Skip to main content
Advertisement

Main menu

  • Home
  • Content
    • Current Issue
    • Accepted Manuscripts
    • Article Preview
    • Past Issue Archive
    • Video Articles
    • AJNR Case Collection
    • Case of the Week Archive
    • Case of the Month Archive
    • Classic Case Archive
  • Special Collections
    • AJNR Awards
    • Low-Field MRI
    • Alzheimer Disease
    • ASNR Foundation Special Collection
    • Photon-Counting CT
    • View All
  • Multimedia
    • AJNR Podcasts
    • AJNR SCANtastic
    • Trainee Corner
    • MRI Safety Corner
    • Imaging Protocols
  • For Authors
    • Submit a Manuscript
    • Submit a Video Article
    • Submit an eLetter to the Editor/Response
    • Manuscript Submission Guidelines
    • Statistical Tips
    • Fast Publishing of Accepted Manuscripts
    • Graphical Abstract Preparation
    • Imaging Protocol Submission
    • Author Policies
  • About Us
    • About AJNR
    • Editorial Board
    • Editorial Board Alumni
  • More
    • Become a Reviewer/Academy of Reviewers
    • Subscribers
    • Permissions
    • Alerts
    • Feedback
    • Advertisers
    • ASNR Home

User menu

  • Alerts
  • Log in

Search

  • Advanced search
American Journal of Neuroradiology
American Journal of Neuroradiology

American Journal of Neuroradiology

ASHNR American Society of Functional Neuroradiology ASHNR American Society of Pediatric Neuroradiology ASSR
  • Alerts
  • Log in

Advanced Search

  • Home
  • Content
    • Current Issue
    • Accepted Manuscripts
    • Article Preview
    • Past Issue Archive
    • Video Articles
    • AJNR Case Collection
    • Case of the Week Archive
    • Case of the Month Archive
    • Classic Case Archive
  • Special Collections
    • AJNR Awards
    • Low-Field MRI
    • Alzheimer Disease
    • ASNR Foundation Special Collection
    • Photon-Counting CT
    • View All
  • Multimedia
    • AJNR Podcasts
    • AJNR SCANtastic
    • Trainee Corner
    • MRI Safety Corner
    • Imaging Protocols
  • For Authors
    • Submit a Manuscript
    • Submit a Video Article
    • Submit an eLetter to the Editor/Response
    • Manuscript Submission Guidelines
    • Statistical Tips
    • Fast Publishing of Accepted Manuscripts
    • Graphical Abstract Preparation
    • Imaging Protocol Submission
    • Author Policies
  • About Us
    • About AJNR
    • Editorial Board
    • Editorial Board Alumni
  • More
    • Become a Reviewer/Academy of Reviewers
    • Subscribers
    • Permissions
    • Alerts
    • Feedback
    • Advertisers
    • ASNR Home
  • Follow AJNR on Twitter
  • Visit AJNR on Facebook
  • Follow AJNR on Instagram
  • Join AJNR on LinkedIn
  • RSS Feeds

AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates

OtherBRAIN

Cortical Involvement in Marchiafava-Bignami Disease

Ken Johkura, Makoto Naito and Takayuki Naka
American Journal of Neuroradiology March 2005, 26 (3) 670-673;
Ken Johkura
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Makoto Naito
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Takayuki Naka
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Data
  • Info & Metrics
  • Responses
  • References
  • PDF
Loading

Abstract

Summary: Marchiafava-Bignami disease (MBD), a rare complication of chronic alcoholism, is characterized by primary demyelination of the corpus callosum. We report two cases of MBD in which fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted imaging studies revealed symmetrical hyperintense lesions in the cerebral cortex (particularly in the lateral-frontal regions) in addition to the callosal lesions, which suggests an association of diffuse cortical lesions such as Morel’s laminar sclerosis with MBD.

Marchiafava-Bignami disease (MBD), which results in demyelination of the corpus callosum, can be observed in people with chronic alcoholism. Although most previous reports of MBD have been based on postmortem pathologic findings (1), the advent of MR imaging has allowed detailed analysis of the distribution of lesions (2, 3). Recent MR imaging studies have shown that lesions may also be found in the hemispheric white matter (3, 4). To the best of our knowledge, however, there has been no reported patient with MBD in whom MR imaging showed cortical abnormalities in addition to callosal lesions. We describe the MR imaging findings in the cerebral cortex of two patients with MBD.

