eMedicine Specialties > Neurology > Inflammatory and Demyelinating Diseases

Marchiafava-Bignami Disease

Author: Stephen A Berman, MD, PhD, Professor, Department of Internal Medicine, Section of Neurology, Dartmouth Medical School; Chief, Neurology Service, White River Junction Veterans Medical Center
Coauthor(s): Mardjohan Hardjasudarma, MD, Chief of Neuroradiology, Program Director, Professor, Departments of Clinical Radiology and Ophthalmology, Louisiana State University Health Sciences Center; Eric Dinnerstein, MD, Consulting Staff Neurologist, Maine Neurology
Contributor Information and Disclosures

Updated: Feb 1, 2007

Introduction

Background

In 1903 Marchiafava and Bignami, 2 Italian pathologists, described 3 men with alcoholism who died after having seizures and coma. In each patient, the middle two thirds of the corpus callosum were severely necrotic. Through the years, approximately 250-300 cases of what is now term Marchiafava-Bignami disease (MBD) have been reported in the medical literature. Most patients are men with alcoholism. For years, Italian heritage was thought to be important because Italian pathologists originally described the disease. However, the disease has been found in persons all over the world. Italian ethnicity is now known to not be a factor. In rare cases, the disease has occurred in people without alcoholism.

In some cases, the damage extends to areas such as the nearby subcortical white matter, the anterior commissure, or both. Cases have occurred in association with Wernicke encephalopathy, which nevertheless appears to be a separate problem. Cortical involvement, particularly of the third and forth cortical layers of the lateral frontal cortex, has also been described together with MBD. Whether such cortical pathology is part of MBD or a separate alcohol-related disorder is a matter of controversy. Although nutritional deficiencies have been suspected, the cause of this disease or syndrome is still unknown.

Pathophysiology

Single-photon emission CT (SPECT) scanning has yielded interesting pathophysiologic data related to MBD. In one published case reported by Ferracci et al in 1999, SPECT scanning showed a bilateral reduction in cerebral blood flow. The patient had left hemispatial neglect in addition to the expected left-handed apraxia and agraphia.

In 2006, Nardone et al reported an interesting study on a patient who had partially recovered from MBD who demonstrated impairment of transcallosal inhibition. When performed in the proper way, transcranial magnetic stimulation of the motor cortex elicits excitatory responses in contralateral hand muscles and it also suppresses tonic voluntary activity in ipsilateral muscles. The inhibitory signal is conveyed across the corpus callosum. This inhibition was reduced in the partially recovered patient described by Nardone et al.

Frequency

United States

MBD is a very rare condition. The authors did not find specific published epidemiological data. In 2001, Helenius et al wrote that they had found approximately 250 cases in published reports, although they also suggested that many cases were undiagnosed. In an attempt to check the Helenius conclusions, the authors did a Medline search and found 148 papers published since 1966. Indeed, approximately 250 cases were included among these papers (although occasionally some papers may have duplicated the same patient). Adding 40 or 50 cases mentioned in older textbooks that would not have been included in Pubmed, the number may be a bit higher than 250, but, essentially Helenius et al are accurate. That being stated, the information cannot be converted to accurate epidemiological data.

International

The comments made above for the US cases also apply to the international cases. One additional comment on international cases deserves mention. Some of the old literature on MBD suggests that this condition is more common in Italians. This is solely an artifact of the fact that the initial cases were found in Italy and that for a certain amount of time the Italian physicians appeared to be the only ones interested in finding such cases. It is now firmly believed that no known national, geographic, ethnic, or racial predilection exists. However, with a small number of reports, the numbers of cases reported from each country would not be expected to be exactly in proportion to the population of each country. Numerous factors, including the autopsy rate and, now, the availability of MRI, could influence the rate of diagnosis. Interestingly, the first Polish case to be diagnosed was reported by Staszewski et al in 2006. This case was detected via MRI.

Mortality/Morbidity

The original cases were diagnosed based on pathology findings; therefore, the mortality rate was thought to be extremely high. More recently, MRI has allowed clinicians to detect cases in patients who survived the initial insult. A few patients have had a virtually complete recovery of their intellectual functions; others have only partially improved. Therapy with thiamine and vitamin B complex, including vitamin B-12 and folate, has been used in many patients who have recovered. However, identical therapy has been used in patients who did not recover. In one patient who recovered, corticosteroids were also given, as reported by Nardone et al in 2006. In patients who improved, the CT and MRI findings also showed improvement.

