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Table of Contents    
LETTERS TO EDITOR
Year : 2019  |  Volume : 67  |  Issue : 1  |  Page : 321-323

MR imaging in the diagnosis of Marchiafava–Bignami syndrome


Department of Radiology and Imaging, Holy Family Hospital and Medical Research Centre, Mumbai, Maharashtra, India

Date of Web Publication7-Mar-2019

Correspondence Address:
Dr. Raina A Tembey
Department of Radiology and Imaging, Holy Family Hospital and Medical Research Centre, Bandra, Mumbai - 400 050, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.253648

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How to cite this article:
Tembey RA, Karnik A, Mani SA. MR imaging in the diagnosis of Marchiafava–Bignami syndrome. Neurol India 2019;67:321-3

How to cite this URL:
Tembey RA, Karnik A, Mani SA. MR imaging in the diagnosis of Marchiafava–Bignami syndrome. Neurol India [serial online] 2019 [cited 2019 May 21];67:321-3. Available from: http://www.neurologyindia.com/text.asp?2019/67/1/321/253648




Sir,

Marchiafava–Bignami syndrome (MBS) is a rare demyelinating disorder seen in chronic alcoholics that is characterized by corpus callosal necrosis and associated white matter changes.[1] Although the clinical presentation and symptoms are variable and often nonspecific, the cross-sectional imaging findings are fairly specific and can help in making a diagnosis in the appropriate clinical setting.

The usual chronology of pathological events in MBS is inflammation (acute phase) followed by demyelination, and eventually necrosis and axonal loss. It is usually seen in chronic alcoholics and is usually caused by deficiency of vitamin B complex.[2]

The clinical features include cognitive impairment, neuropsychiatric features, dysarthria, seizures, pyramidal signs, hypertonia, and signs of interhemispheric disconnection. The clinical subtypes are type A, type B, and chronic. The main clinical features of type A are coma and stupor; type B patients have slightly impaired level of consciousness; and chronic cases mainly have cognitive impairment.[2]

Magnetic resonance imaging (MRI) can identify the different radiological stages of the disease ranging from the early stages of diffuse edema of the corpus callosum to the later stages of atrophy and necrosis.

The key finding is demyelination of the corpus callosum, with MRI findings revealing edema of the corpus callosum in the acute phase seen as restricted diffusion on diffusion weighted imaging (DWI).[3] These changes are seen on conventional sequences as well, with an hypointense signal involving the corpus callosum on T1-weighted images (WI), and hyperintense signals on T2WI and fluid attenuated inversion recovery (FLAIR) imaging.[3],[4] The acute phase may show enhancement of the affected areas on contrast administration.[1] Eventually, follow-up MRI shows cystic changes and atrophy in the chronic phase [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7].[4] Though the condition primarily affects the corpus callosum, lesions may also be found in the corticospinal tracts, hemispheric white matter, and middle cerebellar peduncles.
Figure 1: (a and b) Case 1: Diffusion weighted imaging (DWI) shows bright signals, with dark areas on ADC maps, suggestive of restricted diffusion in the genu and splenium of the corpus callosum

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Figure 2: (a and b) Case 1: The corpus callosal lesions show hypointense signals on T1WI and hyperintense signals on T2WI with involvement of the central layers (sandwich sign)

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Figure 3: Post contrast T1 weighted image shows no abnormal enhancement of the affected areas

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Figure 4: Case 1:Follow up MRI done after 6 weeks. T2WI demonstrates cystic changes in the corpus callosum

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Figure 5: (a and b) Case 2: Diffusion weighted imaging (DWI) shows confluent bright signals, with dark areas on ADC maps, suggestive of restricted diffusion involving almost the entire corpus callosum

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Figure 6: (a and b) Case 2: The corpus callosal lesions show hypointense signals on T1WI and hyperintense signals on T2WI

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Figure 7: Case 2: Post contrast T1 weighted image shows a mild patchy enhancement in the affected areas

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These lesions are best seen on MRI and not very accurately on computed tomography (CT), where they may appear hypoattenuated. The corpus callosal lesions are midline and symmetrical in location.[4]

MBS should be considered in the evaluation of alcoholic or malnourished patients who present with acute or subacute neurological symptoms. With the early detection and treatment, the prognosis of MBS may be good even in cases with severe diffusion restriction of complete corpus callosum.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
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. 1
    
2.
Heinrich A, Runge U, Khaw AV. Clinicoradiologic subtypes of Marchiafava-Bignami disease. J Neurol 2004;251:1050-9.  Back to cited text no. 2
    
3.
Hillbom M, Saloheimo P, Fujioka S, Wszolek ZK, Juvela S, Leone MA. Diagnosis and Management of Marchifava-Bignami Disease: A review of CT/MRI confirmed cases. J Neurol Neurosurg Psychiatry 2014;85:168-73.  Back to cited text no. 3
    
4.
Friese SA, Bitzer M, Freudenstein D, Voigt K, Kuker Wl. Classification of acquired lesions of the corpus callosum with MRI. Neuroradiology 2000;42:795-802.  Back to cited text no. 4
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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