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NEUROIMAGE
Year : 2021  |  Volume : 69  |  Issue : 6  |  Page : 1627-1628

Classical Imaging Finding in Marchiafava Bignami Disease


1 Department of Diagnostic and Interventional Radiology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
2 Department of Psychiatry, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India

Date of Submission12-Apr-2020
Date of Acceptance09-Jun-2020
Date of Web Publication23-Dec-2021

Correspondence Address:
Dr. Sarbesh Tiwari
Department of Diagnostic and Interventional Radiology, All India Institute of Medical Sciences, Jodhpur - 342 005, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.333487

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How to cite this article:
Tiwari S, Dubey P, Swami MK, Yadav T. Classical Imaging Finding in Marchiafava Bignami Disease. Neurol India 2021;69:1627-8

How to cite this URL:
Tiwari S, Dubey P, Swami MK, Yadav T. Classical Imaging Finding in Marchiafava Bignami Disease. Neurol India [serial online] 2021 [cited 2022 Jan 18];69:1627-8. Available from: https://www.neurologyindia.com/text.asp?2021/69/6/1627/333487




A 35-year-old man with a history of chronic alcohol abuse and opioid dependence presented to the de-addiction clinic with complaints of forgetfulness of one year, ataxia, and hand tremors of 1-month duration. On examination, the patient was emaciated with low body mass index (15.2 kg/m2). The patient had severe cognitive impairment (mini-mental state examination score -16) and Romberg's sign was positive.

The presence of significant cognitive impairment and ataxia prompted neuroimaging. MRI brain revealed restricted diffusion involving the body and splenium of the corpus callosum on the diffusion-weighted images (DWI) suggestive of cytotoxic edema [Figure 1]a and [Figure 1]b. The involved region of corpus callosum showed hyperintense signal intensity on T2/FLAIR images [Figure 1]c and [Figure 1]d and hypointense signal on T1 weighted images [Figure 1]e without any contrast enhancement. There was selective involvement of the central layers, with relative sparing of the dorsal and ventral layers of the corpus callosum, described in the literature as “sandwich sign” [Figure 1]f and [Figure 1]g. These imaging features were consistent with the diagnosis of Marchiafava-Bignami disease.
Figure 1: The axial Trace (a) and ADC (b) DWI images shows restricted diffusion involving the splenium and the body of corpus callosum. The involved region of corpus callosum displays a hyperintense signal on T2WI (c) and FLAIR images (d) and appears hypointense on T1WI (e). The sagittal T1 and FLAIR images (f and g) shows involvement of the central portion of corpus callosum with sparing of the dorsal and ventral layers, called sandwich sign

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Marchiafava-Bignami disease is a rare condition characterized by demyelination and necrosis of the corpus callosum, generally associated with chronic alcohol use and malnourishment.[1] The etiology is still unclear, presumably related to alcohol-related neurotoxicity and vitamin B complex deficiency.[2] In the early stage of the disease, cytotoxic edema plays a dominant role followed by demyelination and necrosis in the later stage.[3] In acute stage, patient presents with mental confusion, disorientation, neurocognitive deficits, and seizures. The subacute disease is characterized by dementia, dysarthria, and muscle hypertonia and the chronic form manifests with chronic dementia.[4]

Diagnosis heavily relies on MRI imaging in appropriate clinical background. The characteristic site of involvement is the corpus callosum, especially the central portion. Extracallosal lesions involving hemispheric white matter and cerebral lobes are described and indicates a poor prognosis.[5] During the acute phase, peripheral contrast enhancement of these lesions can be seen with restricted diffusion, suggesting cytotoxic edema. DWI is the best MRI sequence in early phase of the disease. The chronic state of the disease is characterized by cystic change and atrophy of the corpus callosum.[1],[5] The middle layer of corpus callosum is the most affected, with selective sparing of the dorsal and ventral layers (aptly termed the sandwich sign).

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  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
    
2.
Arbelaez A, Pajon A, Castillo M. Acute Marchiafava-Bignami disease: MR findings in two patients. AJNR Am J Neuroradiol 2003;24:1955-7.  Back to cited text no. 2
    
3.
Tung CS, Wu SL, Tsou JC, Hsu SP, Kuo HC, Tsui HW. Marchiafava-Bignami disease with widespread lesions and complete recovery. AJNR Am J Neuroradiol 2010;31:1506-7.  Back to cited text no. 3
    
4.
Heinrich A, Runge U, Khaw AV. Clinicoradiologic subtypes of Marchiafava-Bignami disease. J Neurol 2004;251:1050-9.  Back to cited text no. 4
    
5.
Dong X, Bai C, Nao J. Clinical and radiological features of Marchiafava-Bignami disease. Medicine (Baltimore) 2018;97:e9626.  Back to cited text no. 5
    


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