Marchiafava-Bignami Disease Workup

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

Approach Considerations

Because many patients with Marchiafava-Bignami disease (MBD) present with stupor or coma and seizures, the initial laboratory investigations should include measurements of serum electrolyte and glucose levels, a complete blood count (CBC), and toxicology screening. Glucose and intravenous (IV) thiamine are frequently given in the emergency department immediately after blood is drawn.

A spinal tap often is needed and usually is performed after findings on a brain CT scan have excluded an intracranial mass or hemorrhage.

Electroencephalography

Electroencephalography is frequently performed to evaluate seizures. No electroencephalographic findings are specific for or characteristic of MBD.[23]

CT scanning

Findings on the initial CT scan may confirm the diagnosis of MBD. If callosal damage is mild, however, it may go unnoticed until the radiologist has carefully reviewed the CT scan. In some cases, the lesions may not be visible on the scan.

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MRI

MRI is currently the most sensitive diagnostic tool for MBD.[24, 25] Fast spin-echo, T2-weighted MRI scans show hyperintensity of the lesions due to edema and myelin damage.

Hypointensity on T1-weighted images is mainly related to a total loss of myelin, with replacement of the region by a cyst. Neurons can also be lost, in a situation similar to that of multiple sclerosis. As reported by Sair et al, diffusion tensor imaging and the associated technique of fiber tracking can further increase the sensitivity of MRI.[26]

Acute or subacute lesions are characterized by edema and early myelin damage more than other changes. As lesions become chronic, cystic lesions are likely to develop. Cystic lesions are generally hyperintense around the rim on T2-weighted MRI scans and hypointense in the actual cavity on T1-weighted images. (See the image below.)

T2-weighted axial image in a patient with MarchiafT2-weighted axial image in a patient with Marchiafava-Bignami disease showing a high-signal lesion in the corpus callosum.

Fluid-attenuated inversion recovery (FLAIR) images may be even more sensitive than those described above. Hyperintense rims and hypointense cores on FLAIR images probably represent damage to the myelin at the rim, with a central necrotic area. Uniformly hyperintense lesions may contain a mixture of demyelination and edema. In acute lesions, the area of edema seen is frequently larger than the area of permanent damage.

Pathology may also be seen on diffusion-weighted imaging. Unlike in stroke, however, in MBD, according to a report by Hlaihel et al, it is not uncommon for areas of restricted diffusion to resolve completely without apparent permanent damage.[21, 27]

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Histologic Findings

Degeneration of the corpus callosum is a cardinal feature of MBD. The middle portion (middle lamina) of the myelinated fiber tracts of the corpus callosum degenerates. The degeneration is frequently, but not always, uniform. In some cases, the anterior portion is preferentially involved, with the most severe degeneration in the center of the lesion.

The anterior and posterior commissures, the centrum semiovale, the brachium pontis, and the other white-matter tracts (eg, the long association fibers and the middle cerebral peduncles) may also be affected. However, the internal capsule and corona radiata, as well as the shorter arcuate subgyral association fibers, are typically spared. If the splenium of the corpus callosum is affected, the greatest degeneration most commonly occurs in the lateral portions of the middle segment.

Histopathologic studies reveal abundant macrophages in the areas of lesions. Otherwise, little inflammatory reaction is noted. Axons are demyelinated in the involved areas, but the axon cylinders are relatively spared, particularly in the peripheral portions of the lesions. Deep in the lesion, cavitation, or cyst formation may be seen and corresponds to complete necrosis of all neural and glial elements.

Patients with MBD do not usually have midline lesions, which are typical in patients with Wernicke-Korsakoff syndrome (of the medial thalamus or mamillary bodies).

Finally, as previously mentioned, cortical lesions are sometimes found on postmortem neuropathologic studies. In such cases, neuronal degeneration of the third and fourth layers of the frontal and temporal cortices has been found, with replacement of the neuron by gliosis (ie, Morel cortical laminar sclerosis).

Controversy exists as to whether cortical MRI findings in MBD actually correlate with such pathologic findings and whether they may have implications for prognosis. Whether the cortical findings are secondary to the callosal damage, whether both are caused by a similar process, or whether they are coincidental findings that may also occur separately, particularly in severe alcoholism, malnutrition, and/or other severe impairments, remains unclear.

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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, 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

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

  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].

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

  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].

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

  27. Ihn YK, Hwang SS, Park YH. Acute Marchiafava-Bignami disease: diffusion-weighted MRI in cortical and callosal involvement. Yonsei Med J. Apr 30 2007;48(2):321-4. [Medline].

  28. 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].

 
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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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