Marchiafava Bignami disease

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Marchiafava-Bignami Disease

Marchiafava-Bignami disease (pronunciation: mar-kee-ah-fah-vah big-nah-mee disease) is a rare neurological disorder characterized by progressive demyelination of the corpus callosum. The disease is named after Italian pathologists Ettore Marchiafava and Amico Bignami, who first described it in 1903.

Etymology

The term "Marchiafava-Bignami disease" is derived from the names of the two Italian pathologists who first identified the condition. Ettore Marchiafava and Amico Bignami discovered the disease while studying the effects of chronic alcoholism on the brain.

Symptoms

The symptoms of Marchiafava-Bignami disease can vary widely, but often include cognitive impairment, difficulty walking (gait disturbances), and seizures. In severe cases, the disease can lead to coma or death.

Causes

While the exact cause of Marchiafava-Bignami disease is unknown, it is most commonly associated with chronic alcoholism. However, it has also been reported in individuals with malnutrition, particularly those with a deficiency in vitamin B1 (thiamine).

Diagnosis

Diagnosis of Marchiafava-Bignami disease is typically made through magnetic resonance imaging (MRI), which can reveal the characteristic demyelination of the corpus callosum. Additional tests may include a physical examination, blood tests, and a neurological examination.

Treatment

Treatment for Marchiafava-Bignami disease primarily involves addressing the underlying cause, such as abstaining from alcohol or correcting nutritional deficiencies. In some cases, corticosteroids may be used to reduce inflammation and slow the progression of the disease.

Related Terms

External links

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