eMedicine Specialties > Neurology > Inflammatory and Demyelinating Diseases

Marchiafava-Bignami Disease

Author: Jennifer Ault, DO, DPT, Resident Physician, Department of Neurology, Dartmouth-Hitchcock Medical Center
Coauthor(s): 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; 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: Nov 16, 2009

Introduction

Background

In 1903, 2 Italian pathologists named Marchiafava and Bignami described 3 alcoholic men who died after having seizures and coma. In each patient, the middle two-thirds of the corpus callosum was found to be severely necrotic. Through the years, the medical literature has accumulated hundreds of cases of what is now termed Marchiafava-Bignami disease (MBD).1 Most of these cases are alcoholic men, but the disease does occur in nonalcoholics, although rarely.

In 2007, 2 case reports were published describing MBD in nonalcoholics. Celik et al reported a case of a nonalcoholic patient with acute Marchiafava-Bignami disease that was associated with a gynecologic malignancy. The authors raise the question of a possible paraneoplastic Marchiafava-Bignami disease.2 Rusche-Skolarus et al have described a case of Marchiafava-Bignami disease in a postoperative nonalcoholic female who presented with an encephalopathy.3

For years, Italian heritage was thought to be important because Italian pathologists originally described the disease. However, MBD has since been found in persons from all over the world. In 2004, Heinrich et al described 2 clinical subtypes based on a review of 50 radiologic cases diagnosed in vivo:4

  • 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.

Pathophysiology

Single-photon emission CT (SPECT) scanning has yielded interesting pathophysiologic data related to MBD. In a 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.5

In 2006, Nardone et al reported an interesting study on a partially recovered patient with MBD who demonstrated impairment of transcallosal inhibition. When performed properly, transcranial magnetic stimulation of the motor cortex elicits excitatory responses in contralateral hand muscles and suppresses tonic voluntary activity in ipsilateral muscles. The corpus callosum conveys the inhibitory signal. This inhibition was reduced this patient.6

Frequency

United States

MBD is a very rare condition. In 2001, Helenius et al wrote that they had found approximately 250 cases in published reports, although they also suggested that many cases had gone undiagnosed.7

Since 1966, 176 papers have been published. The authors estimate approximately 300 cases in published reports as of November 2008. Another 40 or 50 cases have been mentioned in textbooks that are too old to have been included in the author's PubMed search.

Now, with the availability of MRI, fewer cases are going undiagnosed. No accurate epidemiological data are available as of November 2008.

International

International cases are similar to US cases, but one additional detail 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 initial cases' being found in Italy and Italian physicians' appearing to be the only ones interested in finding such cases at first. It is now firmly believed that no national, geographic, ethnic, or racial predilection is known. However, with such few reports, the numbers of cases reported from each country could not be expected to be exactly in proportion to the population size of each country. In 2006, Staszewski et al described the first case in Poland, which was detected by MRI.8

Race

No racial predilection is known to exist.

Sex

Although this disease occurs in both men and women, most cases are found in men.

Age

Most cases of MBD occur in persons older than 45 years.

Clinical

History

  • 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

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.

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

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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

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, 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.

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, American Association of Neuromuscular and Electrodiagnostic Medicine, American College of Occupational and Environmental Medicine, and Society of Toxicology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

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; 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.

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: Neurology (Journal of the American Academy of Neurology)  Honoraria Section Editor

 
 
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