013

  3. Dong X, Bai C, Nao J. Clinical and radiological features of Marchiafava-Bignami disease. Medicine. 2018;97(5):e9626. doi:10.1097/MD.0000000000009626

  4. Helenius J, Tatlisumak T, Soinne L, Valanne L, Kaste M. Marchiafava-Bignami disease: two cases with favourable outcome. Eur J Neurol. 2001;8(3):269-72. doi:10.1046/j.1468-1331.2001.00212.x

By Sarah Jividen, RN
Sarah Jividen, RN, BSN, is a freelance healthcare journalist and content marketing writer at Health Writing Solutions, LLC. She has over a decade of direct patient care experience working as a registered nurse specializing in neurotrauma, stroke, and the emergency room.