Marchiafava–Bignami disease

Corpus callosum

Marchiafava–Bignami disease is a rare neurological disorder characterized by the degeneration of the corpus callosum, the thick band of nerve fibers that connects the two hemispheres of the brain. This disease is most commonly associated with chronic alcoholism and malnutrition, although it can also occur in individuals without a history of alcohol abuse.

Signs and Symptoms

The symptoms of Marchiafava–Bignami disease can vary widely but often include:

• Dementia
• Seizures
• Dysarthria (difficulty speaking)
• Hemiparesis (weakness on one side of the body)
• Ataxia (lack of muscle coordination)
• Confusion
• Coma

Pathophysiology

The exact cause of Marchiafava–Bignami disease is not well understood, but it is believed to be related to the toxic effects of alcohol and nutritional deficiencies, particularly of thiamine (vitamin B1). The disease leads to the demyelination and necrosis of the corpus callosum, which disrupts communication between the two hemispheres of the brain.

Diagnosis

Diagnosis is typically made through magnetic resonance imaging (MRI) or computed tomography (CT) scans, which can reveal lesions in the corpus callosum. Clinical history and neurological examination are also important for diagnosis.

Treatment

There is no specific treatment for Marchiafava–Bignami disease. Management focuses on addressing the underlying causes, such as alcohol cessation and nutritional support. Thiamine supplementation may be beneficial. Supportive care, including physical therapy and speech therapy, can help manage symptoms.

Prognosis

The prognosis for individuals with Marchiafava–Bignami disease varies. Some patients may recover partially or fully with appropriate treatment, while others may experience significant neurological deficits or death.

History

The disease was first described in 1903 by Italian pathologists Amico Bignami and Ettore Marchiafava, who observed the condition in chronic alcoholics.

Related Pages

• Corpus callosum
• Alcoholism
• Thiamine deficiency
• Neurological disorder
• Dementia