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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
 

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.

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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.

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.

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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, 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, 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.

Acknowledgements

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.

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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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