Purpose: Marchiafava-Bignami Disease (MBD) is a rare condition mainly associated with alcoholism, although a few non-alcoholic cases have been reported. We performed a comparative study of demographic and clinical differences between alcoholic and non-alcoholic and assessed whether any treatment can be recommended. Methods: We reviewed 157 reports containing data on 168 subjects with Alcoholic MBD (AMBD) and 23 subjects with Non-Alcoholic MBD (NAMBD). The following data were extracted: demographic characteristics; delay from the onset of symptoms to admission; MRI features; location of the corpus callosum lesions; the presence of Wernicke’s disease; drug treatment (thiamine, other vitamins and steroids); outcome. Results: The subjects with AMBD were more frequently men (84.5% vs 47.8, P = 0.000); the ones with AMBD were frequently reported as suffering from malnutrition (81.3% vs 50%, P = 0.019), whereas the NAMBD was frequently reported as suffering from diabetes mellitus (30.4% vs 7.1%, P = 0.002). The lesions in the NAMBD are often located in the splenium (47.8%), whereas single splenial lesions are seen only in 18.7% of the AMBD. 43.5% (10/23) of the NAMBD was reported to have recovered completely, whereas only 15.4% (24/156) AMBD showed a complete recovery. Conclusions: It is important to diagnose NAMBD in the early stage with MRI and to treat the symptoms with thiamine and/or corticosteroids according to aetiology. We recommend prompt treatment of MBD with parenteral thiamine in subjects associated with malnutrition, anorexia nervosa and prolonged vomiting, and reduction of food intake. Corticosteroids may aid in recovery by reducing oedema in subjects associated with diabetes mellitus.
Marchiafava, E. and Bignami, A. (1903) Sopra un alterazione del corpo calloso osservata in soggetti alcoolisti. Rivista di Patologia Nervosa e Mentale, 8, 544-549.
Brust, J.C. (2010) Ethanol and Cognition: Indirect Effects, Neurotoxicity and Neuroprotection: A Review. International Journal of Environmental Research and Public Health, 7, 1540-1557. http://dx.doi.org/10.3390/ijerph7041540
Rickert, C.H., Karger, B., Varchmin-Schultheiss, K., Brinkmann, B. and Paulus, W. (2001) Neglect-Associated Fatal Marchiafava-Bignami Disease in a Non-Alcoholic Woman. International Journal of Legal Medicine, 115, 90-93. http://dx.doi.org/10.1007/s004140100233
Leong, A.S. (1979) Marchiafava-Bignami Disease in a Non-Alcoholic Indian Male. Pathology, 1, 241-249. http://dx.doi.org/10.3109/00313027909061950
Tao, H., Kitagawa, N., Kako, Y., Yamanaka, H., Ito, K., Denda, K. and Koyama, T. (2007) A Case of Anorexia Nervosa with Marchiafava-Bignami Disease That Responded to High-Dose Intravenous Corticosteroid Administration. Psychiatry Research, 156, 181-184. http://dx.doi.org/10.1016/j.pscychresns.2007.04.013
Maki, N., Hokoishi, K., Komori, K. and Tanabe, H. (2001) A Case of Marchiafava-Bignami Disease Caused by Anorexia Nervosa. No To Shinkei, 53, 669-671.
Hillbom, M., Pyhtinen, J., Pylvänen, V. and Sotaniemi, K. (1999) Pregnant, Vomiting, and Coma. Lancet, 353, 1584. http://dx.doi.org/10.1016/S0140-6736(99)01410-5
Cui, Y., Zheng, L., Wang, X., Zhang, W., Yuan, D. and Wei, Y. (2015) Marchiafava-Bignami Disease with Rare Etiology: A Case Report. Experimental and Therapeutic Medicine, 9, 1515-1517.
Furukawa, K., Maeshima, E., Maeshima, S. and Ichinose, M. (2011) Multiple Symptoms of Higher Brain Dysfunction Caused by Marchiafava-Bignami Disease in a Patient with Dermatomyositis. Rheumatology International, 31, 109-112. http://dx.doi.org/10.1007/s00296-009-1164-4
Rusche-Skolarus, L.E., Lucey, B.P., Vo, K.D. and Snider, B.J. (2007) Transient Encephalopathy in a Postoperative Non-Alcoholic Female with Marchiafava-Bignami Disease. Clinical Neurology and Neurosurgery, 109, 713-715. http://dx.doi.org/10.1016/j.clineuro.2007.05.005
Pérez álvarez, A.I., Ramón Carbajo, C., Morís de la Tassa, G. and Pascual Gómez, J. (2015) Marchiafava-Bignami Disease Triggered by Poorly Controlled Diabetes Mellitus. Neurologia.