  • Reliable numbers are not available because of the few reported cases, but the mortality and morbidity rates are still presumed to be high. However, the mild form is thought to be underdiagnosed. If this is the case, the overall outcome may be better than previously thought.
  • As Helenius et al reported in 2001, of the approximately 250 reported patients, 200 have died, 30 remained severely demented or bedridden, and only 20 had a favorable outcome.
  • See also the Prognosis section below.

Race

No racial predilection is known.

Sex

Most reported cases have been in men.

Age

Most reported cases have occurred in persons older than 45 years.

Clinical

History

  • Most patients 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.
  • In 2004, Heinrich et al described 2 clinical subtypes based on a review of 50 radiologic cases diagnosed in vivo:
    • Type A had predominant features of coma and stupor. This subtype is associated with a high prevalence of pyramidal-tract symptoms. Radiologic features include involvement of the entire corpus callosum.
    • Type B is characterized by normal or mildly impaired mental status. Radiologic features are partial or focal callosal lesions.

Physical

Although the physical findings are typically nonspecific, good physical examination may offer clues to the diagnosis. Patients with severe alcoholism who have this syndrome frequently have other problems, such as subdural hemorrhage, Wernicke 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.
    • 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) 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: Disconjugate eye movements, together with confusion, may indicate 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.
  • Reflexes
    • 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.

Causes

  • Alcoholism remains the greatest risk factor, although rare cases have occurred in individuals who did not drink alcohol.
  • Nutritional factors have been suspected, but no specific nutrient has been identified.
  • Electrolyte disturbances (as in central pontine myelinolysis) may be important.
  • Cocaine use is not known to cause this condition.

More on Marchiafava-Bignami Disease

Overview: Marchiafava-Bignami Disease
Differential Diagnoses & Workup: Marchiafava-Bignami Disease
Treatment & Medication: Marchiafava-Bignami Disease
Follow-up: Marchiafava-Bignami Disease
Multimedia: Marchiafava-Bignami Disease
References
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References

  1. Adams RD, Victor M, Ropper AH. Diseases of the nervous system due to nutritional deficiency. In: Principles of Neurology. New York, NY: McGraw-Hill;. 1997: 1158-9.

  2. Berek K, Wagner M, Chemelli AP, et al. Hemispheric disconnection in Marchiafava-Bignami disease: clinical, neuropsychological and MRI findings. J Neurol Sci. May 1994;123(1-2):2-5. [Medline].

  3. Brion S. Marchiafava-Bignami disease. In: Vinken PJ, Bruyn GW, eds. Handbook of clinical neurology. Amsterdam: North H. 1977;317â€"329.

  4. Chang KH, Cha SH, Han MH, et al. Marchiafava-Bignami disease: serial changes in corpus callosum on MRI. Neuroradiology. 1992;34(6):480-2. [Medline].

  5. Charness ME. Brain lesions in alcoholics. Alcohol Clin Exp Res. Feb 1993;17(1):2-11. [Medline].

  6. 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].

  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. 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].

  9. Gabriel S, Grossmann A, Hoppner J, et al. [Marchiafava-Bignami syndrome. Extrapontine myelinolysis in chronic alcoholism]. Nervenarzt. Apr 1999;70(4):349-56. [Medline].

  10. 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].

  11. Gass A, Birtsch G, Olster M, et al. Marchiafava-Bignami disease: reversibility of neuroimaging abnormality. J Comput Assist Tomogr. May-Jun 1998;22(3):503-4. [Medline].

  12. Ghatak NR, Hadfield MG, Rosenblum WI. Association of central pontine myelinolysis and Marchiafave-Bignami disease. Neurology. Dec 1978;28(12):1295-8. [Medline].

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

  14. 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].

  15. 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].

  16. Ishii K, Ikerjiri Y, Sasaki M, et al. Regional cerebral glucose metabolism and blood flow in a patient with Marchiafava-Bignami disease. AJNR Am J Neuroradiol. Aug 1999;20(7):1249-51. [Medline].

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

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

  19. Kalckreuth W, Zimmermann P, Preilowski B, Wallesch CW. Incomplete split-brain syndrome in a patient with chronic Marchiafava-Bignami disease. Behav Brain Res. Oct 20 1994;64(1-2):219-28. [Medline].

  20. 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].

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

  22. Logak M, Feve A, Samson Y, et al. [Contribution of position emission tomography in a case of Marchiafava Bignami disease: Morel's laminar sclerosis?]. Rev Neurol (Paris). Jan 1996;152(1):47-50. [Medline].

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

  24. Mas G, Gonzalez-Caballero G, Martinez-Ortiz MJ, Saez-Castan J. [Marchiafava-Bignami disease in a non-alcoholic patient]. Rev Neurol. May 16-31 2006;42(10):637-8. [Medline].

  25. 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].

