Abstract Details

Title Navigating the Complexities of CNS vasculitis

Topic Cerebrovascular Disease and Interventional Neurology

Presentation(s) P11 - Poster Session 11 (8:00 AM-9:00 AM)

Poster/Presentation
Number 14-002

Objective CNS vasculitis, encompassing both primary (PACNS) and secondary forms, presents significant diagnostic difficulties due to its complex pathophysiology and overlapping clinical manifestations. Despite advancements in imaging techniques, serum analysis, and CSF evaluation, establishing a definitive diagnosis often remains elusive. This case report aims to highlight the challenges associated with diagnosing CNS vasculitis, including its unique clinical features and the complexities of differentiating between PACNS and secondary CNS vasculitis.

Background A 56-year-old female with a long-standing history of stable systemic lupus erythematosus (SLE), managed on immunosuppressive therapy without clinical flares, presented with recurrent cryptogenic ischemic strokes over five years. Her most recent admission was prompted by worsening right-sided weakness, with imaging revealing additional ischemic strokes and SAH, raising concerns for CNS vasculitis.

Design/Methods NA

Results In this case, a comprehensive workup, including serum, CSF analysis, and advanced imaging, was performed. Serum and CSF findings were largely unremarkable. MRA with "black blood protocol" showed concentric enhancement of the MCA and PCA bilaterally, and conventional angiography demonstrated bilateral tapering of the cerebral arteries. Although the patient lacked active systemic sings typically associated with secondary CNS vasculitis, the absence of a brain biopsy remains a significant limitation for establishing a definitive diagnosis. Nevertheless, a treatment plan consisting of high-dose corticosteroids and cyclophosphamide, followed by a steroid taper, was initiated, as it is suitable for managing both PACNS and CNS lupus vasculitis. The patient showed improvement and continued to be stable under ongoing clinical monitoring.

Conclusions PACNS and CNS lupus vasculitis are rare but serious conditions with overlapping clinical features, making differentiation between them a significant challenge. While their acute management and relapse prevention strategies are often similar, distinguishing between these entities is critical for guiding appropriate decisions. Continuous monitoring for disease activity, management of treatment-related adverse effects, and long-term follow-up are essential to mitigate relapses and address complications.

Authors/Disclosures
Alexander Vorobyev, MD (Medical University of South Carolina) PRESENTER	Dr. Vorobyev has nothing to disclose.
Parneet K. Grewal, MD	The institution of Dr. Grewal has received research support from Bristol Myer Squibb Foundation. The institution of Dr. Grewal has received research support from IPSEN Global.
Chirantan Banerjee, MD, MPH (Medical University of South Carolina)	Dr. Banerjee has nothing to disclose.
Gina S. Perez-Giraldo, MD	Dr. Perez-Giraldo has received personal compensation for serving as an employee of TG therapeutics. Dr. Perez-Giraldo has received personal compensation in the range of $500-$4,999 for serving as a Consultant for TG therapeutics.
Ghada A. Mohamed, MD	Dr. Mohamed has nothing to disclose.

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