What is Marchiafava-Bignami disease? Symptoms, causes and treatment


Excessive alcohol consumption is associated with the emergence of various diseases, including Marchiafava-Bignami disease. Learn all about this rare condition.

What is Marchiafava-Bignami disease?  Symptoms, causes and treatment

Last updated: January 25, 2023

Marchiafava-Bignami disease is a rare disease in which erosion of the corpus callosum occurs. with more responsiveness It occurs in men with an alcohol use disorderbut also in people with severe malnutrition.

Symptoms that may appear include seizures, slurred speech, depression, apathy, and aggressiveness. Treatment includes giving thiamine, folic acid, and B vitamins intravenously or by mouth, as well as corticosteroids.

What is Marchiafava-Bignami disease?

this disease It owes its name to the Italian physicians Ettore Marchiafava and Amico Bignamiwhich he described in 1903. In addition, these pathologists are known for studying and identifying the parasite that transmits malaria.

Marchiafava-Bignami disease (MBD for short) is rare. A 2001 study reports a total of 250 reported cases of men of different nationalities.

Many of them suffer from alcohol use disorder.

I know It is characterized by demyelination, as well as eventual necrosis, of the corpus callosum. What does this mean? The corpus callosum is a structure found in the brain that connects the two hemispheres, helping both of them work in coordination.

For its part, myelin is an insulating layer consisting of protein and lipids. It acts like a sheath that covers and insulates nerves. In addition to this function, myelin contributes to the transmission of electrical impulses.

In the central region of the brain is the corpus callosum. It is an anatomical structure that connects the two hemispheres.

Symptoms of Marchiafava-Benami disease

Onset and degree of symptoms of Marschiafava-Bijnami disease It varies from case to case, depending on whether it is acute, subacute, or chronic. Some are related to psychological functioning and others are neurological:

  • twitch
  • non-performance.
  • depression.
  • stutter.
  • Double halves.
  • aggression.
  • seizures.
  • Visual and auditory hallucinations.
  • Spasticity and gait disturbances.
  • Confusion, dementia and paranoia.

Swelling of the corpus callosum can also be estimated on brain imaging. In some cases, signs of interhemispheric separation syndrome appear.

Causes and risk factors

MBD is more common in patients with alcohol use disorder. Although its etiology is not entirely clear, because it also occurs in people who are not addicted to drinking.

In this context, some research suggests that It can occur in patients with chronic malnutrition. In this regard, it is believed that it would be related to a deficiency of b complex vitamins.

Other possible causes mentioned are the following:

  • Sickle cell disease and cerebral malaria.
  • Complications of ketoacidosis in diabetes fat.
  • Malnutrition after surgery transcend Contagious.
  • Heart cancer surgery.

The average age of diagnosed cases is 45 years.

Next to, Marcia Fava-Begnam disease is more common in men. Probably, because alcohol consumption is higher in them, according to statistics.

How is the diagnosis made?

to diagnose this disease a MRI, so that lesions can be seen in the corpus callosum. In this regard, research indicates that people with alcohol use disorder show reduced thickness of the anterior, middle, and posterior corpus callosum, as well as differences in the size of the frontal lobe and cortical sulcus.

The patient’s history is reviewed together and detailed physical and psychological examinations are carried out:

  • Neurological function using some measures (MOHS and mRS).
  • Cognitive function (with tests such as the Montreal or MoCA).
  • Altered consciousness (Glasgow coma scale).
  • Alcohol consumption (MAST-C).

General laboratory tests are recommended, Such as complete blood count, electrolytes, glucose, transaminases, bilirubin and cerebrospinal fluid serology panel. The latter to check for infections in the central nervous system.

Treatment of Marchiafava-Bijnami disease

In the treatment of Marchiafava-Bignami disease Vitamin supplements of thiamine, folate, and others of group B are included. It can be administered intravenously or orally. The dosage varies in each case and depends on the doctor’s indication.

naturally, It is necessary to stop drinking alcohol. But since this can lead to withdrawal symptoms, additional medication or even hospitalization may be required.

Deficiency of B-complex vitamins appears to be the basis of the disorder.

Possible complications

Various studies indicate that patients with MBD Showed a significant improvement after Giving vitamins and corticosteroids. Although in patients with diabetes, stabilization and normalization of plasma glucose is also required.

However, in the acute phase, the affected areas of the corpus callosum may show cytotoxic changes and edema. There is also a possibility of other parts of the brain being affected, such as the cerebral lobes, hemispheric white matter, and basal ganglia.

in untreated patients, as well as those who do not respond to treatment, Demyelination and necrosis may become permanent. Cystic transformation may also occur.

It is not uncommon for other alcohol-related harms or nutritional deficiencies to occur:

  • Abnormal fat metabolism.
  • Diseases of the digestive system.
  • metabolic changes.
  • Vitamin deficiency.
  • liver injury

Likewise, when the corpus callosum is affected, Communication between the hemispheres failsWhich translates into cognitive, sensory, and motor problems. Finally, there are patients who can go into a coma.

Although some may recover on their own, others may die. The death rate is 20%.

weather forecast

Since the severity of this disease is variable, The prognosis is uncertain. Even with the appearance of symptoms, a person can live for years, until he recovers or, conversely, deteriorates to death.

The chances of a patient being cured are greater the earlier the diagnosis is made and the sooner the treatment is achieved. This includes stopping alcohol.

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