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Table of Contents    
NEUROIMAGE
Year : 2021  |  Volume : 69  |  Issue : 6  |  Page : 1619-1620

Primary CNS Vasculitis: Radiopathological Correlation


1 Department of Radiology, KokilabenDhirubhai Ambani Hospital, Andheri West, Mumbai, Maharashtra, India
2 Department of Neurology, KokilabenDhirubhai Ambani Hospital, Andheri West, Mumbai, Maharashtra, India
3 Department of Pathology, KokilabenDhirubhai Ambani Hospital, Andheri West, Mumbai, Maharashtra, India
4 Department of Critical Care, KokilabenDhirubhai Ambani Hospital, Andheri West, Mumbai, Maharashtra, India

Date of Submission26-Jan-2019
Date of Decision18-Jul-2019
Date of Acceptance30-Sep-2019
Date of Web Publication23-Dec-2021

Correspondence Address:
Dr. Darshana Sanghvi
Departments of Radiology, Kokilaben Dhirubhai Ambani Hospital, Andheri West, Mumbai - 400 072, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.333516

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How to cite this article:
Sanghvi D, Aggarwal A, Kulkarni B, Singh S, Kothari V. Primary CNS Vasculitis: Radiopathological Correlation. Neurol India 2021;69:1619-20

How to cite this URL:
Sanghvi D, Aggarwal A, Kulkarni B, Singh S, Kothari V. Primary CNS Vasculitis: Radiopathological Correlation. Neurol India [serial online] 2021 [cited 2022 Jan 19];69:1619-20. Available from: https://www.neurologyindia.com/text.asp?2021/69/6/1619/333516





  Case Summary Top


A 63-year-old woman on hemodialysis presented with fever and non-convulsive-status-epilepsy (Glasgow Coma Scale 12/15).3TMRI [Figure 1] showed acute right parietal ischemic edema with patchy restricted diffusion, microhemorrhages, and sulcal enhancement. Chronic ischemic changes werealso noted in bilateral cerebral parenchyma. Mural thickening and persistent enhancement of large, medium, and small-sized arteries indicated vessel wall inflammation. Delayed FLAIR images showed extensive subarachnoid contrast extravasation representing BBB permeability. Hemogram, vasculitic screen and CSF were normal. Vessel wall inflammation with concomitant parenchymal hemorrhagic and ischemic lesions, sulcal enhancement and subarachnoid contrast extravasation on MRI are diagnostic of primary CNS vasculitis.[1] Brain biopsy [Figure 2] confirmed the diagnosis. Tissue diagnosis is essential[2] as therapy is long-term and associated with serious adverse effects. Patient improved with pulse steroids, plasma exchange, and cyclophosphamide.
Figure 1: DW, FLAIR, SW, and postcontrast T1W MRI show focal right parietal cytotoxic edema (a and b) with petechial hemorrhages (c) and localized sulcal enhancement (d). Delayed contrast T1WI shows mural thickening and persistent enhancement (e) of basilar, carotid, middle, and posterior cerebral arteries. Delayed postcontrast FLAIR image shows diffuse subarachnoid extravasation of contrast (f)

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Figure 2: H and E stain 40×shows small vessel wall neutrophilic infiltration (a) corresponding to sulcal enhancement from pial vessel wall inflammation on MRI [Figure 1]d) and mural enhancement of medium and large vessels [Figure 1]e. Masson trichrome stain 40×shows vessel wall fibrinoid necrosis (b, arrow). Parenchymal RBC extravasation (b, arrowhead) corresponds to blood and contrast extravasation on MRI [Figure 1]c and [Figure 1]f

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  References Top

1.
Abdel Razek AA, Alvarez H, Bagg S, Refaat S, Castillo M. Imaging spectrum of CNS vasculitis. Radiographics2014;34:873-94.  Back to cited text no. 1
    
2.
Appireddy R, Shukla G. Primary central nervous system vasculitis in India -need for a multicenter prospective cohort study. Neurol India 2019;67:115-7.  Back to cited text no. 2
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