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Marchiafava-Bignami Disease Clinical Presentation

  • Author: Jennifer Ault, DO, DPT; Chief Editor: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS  more...
Updated: Oct 06, 2014


Most patients diagnosed with Marchiafava-Bignami disease (MBD) have a history of alcoholism and poor nutrition. The tempo of onset and the range of clinical symptoms vary. Some patients present to the hospital with sudden onset of stupor or coma, and some present with seizures. Other patients have acute, subacute, or chronic onset of dementia and/or gait problems. Spasticity often complicates the gait disorder. Psychiatric disturbances, incontinence, hemiparesis, aphasia, and apraxia have been described.


Physical Examination

Although the physical findings in MBD are typically nonspecific, a good physical examination may offer clues to the diagnosis. However, patients with severe alcoholism who have this syndrome frequently have other problems, such as subdural hemorrhage, Wernicke-Korsakoff syndrome, and alcoholic liver disease. Therefore, the diagnosis is not often clear.

General appearance and constitution

Patients later found to have MBD frequently present to an emergency department in a disheveled condition suggestive of chronic problems with alcohol.

Mental status

Patients can be lethargic, stuporous, or even unconscious (coma or seizures). If a patient is sufficiently alert for extensive neuropsychological testing, testing for ideomotor apraxia (ie, inability to perform motor activities that is not explainable by overt motor or sensory loss) may be revealing.[22]

Apraxia of the left (or nondominant) hand suggests interhemispheric disconnection (ie, impaired transfer of information from the left hemisphere to the right hemisphere). Damage to the fibers of the corpus callosum is the cause.

Inability to retain new information (ie, Korsakoff syndrome, the chronic phase of Wernicke-Korsakoff syndrome) and delirium tremens should suggest alcoholism and prompt the examiner to consider other alcohol-related problems, such as MBD. Dementia and aphasia have been noted in some patients with this disease.

Cranial nerves

Nystagmus or disconjugate eye movements, possibly together with confusion and/or ataxia, may indicate the acute/subacute encephalopathic Wernicke phase of the Wernicke-Korsakoff syndrome, which should prompt the examiner to consider MBD.

Motor function

Tremors, weakness, spasticity, and gait abnormalities, although nonspecific, have been seen in patients with MBD.

Delirium tremens is another alcohol-induced problem that patients with MBD may have. Currently, no evidence suggests that the presence of one is either positively or negatively correlated with the presence of the other.

Sensory function

Sensory loss may suggest an alcoholic neuropathy.

Cerebellar functions

Wide-based gait and truncal ataxia suggest alcoholism.


Alcoholic neuropathy can cause a loss of deep tendon reflexes and, therefore, prompt the consideration of MBD in some patients. The presence or absence of Babinski signs is not known to be specifically related to MBD.

Contributor Information and Disclosures

Jennifer Ault, DO, DPT Resident Physician, Department of Neurology, Dartmouth-Hitchcock Medical Center

Jennifer Ault, DO, DPT is a member of the following medical societies: American Academy of Neurology, American Academy of Osteopathy, American Medical Association, American Physical Therapy Association

Disclosure: Nothing to disclose.


Stephen A Berman, MD, PhD, MBA Professor of Neurology, University of Central Florida College of Medicine

Stephen A Berman, MD, PhD, MBA is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, Phi Beta Kappa

Disclosure: Nothing to disclose.

Mardjohan Hardjasudarma, MD, MS Chief of Neuroradiology, Program Director, Professor, Departments of Clinical Radiology and Ophthalmology, Louisiana State University School of Medicine in Shreveport

Mardjohan Hardjasudarma, MD, MS is a member of the following medical societies: American College of Radiology, American Medical Association, American Society of Neuroradiology, Canadian Medical Association, Ontario Medical Association, Pennsylvania Medical Society, Southern Medical Association

Disclosure: Nothing to disclose.

Eric Dinnerstein, MD Consulting Staff Neurologist, Maine Medical Partners Neurology

Eric Dinnerstein, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Received grant/research funds from Janssen Pharmaceuticals for pi conpensation.