Case Reports

Patient 1

A 72-year-old man with a 40-year history of alcohol (Japanese sake) abuse experienced confusion, lethargy, and diminished appetite for approximately 1 week. He was hospitalized and referred to our department because of his rapidly decreasing level of consciousness. On examination, he was emaciated, dehydrated, and in a deep coma. Results of cerebral spinal fluid (CSF) studies were normal, and electroencephalography (EEG) showed generalized ς rhythm intermingled with δ waves. Fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted images obtained on admission showed hyperintense lesions involving the splenium and body of the corpus callosum (Fig 1). In addition, symmetrical cerebral cortical hyperintense lesions were observed mainly in lateral-frontal regions on FLAIR and diffusion-weighted images (Fig 1). Apparent diffusion coefficient (ADC) mapping showed relatively hypointensity in the callosal and cortical lesions (Fig 1E, -F). On the basis of clinical history and imaging features, MBD was diagnosed, and high-dose vitamin B complex including 500 mg/day thiamine was administered intravenously for 7 days. The patient, however, remained in a persistent vegetative state for more than 3 months. Follow-up T2-weighted sagittal MR imaging study 10 days after the initial study confirmed that lesions were most extensive in the central parts of the corpus callosum, with relative sparing of the dorsal and ventral layers (Fig 1G).

Fig 1.
  • Download figure
  • Open in new tab
  • Download powerpoint
Fig 1.

MR images in patient 1. Axial FLAIR images on admission show hyperintensity in the corpus callosum (A) and the cerebral cortex (B). Diffusion-weighted images (C and D) also show hyperintensity in these regions with relatively decreased ADC values (see Fig 3). The decreased ADC values, however, are inconspicuous on ADC mapping (E and F). Follow-up T2-weighted sagittal image obtained 10 days after the initial study shows callosal lesions mainly involving the central part of the splenium and body (G).

Patient 2

A 56-year-old man was admitted to our hospital because of acute onset of seizures and altered mental state. He had abused alcohol (Japanese sake) for 30 years. At examination, the patient was confused. Although there was no weakness in his extremities, he showed gait disturbance and lack of motor coordination. Routine blood tests and CSF studies revealed no abnormalities. EEG showed diffuse slow waves of 6–8 Hz without epileptiform discharge. FLAIR, and diffusion-weighted images showed abnormal hyperintensity in the corpus callosum and lateral-frontal cerebral cortices with relatively reduced ADC, findings similar to those in case 1 (Fig 2).

Fig 2.
  • Download figure
  • Open in new tab
  • Download powerpoint
Fig 2.

MR images in patient 2. Axial FLAIR images on admission show hyperintensity in the corpus callosum (A) and the cerebral cortex (B). Diffusion-weighted images (C and D) also show hyperintensity in these regions with relatively decreased ADC values (see Fig 3). The decreased ADC values, however, are inconspicuous on ADC mapping (E and F). Follow-up T2-weighted sagittal image obtained 10 days after the initial study shows callosal lesions mainly involving the central part of the splenium and body (G).

Fig 3.
  • Download figure
  • Open in new tab
  • Download powerpoint
Fig 3.

Comparison of signal intensities on diffusion-weighted images with ADC values on ADC maps. Each coordinate of regions of interest in the ADC maps is the same as that in diffusion-weighted images. ADC values are reduced in regions with high signal intensity on diffusion-weighted images (regions 3 and 4 in each patient). Asterisks denote regions in affected cortex identified by high signal intensity on diffusion-weighted images.

Although the patient was given high-dose vitamin B complex, including 500 mg/day thiamine, intravenously for 7 days with 3 days of high-dose intravenous corticosteroids, he remained in an apathetic state for more than 3 months with long- and short-term memory deficits.

Discussion

The main pathologic findings in MBD consist of symmetrical demyelination and necrosis of the central part of the corpus callosum, with relative sparing of thin upper and lower edges (5). The diagnosis of MBD rests mainly on evidence of these callosal lesions. The corpus callosum may also be affected in other diseases such as ischemic stroke, contusion, multiple sclerosis, and lymphoma. MBD, however, is distinguished from these disorders by the symmetry of the callosal lesions (6). MR imaging is the best technique with which to evaluate such MBD lesions, and it showed these neuroradiologic features characteristic of MBD in both our patients.

