Sean Spence1, Denise Ng2, Colin Casault3. 1. Department of Critical Care Medicine, University of Calgary, Calgary, AB T2N 1N4, Canada. 2. Department of Pathology & Laboratory Medicine, University of Calgary, Calgary, AB T2N 1N4, Canada. 3. Department of Critical Care Medicine, University of Calgary, Calgary, AB T2N 1N4, Canada. Electronic address: Colin.Casault@albertahealthservices.ca.
Abstract
BACKGROUND: Primary Angiitis of the Central Nervous System (PACNS) is a rare cause of CNS vasculitis that should be included as part complete differential diagnosis, especially in cases with suggestive imaging findings and an absence of secondary causes for CNS vasculitis. CASE PRESENTATION: We describe a case of a 47-year-old previously healthy Caucasian male presenting with rapid progression of encephalopathy and fevers. Extensive infectious, autoimmune, and imaging workups were unrevealing. A diagnosis of PACNS was made posthumously on histopathology. CONCLUSIONS: PACNS is a challenging diagnosis owing to frequent discrepancies between radiologic and histopathologic findings. Tissue biopsy is key to diagnosing PACNS.
BACKGROUND:Primary Angiitis of the Central Nervous System (PACNS) is a rare cause of CNS vasculitis that should be included as part complete differential diagnosis, especially in cases with suggestive imaging findings and an absence of secondary causes for CNS vasculitis. CASE PRESENTATION: We describe a case of a 47-year-old previously healthy Caucasian male presenting with rapid progression of encephalopathy and fevers. Extensive infectious, autoimmune, and imaging workups were unrevealing. A diagnosis of PACNS was made posthumously on histopathology. CONCLUSIONS: PACNS is a challenging diagnosis owing to frequent discrepancies between radiologic and histopathologic findings. Tissue biopsy is key to diagnosing PACNS.
Authors: Gelsomina Mansueto; Giuseppe Lanza; Francesco Fisicaro; Danielle Alaouieh; Emily Hong; Sara Girolami; Marco Montella; Alessandro Feola; Mario Di Napoli Journal: Curr Neurol Neurosci Rep Date: 2022-02-09 Impact factor: 6.030