Marchiafava-Bignami disease: longitudinal MR imaging and MR spectroscopy study

Area of Science:

• Neuroimaging
• Neurology
• Biochemistry

Background:

• Marchiafava-Bignami disease is a rare neurological disorder primarily affecting the corpus callosum.
• Early diagnosis and monitoring of treatment response are crucial for Marchiafava-Bignami disease management.

Observation:

• Serial MR imaging demonstrated evolving signal intensity abnormalities in the corpus callosum.
• Macroscopic changes were absent initially, but callosal atrophy was noted in later stages.

Findings:

• MR spectroscopy revealed a decreasing N-acetylaspartate to creatine ratio, indicating neuronal damage, with partial recovery.
• Choline to creatine ratio normalized, and lactate was replaced by lipids, reflecting metabolic shifts during disease progression and recovery.
• These findings correlate with the effectiveness of vitamin therapy.

Implications:

• MR imaging is essential for diagnosing Marchiafava-Bignami disease.
• MR spectroscopy provides valuable insights into the disease's pathogenesis, evolution, and therapeutic response, guiding clinical management.