Marchiafava-Bignami disease (MBD) is a rare CNS disorder usually seen in the context of alcoholism and malnutrition. The condition classically involves the corpus callosum with necrosis and demyelination.
MBD is in most instances seen in chronic alcoholics, and as such the epidemiology reflects this demographic, with middle-aged to elderly male patients (40-60 years of age) being most commonly affected 2.
The clinical presentation of MBD can vary and patients often present with nonspecific clinical features such as motor or cognitive disturbances, a hemispheric disconnection syndrome (apraxia, hemialexia, dementia) and/or seizures.
The disease is attributed to a deficiency of all eight types of vitamin B group and results in necrosis and demyelination of the corpus callosum. Some reports present cases of extension into the hemispheric white matter, internal capsule and middle cerebellar peduncle. Although rare, Morel laminar sclerosis can also be seen.6
Histologically, the myelin loss may be striking and may be accompanied by axonal degeneration 5.
Prior to the advent of cross-sectional imaging, the diagnosis was only limited to postmortem examination and only the acute variety was diagnosed.
Marchiafava-Bignami disease typically begins in the body of the corpus callosum and later involves the genu and then splenium 2. It classically involves the central layers with relative sparing of the dorsal and ventral extremes (which may be seen as a sandwich sign on sagittal MRI imaging).
- may typically show hypoattenuating regions in the corpus callosum
- in exceptional situations of hemorrhage, these regions may turn into iso- or hyperattenuating 3
Corpus callosum may appear edematous in the acute phase and atrophic in the chronic phase.
- T1: may show hypointense foci in the corpus callosum in the acute phase
- acute phase: hyperintensities in the corpus callosum in the correct clinical setting may aid in the diagnosis
- subacute phase: may show hypointense focal lesions (likely as a result of hemosiderin) 3,4
- ears of the lynx sign may be seen.
- MR spectroscopy: the NAA/Cr ratio may show a progressive decrease to a minimum level after the first few months followed by a partial recovery after around 11 months 3
Treatment and prognosis
Administration of vitamin B complex results in improvement in many patients, although some do not recover and may die of the disease 2.
History and etymology
The disease was originally described in Italian alcoholic patients by Italian pathologists Ettore Marchiafava and Amico Bignami.
General imaging differential considerations include:
- multiple sclerosis with callosal demyelination: occurs in a different clinical setting
- diffuse axonal injury (DAI) to the corpus callosum: preceding trauma
- callosal infarction: rare
- transient lesions of the splenium of the corpus callosum
- 1. Johkura K, Naito M, Naka T. Cortical involvement in Marchiafava-Bignami disease. AJNR Am J Neuroradiol. 2005;26 (3): 670-3. AJNR Am J Neuroradiol (citation) - Pubmed citation
- 2. Arbelaez A, Pajon A, Castillo M. Acute Marchiafava-Bignami disease: MR findings in two patients. AJNR Am J Neuroradiol. 24 (10): 1955-7. AJNR Am J Neuroradiol (citation) - Pubmed citation
- 3. Gambini A, Falini A, Moiola L et-al. Marchiafava-Bignami disease: longitudinal MR imaging and MR spectroscopy study. AJNR Am J Neuroradiol. 2003;24 (2): 249-53. AJNR Am J Neuroradiol (citation) - Pubmed citation
- 4. Bourekas EC, Varakis K, Bruns D et-al. Lesions of the corpus callosum: MR imaging and differential considerations in adults and children. AJR Am J Roentgenol. 2002;179 (1): 251-7. AJR Am J Roentgenol (citation) - Pubmed citation
- 5. Felipe Torres Pacheco, Milena Morais Rego, Jose Iram Mendonça do Rego, Antonio J. da Rocha. “Ears of the Lynx” Sign in a Marchiafava–Bignami patient: Structural Basis and Fiber‐Tracking DTI Contribution to the Understanding of this Imaging Abnormality. (2014) Journal of Neuroimaging. 24 (2): 205. doi:10.1111/j.1552-6569.2012.00714.x - Pubmed
- 5. Haaga JR, Boll D. CT and MRI of the whole body. Mosby. (2009) ISBN:0323053750. Read it at Google Books - Find it at Amazon
- 6. Johkura K, Naito M, Naka T. Cortical involvement in Marchiafava-Bignami disease. (2005) AJNR. American journal of neuroradiology. 26 (3): 670-3.