Atormac
Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 1916  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
 Search
 
  
 Resource Links
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Article in PDF (870 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this Article
   References
   Article Figures

 Article Access Statistics
    Viewed1228    
    Printed29    
    Emailed0    
    PDF Downloaded44    
    Comments [Add]    

Recommend this journal

 


 
Table of Contents    
NEUROIMAGES
Year : 2017  |  Volume : 65  |  Issue : 6  |  Page : 1440-1441

Marchiafava Bignami disease


1 Department of Medicine, University of California San Diego, San Diego, California, USA
2 Department of Medicine, University of Virginia, Charlottesville, Virginia, USA
3 Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee, USA

Date of Web Publication10-Nov-2017

Correspondence Address:
Dr. Rakesh Malhotra
Department of Medicine, University of California San Diego, San Diego, California
USA
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.217948

Rights and Permissions



How to cite this article:
Malhotra R, Chopra T, Al Nimri O. Marchiafava Bignami disease. Neurol India 2017;65:1440-1

How to cite this URL:
Malhotra R, Chopra T, Al Nimri O. Marchiafava Bignami disease. Neurol India [serial online] 2017 [cited 2019 Oct 13];65:1440-1. Available from: http://www.neurologyindia.com/text.asp?2017/65/6/1440/217948


A 79-year old, right-handed male, with a history of chronic alcohol use and prostate cancer, presented with subacute alteration of mental status and progressive inability to walk. On neurological examination, the patient was alert but oriented only to person. He had left-sided neglect, reduced sensation on the left side of the body, and stiffness with 4/5 strength in bilateral lower extremities. Magnetic resonance imaging (MRI) of the brain revealed diffusion restriction and T2 and fluid attenuated inversion recovery (FLAIR) hyperintensities in the left genu and bilateral splenium of corpus callosum as well as medial bilateral parietal lobe without abnormal enhancement [Figure 1]a and [Figure 1]b. The patient was positive for Anti-65-kDa anti-glutamate decarboxylase antibody (GAD) antibody. Treatment was initiated with high-dose corticosteroid and intravenous (IV) thiamine followed by intravenous immunoglobulin IVIG) 0.4 g/kg/day for 10 days every alternate day, with no significant cognitive and neurological improvement. The patient subsequently died of pulmonary edema. The neuropathologic findings on autopsy included degeneration and necrosis of the corpus callosum, degeneration of the mammillary body, and cerebellar degeneration affecting the superior and inferior cerebellar vermis, which was consistent with the diagnosis of Marchiafava -Bignamy disease (MBD).[1] The diagnosis of MBD has always been a clinical challenge with its varied clinical presentations. The potential role of the neoplastic disease contributing to the dramatic clinical course of MBD in this patient remains speculative. The combination of patient's clinical history, including prior history of malignancy, alcohol use, paraneoplastic autoantibody serum evaluation, physical examination, and characteristic MRI and autopsy findings facilitated the accurate diagnosis of MBD.
Figure 1: (a and b) Magnetic resonance imaging (MRI) of the brain revealed diffusion restriction and T2 and fluid attenuated inversion recovery (FLAIR) hyperintensities in the left genu and bilateral splenium of corpus callosum as well as medial bilateral parietal lobe without abnormal enhancement

Click here to view


Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Hillbom M, Saloheimo P, Fujioka S, Wszolek ZK, Juvela S, Leone MA. Diagnosis and management of Marchiafava-Bignami disease: A review of CT/MRI confirmed cases. J Neurol Neurosurg Psychiatry 2014;85:168-73.  Back to cited text no. 1
    


    Figures

  [Figure 1]



 

Top
Print this article  Email this article
   
Online since 20th March '04
Published by Wolters Kluwer - Medknow