A highly unusual feature in our patients was the diffuse cortical abnormality of the lateral-frontal cerebral cortices. In a few cases of Wernicke’s encephalopathy, another alcoholism-induced encephalopathy, characterized by lesions involving medial thalami, mamillary bodies, and periaqueductal brain stem, cortical abnormality has been described (7). Our patients’ MR images did not show the midline lesions characteristic of Wernicke’s encephalopathy. The coexistence of subclinical or an extremely mild form of Wernicke’s encephalopathy cannot be ruled out in our patients. With Wernicke’s encephalopathy, the cortical lesions are usually restricted to the motor and premotor cortices (7). Thus, our patients’ diffuse cortical lesions are more likely associated with MBD than with Wernicke’s encephalopathy.

Postmortem neuropathologic study in patients with MBD sometimes reveals a type of cerebral cortical lesion (8, 9). This cortical lesion, known as Morel’s laminar sclerosis, is characterized by cortical laminar necrosis and gliosis, mainly in the third layer and especially in the lateral-frontal cortex (10). Although Morel’s laminar sclerosis was reported as the only manifestation of alcoholic encephalopathy in one patient (11), it is usually associated with, and probably secondary to, the callosal lesions of MBD (10).

In contrast to postmortem neuropathologic study findings, cortical abnormality in MBD is rarely reported on the basis of intravital neuroradiologic examination. There is one report of a severe positron-emission tomography–detected decrease in cortical metabolism in a patient with MBD (12). Cortical abnormality detected by MR imaging, however, has not been reported previously in patients with MBD. Morel’s laminar sclerosis is seen mainly in the lateral-frontal cortices (10). Our patients’ MR imaging abnormalities were also seen mainly in the lateral-frontal cortices and thus may reflect Morel’s laminar sclerosis. If this assumption is correct, a high signal intensity on diffusion-weighted images with relatively reduced ADC in the cortical lesions (Fig 3) suggests the pathologic change of the acute phase of Morel’s laminar sclerosis to be a cytotoxic edema; however, further studies are necessary to delineate the pathologic details of cortical lesions associated with MBD.

References

  1. ↵
    Koeppen AH, Barron KD. Marchiafava-Bignami disease. Neurology 1978;28:290–294
    Abstract/FREE Full Text
  2. ↵
    Gambini A, Falini A, Moiola L, Comi G, Scotti G. Marchiafava-Bignami disease: longitudinal MR imaging and MR spectroscopy study. AJNR Am J Neuroradiol 2003;24:249–253
    Abstract/FREE Full Text
  3. ↵
    Arbelaez A, Pajon A, Castillo M. Acute Marchiafava-Bignami disease: MR findings in two patients. AJNR Am J Neuroradiol 2003;24:1955–1957
    Abstract/FREE Full Text
  4. ↵
    Ruiz-Martínez J, Martínez Pérez-Balsa A, Ruibal M, et al. Marchiafava-Bignami disease with widespread extracallosal lesions and favourable course. Neuroradiology 1999;41:40–43
    CrossRefPubMed
  5. ↵
    Harper C, Butterworth R. Nutritional and metabolic disorders. In: Graham DI, Lantos PL, eds. Greenfield’s Neuropathology. 6th ed. London: Arnold,1997 :616–617
  6. ↵
    Friese SA, Bitzer M, Freudenstein D, et al. Classification of acquired lesions of the corpus callosum with MRI. Neuroradiology 2000;42:795–802
    CrossRefPubMed
  7. ↵
    Yamashita M, Yamamoto T. Wernicke encephalopathy with symmetric pericentral involvement: MR findings. J Comput Assist Tomogr 1995;19:306–308
    PubMed
  8. ↵
    Sato Y, Tabira T, Tateishi J. Marchiafava-Bignami disease, striatal degeneration, and other neurological complications of chronic alcoholism in a Japanese. Acta Neuropathol (Berl) 1981;83:15–20
  9. ↵
    Regadera JF, Enríquez R, Morales M, et al. Associated Marchiafava-Bignami disease, central pontine myelinolysis and Morel’s laminar sclerosis: a case report [in Spanish]. Med Clin (Barc) 1984;82:117–120
    PubMed
  10. ↵
    Poirier J, Gray F, Escourolle R. Neuropathology of general pathological processes. In: Manual of basic neuropathology. Philadelphia: WB Saunders;1990 :163–179
  11. ↵
    Naeije R, Franken L, Jacobovitz D, Flament-Durand J. Morel’s laminar sclerosis. Eur Neurol 1978;17:155–159
    PubMed
  12. ↵
    Logak M, Fève A, Samson Y, et al. Contribution of positron emission tomography in a patient with Marchiafava Bignami disease: laminar sclerosis of Morel [in French]? Rev Neurol (Paris) 1996;152:47–50
    PubMed
  • Received April 16, 2004.
  • Accepted after revision June 18, 2004.
  • Copyright © American Society of Neuroradiology
View Abstract
PreviousNext
Back to top