Chief Editor

Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS Professor Emeritus of Neurology and Psychiatry, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Neuroscience Director, Department of Neurology, Crouse Irving Memorial Hospital

Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS is a member of the following medical societies: American College of International Physicians, American Heart Association, American Stroke Association, American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners Institute, National Association of Managed Care Physicians, American College of Physicians, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, Royal Society of Medicine

Disclosure: Nothing to disclose.


Jonathan S Rutchik, MD, MPH Assistant Professor, Department of Occupational and Environmental Medicine, University of California at San Francisco

Jonathan S Rutchik, MD, MPH is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American College of Occupational and Environmental Medicine, and Society of Toxicology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

Florian P Thomas, MD, MA, PhD, Drmed Director, Spinal Cord Injury Unit, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Director, Neuropathy Association Center of Excellence, Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, St Louis University School of Medicine

Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Neurological Association, American Paraplegia Society, Consortium of Multiple Sclerosis Centers, and National Multiple Sclerosis Society

Disclosure: Nothing to disclose.

  1. Marchiafava E, Bignami A. Sopra un alterazione del corpo calloso osservata in soggetti alcoolisti. Riv Patol Nerv. 1903. 8:544.

  2. Celik Y, Temizoz O, Genchellac H, Cakir B, Asil T. A non-alcoholic patient with acute Marchiafava-Bignami disease associated with gynecologic malignancy: paraneoplastic Marchiafava-Bignami disease?. Clin Neurol Neurosurg. 2007 Jul. 109(6):505-8. [Medline].

  3. Rusche-Skolarus LE, Lucey BP, Vo KD, Snider BJ. Transient encephalopathy in a postoperative non-alcoholic female with Marchiafava-Bignami disease. Clin Neurol Neurosurg. 2007. 109:713-5.

  4. Heinrich A, Runge U, Khaw AV. Clinicoradiologic subtypes of Marchiafava-Bignami disease. J Neurol. 2004 Sep. 251(9):1050-9. [Medline].

  5. Morel F. Une forme anatomo-clinique particuliere de l;alcoolisme chronique: Sclerose corticale laminaire alcoolique. Rev Neurol. Rev Neurol. 1939. 71:280-288.

  6. Jequier M, Wildi E. Not Available. Schweiz Arch Neurol Psychiatr. 1956. 77(1-2):393-415. [Medline].

  7. DELAY J, BRION S, ESCOUROLLE R, SANCHEZ A. [Necrosis of the Marchiafava-Bignami corpus callosum and Morel's cortical laminar sclerosis.]. Rev Neurol (Paris). 1959 Oct. 101:560-2. [Medline].

  8. DELAY J, BRION S, ESCOUROLLE R, SANCHEZ A. [Relation between Marchiafava-Bignami degeneration of the corpus callosum and Morel's cortical laminar sclerosis (apropos of 5 anatomo-clinical case reports).]. Encephale. 1959. 48:281-312. [Medline].

  9. Ropper AH, Brown RH. Chapter 41 Diseases of the Nervous System due to Nutritiozal Deficiency. Marchiafava-Bignami Disease(Primary Degeneration of theCorpus Callosum). In: Principles of Neurology. 2005. 998-999.

  10. Naeije R, Franken L, Jacobovitz D, et al. Morel's laminar sclerosis. Eur Neurol. 1978. 17(3):155-9. [Medline].

  11. Okeda R, Kitano M, Sawabe M, et al. Distribution of demyelinating lesions in pontine and extrapontine myelinolysis--three autopsy cases including one case devoid of central pontine myelinolysis. Acta Neuropathol (Berl). 1986. 69(3-4):259-66. [Medline].

  12. Ferracci F, Conte F, Gentile M, et al. Marchiafava-Bignami disease: computed tomographic scan, 99mTc HMPAO-SPECT, and FLAIR MRI findings in a patient with subcortical aphasia, alexia, bilateral agraphia, and left-handed deficit of constructional ability. Arch Neurol. 1999 Jan. 56(1):107-10. [Medline].

  13. Gambini A, Falini A, Moiola L, et al. Marchiafava-Bignami disease: longitudinal MR imaging and MR spectroscopy study. AJNR Am J Neuroradiol. 2003 Feb. 24(2):249-53. [Medline].

