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|Year : 2017 | Volume
| Issue : 6 | Page : 1440-1441
Marchiafava Bignami disease
Rakesh Malhotra1, Tushar Chopra2, Omar Al Nimri3
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 Publication||10-Nov-2017|
Dr. Rakesh Malhotra
Department of Medicine, University of California San Diego, San Diego, California
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Malhotra R, Chopra T, Al Nimri O. Marchiafava Bignami disease. Neurol India 2017;65:1440-1
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). 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|
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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.
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Conflicts of interest
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| » References|| |
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.