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|Year : 2021 | Volume
| Issue : 6 | Page : 1619-1620
Primary CNS Vasculitis: Radiopathological Correlation
Darshana Sanghvi1, Annu Aggarwal2, Bijal Kulkarni3, Sweta Singh4, Vatsal Kothari4
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 Submission||26-Jan-2019|
|Date of Decision||18-Jul-2019|
|Date of Acceptance||30-Sep-2019|
|Date of Web Publication||23-Dec-2021|
Dr. Darshana Sanghvi
Departments of Radiology, Kokilaben Dhirubhai Ambani Hospital, Andheri West, Mumbai - 400 072, Maharashtra
Source of Support: None, Conflict of Interest: None
|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
| » Case Summary|| |
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. Brain biopsy [Figure 2] confirmed the diagnosis. Tissue diagnosis is essential 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|| |
Abdel Razek AA, Alvarez H, Bagg S, Refaat S, Castillo M. Imaging spectrum of CNS vasculitis. Radiographics2014;34:873-94.
Appireddy R, Shukla G. Primary central nervous system vasculitis in India -need for a multicenter prospective cohort study. Neurol India 2019;67:115-7.
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[Figure 1], [Figure 2]