Alexander Vorobyev1, Parneet Grewal2, Chirantan Banerjee2, Gina Perez-Giraldo2, Ghada Mohamed1
1Medical University of South Carolina, 2Neurology, Medical University of South Carolina
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.
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.
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