Marchiafava-Bignami Disease Treatment & Management

  • Author: Jennifer Ault, DO, DPT; Chief Editor: B Mark Keegan, MD, FRCPC   more...
 
Updated: Feb 3, 2012
 

Approach Considerations

No specific, proven treatment is available for Marchiafava-Bignami disease (MBD). Various treatments similar to those commonly administered for Wernicke-Korsakoff syndrome or for alcoholism in general have been given to patients with MBD. Some patients have improved and some have not. The most common treatments are thiamine, folate, and other B vitamins (especially vitamin B-12). Folate is commonly given with B-12.

With regard to more unusual treatments, a case report by Staszewski et al described amantadine given together with thiamine, vitamin B-12, and folate; the patient improved.[17] In another case, reported by Kikkawa et al, administration of high-dose corticosteroids was said to precede clinical improvement. In patients who improved, the CT and MRI scan findings also improved, at least somewhat.[28]

Inpatient care

Patients are usually admitted because they present with stupor, coma, and, frequently, seizures.

Follow-up

Patients who survive should receive rehabilitation and, if appropriate, alcohol and nutritional counseling.

Consultations

Depending on the specific presentation and course of MBD, the patient may require consultation with the following specialists:

  • Neurologist - For seizure control
  • Critical care specialist - For coma management
  • Neuropsychologist - For workup of the dementia
  • Neurorehabilitation specialist
  • Psychiatrist or psychologist - For treatment of alcoholism
Proceed to Medication
 
 
Contributor Information and Disclosures
Author

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, and American Physical Therapy Association

Disclosure: Nothing to disclose.

Coauthor(s)

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, and Phi Beta Kappa

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: Janssen Pharmaceuticals Grant/research funds PI conpensation

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, and Southern Medical Association

Disclosure: Nothing to disclose.

Chief Editor

B Mark Keegan, MD, FRCPC  Assistant Professor of Neurology, College of Medicine, Mayo Clinic; Master's Faculty, Mayo Graduate School; Consultant, Department of Neurology, Mayo Clinic, Rochester

B Mark Keegan, MD, FRCPC is a member of the following medical societies: American Academy of Neurology, American Medical Association, and Minnesota Medical Association

Disclosure: Novartis Consulting fee Consulting; Bionest Consulting fee Consulting

Additional Contributors

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.

References

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  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. Jul 2007;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. Sep 2004;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). Oct 1959;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. Jan 1999;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. Feb 2003;24(2):249-53. [Medline].

  14. Johkura K, Naito M, Naka T. Cortical involvement in Marchiafava-Bignami disease. AJNR Am J Neuroradiol. Mar 2005;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. Jul 2006;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. May 2001;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. Mar-Apr 2006;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. Aug 9 2005;65(3):475-7. [Medline].

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

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  21. Hlaihel C, Gonnaud PM, Champin S, et al. Diffusion-weighted magnetic resonance imaging in Marchiafava-Bignami disease: follow-up studies. Neuroradiology. Jul 2005;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. Jul 2011;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. Mar 23 2010;2(1):19-23. [Medline]. [Full Text].

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