A New Case of Cerbral Angiitis in the ICU

Abstract

SESSION TITLE: Student/Resident Case Report Poster - Critical Care III SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Primary central nervous system vasculitis, also knowns as Primary Angiitis of the Central Nervous system (PACNS), is an extremely rare condition, often presenting with nonspecific findings of worsening headache, confusion, and occasionally visual disturbance.1 Age of onset is often in the 40-50’s with no specific gender predilection.2 Definitive diagnosis requires evidence of isolated CNS non-caseating granulomatous changes, with no other clear cause. We present a case of 47 year old patient, with a presentation initially concerning for vasospastic condition, with Cerebral Angiogram and initial Brain biopsy findings consistent with PACNS. CASE PRESENTATION: 47 year old female patient with a history of chronic low back pain, was admitted to the MICU with two weeks of progressive headaches and acute mental status changes. Initial MRA findings showed diffuse intermittent luminal narrowing throughout the anterior and posterior cerebral vasculature with microinfarction. She was initially placed on Nimodipine to prevent Vasospasm. Cerebral angiogram showed diffuse proximal beading, consistent with vasculitis. She was placed on high dose steroids. Initial lab work up was unremarkable. Anti-nuclear antibody titer, anti-lupus antibodies, and anti-neutrophil cytoplasmic antibodies were all negative. Extensive additional serologic work up was only remarkable for mildly elevated extractable nuclear antigen SSB (LA), but negative for extractable antigen SSA (RO), and otherwise unremarkable. Brain biopsy was performed, and initial read appeared consistent with PACNS. DISCUSSION: PACNS is a rare, but often treatable condition, often presenting to the Medical or neurologic intensive care unit on initial admission. It may be confused with vasospastic conditions, other forms of CNS vasculitis, primary systemic rheumatologic conditions, or stroke. Initial presentation is often non-specific, as was the case with this patient, and requires a high degree of clinical suspicion.3 In this case, cerebral angiogram was necessary to differentiate from a vasospastic condition. Additional serology’s were all negative. Brain biopsy was performed for definitive diagnosis. CONCLUSIONS: PACNS, while rare is an important diagnosis for intensivists in all settings to be aware, that may improve with treatment. Initial diagnosis with cerebral angiogram proved superior to MRI/MRA alone in this case. Serologies and brain biopsy provided an extra level of confirmation of this diagnosis. Reference #1: Bhattacharyya S. Primary angiitis of the Central Nervous System: Avoiding Misdiagnosis. Pract Neurol FEB-2016. Reference #2: Schuster, S. Primary Central Nervous System Vasculitis. Z Rheumatol. 2015 Dec;74. Reference #3: Baja B. Primary angiitis of the Central Nervous system. J Neurosci Rural Pract. 2015 Jul-Sep;6(3):399-401. DISCLOSURE: The following authors have nothing to disclose: Jason Unger, Michael Switzer, Paul Clark No Product/Research Disclosure Information