eMedicine Specialties > Neurology > Inflammatory and Demyelinating Diseases

Marchiafava-Bignami Disease: Follow-up

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

Follow-up

Prognosis

  • No systematic study has been conducted to assess the prognosis of patients with MBD. In the pre-CT era, almost all patients were discovered at autopsy. They usually died from the effects of alcoholism and typically had severe neuropsychological deficits before death.
  • Modern CT scanning and MRI allow the detection of mild cases, and some patients have recovered with minimal deficits.
  • If the underlying cause is alcoholism, the prognosis is poor unless the patient adheres to an alcohol treatment program.
  • Recent data suggest an improved overall prognosis, which is correlated with the subtype, as follows:
    • Type A has a long-term disability rate of 86% and a mortality rate of 21%.
    • Type B has a long-term disability rate of 19% and a mortality rate of 0%.
  • As Helenius et al reported in 2004, of the approximately 250 reported patients, 200 died, 30 remained severely demented or bedridden, and only 20 had a favorable outcome.
    • Alcohol abuse is such a common problem that underdiagnosis of MBD seems likely.
    • Many cases may be diagnosed but not reported, and autopsies are largely not performed.
    • Hence, the disease may be more common than thought, and the overall outcome may be better than previously believed.
 


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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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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