  14. Johkura K, Naito M, Naka T. Cortical involvement in Marchiafava-Bignami disease. AJNR Am J Neuroradiol. 2005 Mar. 26(3):670-3. [Medline].

  15. Nardone R, Venturi A, Buffone E, et al. Transcranial magnetic stimulation shows impaired transcallosal inhibition in Marchiafava-Bignami syndrome. Eur J Neurol. 2006 Jul. 13(7):749-53. [Medline].

  16. Helenius J, Tatlisumak T, Soinne L, et al. Marchiafava-Bignami disease: two cases with favourable outcome. Eur J Neurol. 2001 May. 8(3):269-72. [Medline].

  17. Staszewski J, Macek K, Stepien A. [Reversible demyelinisation of corpus callosum in the course of Marchiafava-Bignami disease]. Neurol Neurochir Pol. 2006 Mar-Apr. 40(2):156-61. [Medline].

  18. Menegon P, Sibon I, Pachai C, et al. Marchiafava-Bignami disease: diffusion-weighted MRI in corpus callosum and cortical lesions. Neurology. 2005 Aug 9. 65(3):475-7. [Medline].

  19. Khaw AV, Heinrich A. Marchiafava-Bignami disease: diffusion-weighted MRI in corpus callosum and cortical lesions. Neurology. 2006 Apr 25. 66(8):1286; author reply 1286. [Medline].

  20. Brion S. Marchiafava-Bignami disease. Vinken PJ, Bruyn GW, eds. Handbook of clinical neurology. Amsterdam: North H; 1977. 317.

  21. Hlaihel C, Gonnaud PM, Champin S, et al. Diffusion-weighted magnetic resonance imaging in Marchiafava-Bignami disease: follow-up studies. Neuroradiology. 2005 Jul. 47(7):520-4. [Medline].

  22. Hirayama K, Tachibana K, Abe N, Manabe H, Fuse T, Tsukamoto T. Simultaneously cooperative, but serially antagonistic: a neuropsychological study of diagonistic dyspraxia in a case of Marchiafava-Bignami disease. Behav Neurol. 2008. 19(3):137-44. [Medline].

  23. Fang SC. EEG coherence for a patient with Marchiafava-Bignami disease. Clin EEG Neurosci. October 2007. 38(pt 4):207.

  24. Lee SH, Kim SS, Kim SH, Lee SY. Acute Marchiafava-Bignami disease with selective involvement of the precentral cortex and splenium: a serial magnetic resonance imaging study. Neurologist. 2011 Jul. 17(4):213-7. [Medline].

  25. Yoshizaki T, Hashimoto T, Fujimoto K, Oguchi K. Evolution of Callosal and Cortical Lesions on MRI in Marchiafava-Bignami Disease. Case Rep Neurol. 2010 Mar 23. 2(1):19-23. [Medline]. [Full Text].

  26. Sair HI, Mohamed FB, Patel S, Kanamalla US, Hershey B, Hakma Z, et al. Diffusion tensor imaging and fiber-tracking in Marchiafava-Bignami disease. J Neuroimaging. 2006 Jul. 16(3):281-5. [Medline].

  27. Ihn YK, Hwang SS, Park YH. Acute Marchiafava-Bignami disease: diffusion-weighted MRI in cortical and callosal involvement. Yonsei Med J. 2007 Apr 30. 48(2):321-4. [Medline].

  28. Hillbom M, Saloheimo P, Fujioka S, Wszolek ZK, Juvela S, Leone MA. Diagnosis and management of Marchiafava-Bignami disease: a review of CT/MRI confirmed cases. J Neurol Neurosurg Psychiatry. 2014 Feb. 85(2):168-73. [Medline]. [Full Text].

  29. Kikkawa Y, Takaya Y, Niwa N. [A case of Marchiafava-Bignami disease that responded to high-dose intravenous corticosteroid administration]. Rinsho Shinkeigaku. 2000 Nov. 40(11):1122-5. [Medline].

T2-weighted axial image in a patient with Marchiafava-Bignami disease showing a high-signal lesion in the corpus callosum.
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