In this issue

American Journal of Neuroradiology: 26 (3)
American Journal of Neuroradiology
Vol. 26, Issue 3
1 Mar 2005
  • Table of Contents
  • Index by author
Advertisement
Print
Download PDF
Email Article

Thank you for your interest in spreading the word on American Journal of Neuroradiology.

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
Cortical Involvement in Marchiafava-Bignami Disease
(Your Name) has sent you a message from American Journal of Neuroradiology
(Your Name) thought you would like to see the American Journal of Neuroradiology web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Cite this article
Ken Johkura, Makoto Naito, Takayuki Naka
Cortical Involvement in Marchiafava-Bignami Disease
American Journal of Neuroradiology Mar 2005, 26 (3) 670-673;

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
0 Responses
Respond to this article
Share
Bookmark this article
Cortical Involvement in Marchiafava-Bignami Disease
Ken Johkura, Makoto Naito, Takayuki Naka
American Journal of Neuroradiology Mar 2005, 26 (3) 670-673;
del.icio.us logo Twitter logo Facebook logo Mendeley logo
  • Tweet Widget
  • Facebook Like
  • Google Plus One

Jump to section

  • Article
    • Abstract
    • Case Reports
    • Discussion
    • References
  • Figures & Data
  • Info & Metrics
  • Responses
  • References
  • PDF

Related Articles

  • No related articles found.
  • PubMed
  • Google Scholar

Cited By...

  • Partial interhemispheric disconnection syndrome (P-IHDS) secondary to Marchiafava-Bignami disease type B (MBD-B)
  • Diagnosis and management of Marchiafava-Bignami disease: a review of CT/MRI confirmed cases
  • Marchiafava-Bignami disease: Diffusion-weighted MRI in corpus callosum and cortical lesions
  • Crossref
  • Google Scholar

This article has not yet been cited by articles in journals that are participating in Crossref Cited-by Linking.

More in this TOC Section

  • Predictors of Reperfusion in Patients with Acute Ischemic Stroke
  • Enhanced Axonal Metabolism during Early Natalizumab Treatment in Relapsing-Remitting Multiple Sclerosis
  • Progression of Microstructural Damage in Spinocerebellar Ataxia Type 2: A Longitudinal DTI Study
Show more BRAIN

Similar Articles

Advertisement

Indexed Content

  • Current Issue
  • Accepted Manuscripts
  • Article Preview
  • Past Issues
  • Editorials
  • Editor's Choice
  • Fellows' Journal Club
  • Letters to the Editor
  • Video Articles

Cases

  • Case Collection
  • Archive - Case of the Week
  • Archive - Case of the Month
  • Archive - Classic Case

More from AJNR

  • Trainee Corner
  • Imaging Protocols
  • MRI Safety Corner
  • Book Reviews

Multimedia

  • AJNR Podcasts
  • AJNR Scantastics

Resources

  • Turnaround Time
  • Submit a Manuscript
  • Submit a Video Article
  • Submit an eLetter to the Editor/Response
  • Manuscript Submission Guidelines
  • Statistical Tips
  • Fast Publishing of Accepted Manuscripts
  • Graphical Abstract Preparation
  • Imaging Protocol Submission
  • Evidence-Based Medicine Level Guide
  • Publishing Checklists
  • Author Policies
  • Become a Reviewer/Academy of Reviewers
  • News and Updates

About Us

  • About AJNR
  • Editorial Board
  • Editorial Board Alumni
  • Alerts
  • Permissions
  • Not an AJNR Subscriber? Join Now
  • Advertise with Us
  • Librarian Resources
  • Feedback
  • Terms and Conditions
  • AJNR Editorial Board Alumni

American Society of Neuroradiology

  • Not an ASNR Member? Join Now

© 2025 by the American Society of Neuroradiology All rights, including for text and data mining, AI training, and similar technologies, are reserved.
Print ISSN: 0195-6108 Online ISSN: 1936-959X

Powered by HighWire