A Devastating Detour: When Immune Checkpoint Inhibitors Triggers CNS Vasculitis
Abhiram Parameswaran1, Abhigyan Datta1, Katherine Cooke1, Teoh Deanna2, Praveen Hariharan1
1University of Minnesota, 2Gynecology-Oncology, University of Minnesota
Objective:
Immune Checkpoint inhibitors (ICI) are increasingly used to treat solid organ tumors, but they can cause a variety of immune-related adverse events (irAEs). Neurologic irAEs are reported in about 1-12% of patients, with central nervous system (CNS) vasculitis being rare. Here, we present a case of ICI-related CNS vasculitis to increase awareness of this devastating complication. 
Background:

A 36-year-old female presented with acute onset right face and left hemibody numbness/weakness and was found to have scattered subacute right hemispheric infarcts. MRA revealed near occlusion of right ICA terminus with distal reconstitution of right MCA and severe left ACA stenosis. She had been diagnosed with Stage IVB cervical cancer, 9 months prior, and was treated with Cisplatin/Paclitaxel/Bevacizumab for 3 cycles with addition of Pembrolizumab for cycles 4-6, followed by maintenance therapy for 5 cycles prior to presentation. One month prior to presentation she developed immune-mediated gastritis/pancreatitis, leading to the discontinuation of Pembrolizumab and subsequent initiation of a steroid tape.

On comprehensive stroke evaluation, cerebrospinal fluid analysis showed normal protein (20 mg/dL), cell count(2/mm3), negative meningitis panel, and cytology/flow cytometry. Vessel wall imaging revealed extensive concentric vessel wall enhancement in right ICA/MCA. Skin biopsy showed no evidence of intravascular lymphoma. Given high suspicion for ICI-related vasculitis, she was treated with intravenous methylprednisone 1g daily for 5 days. Subsequently, Due to worsening of right hemispheric infarcts, she received cyclophosphamide induction therapy and 5 sessions of plasma exchange over the next 2 weeks. Ultimately she required decompressive hemicraniectomy. She remained clinically stable for the next 2 weeks and was discharged on prolonged steroid taper. At follow-up, she remained clinically/radiographically stable with residual left-sided weakness.

Design/Methods:
NA
Results:
NA
Conclusions:
Early recognition and aggressive management are crucial to improving outcomes in ICI-related CNS vasculitis. The diagnosis should be considered in patients with recent ICI initiation and concurrent systemic irAEs. 
10.1212/WNL.0000000000210858
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