Hinal Parmar¹*, Sayali Kalbhor¹, Sudhir Kothari¹ and Deepak Phalgune²

¹Department of Neurology, Poona Hospital and Research Centre, Pune, India
²MBBS, MD (P & SM), PhD, Research Consultant, Dept. of Research, Poona Hospital & Research Centre, Pune, India

*Corresponding Author: Hinal Parmar, Department of Neurology, Poona Hospital and Research Centre, Pune, India.

Received: December 01, 2025; Published: January 06, 2026

Abstract

Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) is a necrotizing small-vessel vasculitis with infrequent central nervous system (CNS) involvement. CNS manifestations may occur through contiguous granulomatous spread, de novo intrac- ranial granuloma formation, or primary vasculitic involvement of cerebral vessels [1], but are reported in only about 15% of patients with AAV [2]. Isolated CNS involvement without systemic features is particularly rare and poses significant diagnostic challenges. We report a 20-year-old female with subacute progressive cerebellar symptoms over six months. Brain MRI demonstrated multiple T2/FLAIR hyperintense lesions involving deep gray matter and brainstem structures, with branching linear and nodular perivascular enhancement along Virchow–Robin spaces, without leptomeningeal enhancement. Cerebrospinal fluid analysis was non-inflamma- tory, infectious and granulomatous etiologies were excluded, and c-ANCA was positive. Based on clinicoradiological correlation and exclusion of alternative diagnoses, a diagnosis of probable c-ANCA–associated CNS vasculitis was made. Treatment with high-dose corticosteroids followed by rituximab resulted in significant clinical improvement.

Keywords: Antineutrophil Cytoplasmic Antibody (ANCA); Vasculitis; AAV; Small Vessels; CNS; Rituximab

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Citation

Citation: Hinal Parmar., et al. “C-Anca Associated Cns Vasculitis - A Rare Presentation". Acta Scientific Neurology 9.2 (2026): 11-15.

Copyright

Copyright: © Hinal Parmar., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.