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Marchiafava-Bignami Disease - Causes, Symptoms, Diagnosis, and Treatment

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Marchiafava-Bignami disease is a condition characterized by damage to the corpus callosum (a group of nerve fibers connecting two regions of the brain).

Medically reviewed by

Dr. Prakashkumar P Bhatt

Published At November 22, 2022
Reviewed AtDecember 13, 2022

Introduction:

Marchiafava-Bignami disease is a rare disorder that damages the protective layer of the nerve fibers in the brain. This disease was found by Italian pathologists Ettore Marchiafava and Amico Bignami in the year 1903 in red wine drinkers of Italy. It is mostly seen in people who have a history of prolonged alcohol consumption. Although the Marchiafava-Bignami disease can affect anybody, it is commonly seen in middle-aged and elderly males.

What Is Marchiafava-Bignami Disease?

Marchiafava-Bignami disease is a rare neurological (related to the nervous system) disorder that involves the demyelination (damage of the protective lining of the nerve fibers present in the brain) and necrosis (death of the body cells due to reduced blood flow) of the corpus callosum and subcortical white matter. The corpus callosum is a slit-like structure containing nerve fibers that connect the left and right parts of the brain. It acts as a communication medium that helps pass information between two brain parts. The subcortical white matter is the deeper part of the brain containing nerve fibers that help carry out important functions of the brain, like memory, hormone secretion, and transmitting nerve signals.

What Are the Causes of Marchiafava-Bignami Disease?

The exact cause of the Marchiafava-Bignami disease is not clear. However, the possible causes of this disease are as follows:

  • Prolonged alcohol consumption history.

  • Deficiency of vitamin B.

  • Uncontrolled diabetes mellitus. Ketoacidosis occurs as a complication of diabetes mellitus, which is a condition when the body does not have sufficient insulin to break down the blood sugar to produce energy.

  • Carbon monoxide poisoning.

  • Cerebral malaria (severe nervous system complication resulting from malaria, which is a disease transmitted from the bite of infected mosquitoes).

  • Sepsis (a life-threatening condition that is due to the body’s response to an infection).

  • Sickle cell disease (a condition in which the red blood cells that have oxygen-carrying capacity attain an abnormal shape).

  • Surgeries (done to remove tumors in the heart).

What Are the Symptoms of Marchiafava-Bignami Disease?

The symptoms of the Marchiafava-Bignami disease are as follows:

  • Dementia (reduced ability to think, remember, and make decisions).

  • Poor control of muscles leading to difficulty in walking, maintaining balance, and hand coordination.

  • Lack of control over functions like swallowing, speaking, and eye movements.

  • Stiffness of muscles that may lead to discomfort and difficulty in movements.

  • Confused mental state with difficulty paying attention.

  • Difficulty in opening the mouth fully.

  • Lack of coordination.

  • Abnormal style of walking.

  • Loss of ability to write.

  • Inability to remember the name of a known person.

  • Difficulty in simple activities like running, jumping, and kicking a ball.

  • Seizures (uncontrolled and sudden disturbances in the brain).

  • Coma (a condition characterized by prolonged periods of unconsciousness).

How to Diagnose Marchiafava-Bignami Disease?

There is no definitive test to diagnose Marchiafava-Bignami disease. However, the following tests are done to diagnose this disorder. They are:

  • Medical History and Physical Examination:

    The doctor collects detailed information from the patient regarding history of alcohol consumption and problems related to memory. The doctor asks about the symptoms experienced by the patient. The doctor also examines the patient to check for any abnormalities in walking and maintaining balance.

  • Blood Tests:

    Blood tests are done to assess the levels of sugar and electrolytes present in the blood. The presence of any toxic substances is also checked.

  • Computed Tomography (CT) Scans:

    Computed tomography of the brain is an imaging test that uses a computer and X-rays to obtain clear pictures of the brain from different angles. The X-ray information is sent to the computer that displays the two-dimensional image of the brain. This test helps to detect any abnormalities present in the brain. In patients with Marchiafava-bignami disease, damage to the corpus callosum region of the brain is observed in the CT scan.

  • Magnetic Resonance Imaging (MRI):

    Magnetic resonance imaging of the brain is a painless imaging test that uses a magnet, a computer, and radio waves to obtain clear images of the brain. This imaging test is more accurate than computed tomography in diagnosing Marchiafava disease. Magnetic resonance imaging test shows the loss of protective layers covering the brain's nerve fibers. The characteristic feature seen is the appearance of a dense or black area in the center of the corpus callosum with whitish areas in the front and back. This is known as a sandwich sign which is a unique feature of the Marchiafava-Bignami disease. Magnetic resonance imaging with gadolinium as a contrast agent (dye) is more effective in finding out the abnormality in the brain.

  • Positron Emission Tomography (PET):

    Positron emission tomography of the brain is an imaging test that is used to find out any abnormalities in the brain with the help of a radioactive substance that acts as a tracer. It also gives information about the functioning of the brain tissues.

How Is Marchiafava-Bignami Disease Treated?

There is no specific treatment that has been proven to cure Marchiafava-Bignami disease. However, few treatment methods are followed to relieve the symptoms and bring the disease under control. They are as follows:

  • Most of the patients affected with Marchiafava disease have a prolonged history of consumption of alcohol. Hence the doctor would advise the patient to withdraw from consuming alcohol. Sudden stoppage of alcohol consumption may lead to alcohol withdrawal syndrome. Withdrawal symptoms may include sleep problems, restlessness, sweating, anxiety, vomiting, increased heartbeat, and seizures. Hence, the patient is asked to take sedative medications to prevent anxiety and sleep problems.

  • Patients are advised to take vitamin B complex pills and other multivitamin tablets.

  • The administration of thiamine (vitamin B1) has shown great improvement in patients affected by the Marchiafava-bignami disease. The main function of thiamine is to convert carbohydrates into energy that is essential for the functioning of the brain and nervous system. Early administration of thiamine (within two weeks of the onset of symptoms) has shown the best results in patients. During the initial two weeks, the administration of thiamine is by intravenous or intramuscular route. Later as the symptoms improve, the doctor may prescribe intake of thiamine orally. The thiamine therapy should be continued until the patient has fully recovered.

  • Patients with serious complications like coma should be hospitalized and put under the care of a critical care specialist for the management of symptoms.

  • After recovery, some patients who cannot control the urge to consume alcohol may be put in rehabilitation centers.

  • A protein and vitamin-rich diet advised by the nutritionist should be followed by the patient.

Conclusion:

Marchiafava-Bignami disease is a very serious condition that might lead to coma if left untreated. It is a disease that is difficult to manage in the later stages. Hence, early diagnosis and effective treatment are the key factors in the management of this disorder. With the right treatment and healthy lifestyle, patients can achieve complete relief from the disease and lead a normal life.

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Dr. Prakashkumar P Bhatt
Dr. Prakashkumar P Bhatt

Neurology

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marchiafava-bignami diseasealcoholism
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