  26. Miller JR, Sadiq SA. Marchiafava-Bignami Disease. In: Rowland LP, ed. Merritt's Textbook of Neurology. 9th ed. Baltimore, MD: Lippincott Williams and Wilkins;. 1995: 826-9.

  27. Moreaud O, Dufosse N, Pellat J. [Marchiafava-Bignami disease: development with flare-up]. Rev Neurol (Paris). Aug-Sep 1996;152(8-9):560-2. [Medline].

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

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

  30. Namba Y, Bando M, Takeda K, et al. [Marchiafava-Bignami disease with symptoms of the motor impersistence and unilateral hemispatial neglect]. Rinsho Shinkeigaku. Jun 1991;31(6):632-5. [Medline].

  31. 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].

  32. Navarro JF, Noriega S. [Marchiafava-Bignami disease]. Rev Neurol. Mar 1-15 1999;28(5):519-23. [Medline].

  33. Niwa K, Matsushima K, Yamamoto M, Shinohara Y. [Morel's laminar necrosis like findings on MRI in a case of extra-pontine myelinolysis]. Rinsho Shinkeigaku. Mar 1991;31(3):327-30. [Medline].

  34. 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].

  35. Pappata S, Chabriat H, Levasseur M, et al. Marchiafava-Bignami disease with dementia: severe cerebral metabolic depression revealed by PET. J Neural Transm Park Dis Dement Sect. 1994;8(1-2):131-7. [Medline].

  36. Pfefferbaum A, Lim KO, Desmond JE, Sullivan EV. Thinning of the corpus callosum in older alcoholic men: a magnetic resonance imaging study. Alcohol Clin Exp Res. Jun 1996;20(4):752-7. [Medline].

  37. Regadera JF, Enriquez R, Morales M, et al. [Association of Marchiafava-Bignami disease with centro-pontine myelinosis and Morel's laminar sclerosis: presentation of a case]. Med Clin (Barc). Jan 28 1984;82(3):117-20. [Medline].

  38. 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.

  39. Rosa A, Demiati M, Cartz L, Mizon JP. Marchiafava-Bignami disease, syndrome of interhemispheric disconnection, and right-handed agraphia in a left-hander. Arch Neurol. Sep 1991;48(9):986-8. [Medline].

  40. Sair HI, Mohamed FB, Patel S, et al. Diffusion tensor imaging and fiber-tracking in Marchiafava-Bignami disease. J Neuroimaging. Jul 2006;16(3):281-5. [Medline].

  41. Shiota J, Kawamura M, Hirayama K, et al. [Antemortem diagnosis of Marchiafava-Bignami disease]. Rinsho Shinkeigaku. Jun 1989;29(6):701-6. [Medline].

  42. 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].

  43. Tobita M, Mochizuki H, Takahashi S, et al. A case of Marchiafava-Bignami disease with complete recovery: sequential imaging documenting improvement of callosal lesions. Tohoku J Exp Med. Jun 1997;182(2):175-9. [Medline].

  44. Toelle SP, Huisman TA, Martin E, Boltshauser E. Marchiafava-Bignami-like injury of the corpus callosum in an infant. Neuropediatrics. Oct 2005;36(5):328-31. [Medline].

  45. Tomasini P, Guillot D, Sabbah P, et al. [Marchiafava-Bignami's disease with a favourable course. Apropos of a case]. Ann Radiol (Paris). 1993;36(4):319-22. [Medline].

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Further Reading

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Keywords

Marchiafava-Bignami syndrome, MBD, MBS, primary degeneration of the corpus callosum, symmetrical demyelination or necrosis of the middle portion of the corpus callosum and adjacent subcortical tissue, occurring predominantly in malnourished alcoholics

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Contributor Information and Disclosures

Author

Stephen A Berman, MD, PhD, Professor, Department of Internal Medicine, Section of Neurology, Dartmouth Medical School; Chief, Neurology Service, White River Junction Veterans Medical Center
Stephen A Berman, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Coauthor(s)

Mardjohan Hardjasudarma, MD, Chief of Neuroradiology, Program Director, Professor, Departments of Clinical Radiology and Ophthalmology, Louisiana State University Health Sciences Center
Mardjohan Hardjasudarma, MD 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.

Eric Dinnerstein, MD, Consulting Staff Neurologist, Maine Neurology
Eric Dinnerstein, MD is a member of the following medical societies: American Academy of Neurology and American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

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 and Association of American Physicians and Surgeons
Disclosure: Nothing to disclose.

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Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
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Managing Editor

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; Associate Program Director, Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, St Louis University
Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Paraplegia Society, and National Multiple Sclerosis Society
Disclosure: Nothing to disclose.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
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