Yadala, S. and Luo, J.J. (2013) Marchiafava-Bignami Disease in a Nonalcoholic Diabetic Patient. Case Reports in Neurological Medicine, 2013, Article: 979383. http://dx.doi.org/10.1155/2013/979383
Suzuki, Y., Oishi, M., Ogawa, K. and Kamei, S. (2012) A Patient with Marchiafava-Bignami Disease as a Complication of Diabetes Mellitus Treated Effectively with Corticosteroid. Journal of Clinical Neuroscience, 19, 761-762. http://dx.doi.org/10.1016/j.jocn.2011.07.040
Hlaihel, C., Gonnaud, P.M., Champin, S., Rousset, H., Tran-Minh, V.A. and Cotton, F. (2005) Diffusion-Weighted Magnetic Resonance Imaging in Marchiafava-Bignami Disease: Follow-Up Studies. Neuroradiology, 47, 520-524. http://dx.doi.org/10.1007/s00234-005-1368-6
Kilinc, O., Ozbek, D., Ozkan, E. and Midi, I. (2015) Neurological and Psychiatric Findings of Marchiafava-Bignami Disease in a Nonalcoholic Diabetic Patient with High Blood Glucose Levels. Journal of Neuropsychiatry & Clinical Neurosciences, 27, e149-e150. http://dx.doi.org/10.1176/appi.neuropsych.14030056
Caulo, M., Briganti, C., Notturno, F., Committeri, G., Mattei, P.A., Tartaro, A., et al. (2009) Non-Alcoholic Partially Reversible Marchiafava-Bignami Disease: Review and Relation with Reversible Splenial Lesions. A Case Report and Literature Review. The Neuroradiology Journal, 22, 35-40. http://dx.doi.org/10.1177/197140090902200106
Murthy, S.B., Jawaid, A., Bock, J.E., Qureshi, S.U. and Schulz, P.E. (2010) Marchiafava-Bignami Disease (MBD) in a Nonalcoholic Patient: A Case Report. The Canadian Journal of Neurological Sciences, 37, 138-140. http://dx.doi.org/10.1017/S0317167100009823
Ménégon, P., Sibon, I., Pachai, C., Orgogozo, J.M. and Dousset, V. (2005) Marchiafava-Bignami Disease: Diffusion-Weighted MRI in Corpus Callosum and Cortical Lesions. Neurology, 65, 475-477. http://dx.doi.org/10.1212/01.wnl.0000171348.55820.89
Kashiwagi, Y., Horiuchi, T., Harashima, C., Matsubara, F. and Harashima, S. (2010) Marchiafava-Bignami Disease-Like Lesions Due to Central Nervous System Lupus. BMJ Case Reports, bcr0820103269. http://dx.doi.org/10.1136/bcr.08.2010.3269
Boutboul, D., Lidove, O., Aguilar, C., Klein, I. and Papo, T. (2010) Marchiafava-Bignami Disease Complicating SC Hemoglobin Disease and Plasmodium falciparum Infection. La Presse Médicale, 39, 990-993. http://dx.doi.org/10.1016/j.lpm.2010.02.052
Celik, Y., Temizoz, O., Genchellac, H., Cakir, B. and Asil, T. (2007) A Non-Alcoholic Patient with Acute Marchiafava-Bignami Disease Associated with Gynecologic Malignancy: Paraneoplastic Marchiafava-Bignami Disease? Clinical Neurology and Neurosurgery, 109, 505-508. http://dx.doi.org/10.1016/j.clineuro.2007.02.011
Jorge, J.M., Gold, M., Sternman, D., Prabhakaran, K. and Yelon, J. (2015) Marchiafava-Bignami Disease in a Trauma Patient. Journal of Emergencies, Trauma, and Shock, 8, 52-54. http://dx.doi.org/10.4103/0974-2700.150399
Ma, K.C. and Chen, K.J. (1983) Primary Necrosis of Corpus Callosum with Dystrophic Astrogliosis and Rosenthal- Like Fiber Formation. The First Chinese Case of Marchiafava-Bignami’s Disease (MBD). Acta Neuropathologica, 60, 283-290. http://dx.doi.org/10.1007/BF00691878
Tozakidou, M., Stippich, C. and Fischmann, A. (2011) Teaching Neuroimages: Radiologic Findings in Marchiafava-Bignami Disease. Neurology, 77, e67. http://dx.doi.org/10.1212/wnl.0b013e31822e144b
Thomson, A.D., Guerrini, I. and Marshall, E.J. (2012) The Evolution and Treatment of Korsakoff’s Syndrome: Out of Sight, Out of Mind? Neuropsychology Review, 22, 81-92. http://dx.doi.org/10.1007/s11065-012-9196-z
Hillbom, M., Saloheimo, P., Fujioka, S., Wszolek, Z.K., Juvela, S. and Leone, M.A. (2014) Diagnosis and Management of Marchiafava-Bignami Disease: A Review of CT/MRI Confirmed Cases. Journal of Neurology, Neurosurgery & Psychiatry, 85, 168-173. http://dx.doi.org/10.1136/jnnp-2013-305979
Wenz, H., Eisele, P., Artemis, D., Förster, A. and Brockmann, M.A. (2014) Acute Marchiafava-Bignami Disease with Extensive Diffusion Restriction and Early Recovery: Case Report and Review of the Literature. Journal of Neuroimaging, 24, 421-424. http://dx.doi.org/10.1111/j.1552-6569.2012.00755.x