Abstract 137: Reversible Cerebral Vasoconstriction Syndrome due to Teprotumumab: Two Case Reports

• Published in: Stroke: vascular and interventional neurology Vol. 4; no. S1
• Main Authors: Elfil, M, Lookian, P P, Kumari, K, Aladawi, M, M Jedras, Najdawi, Z, Phillips, S M, Sattur, M G
• Format: Journal Article
• Language: English
• Published: Phoenix Wiley Subscription Services, Inc 01-11-2024
• Subjects: Medical imaging; Stroke
• ISSN: 2694-5746; 2694-5746
• DOI: 10.1161/SVIN.04.suppl_1.137

BackgroundReversible Cerebral Vasoconstriction Syndrome (RCVS) is a rare, yet increasingly recognized condition characterized by segmental narrowing and dilation of cerebral vasculature. We present two novel cases of RCVS possibly linked to teprotumumab; a monoclonal antibody against insulin‐like growth factor 1 receptor (IGF‐1R) used in treating Graves’ eye disease (GED). The possible association between teprotumumab and RCVS prompts the need for increased awareness and further investigation.Case PresentationCase 1A 59‐year‐old female with GED developed acute bilateral leg weakness and severe headache after starting teprotumumab. Computed tomography (CT) showed bilateral hypoattenuation within the paramedian parietal and occipital lobes (Figure 1 A). CT angiography (CTA) showed multifocal stenosis in bilateral middle cerebral arteries (MCAs) and posterior cerebral arteries (PCAs), along with bilateral multifocal luminal irregularity in anterior cerebral arteries (ACAs) (Figure 1 B). Verapamil mitigated vasospasm, but she later presented with convulsive status epilepticus. Subsequent CT showed evolving bilateral infarcts involving the bilateral ACA territories, and CTA demonstrated improved caliber of prior ACA, MCA, and PCA stenosis along with narrowing of distal MCA segments (Figure 2). Digital subtraction angiography (DSA) showed distal segmental narrowing of MCA and PCA cortical branches (Figure 3). Control of seizures and secondary stroke prevention measures were initiated, resulting in a stable discharge to rehabilitation.Case 2A 71‐year‐old female with GED experienced a sudden thunderclap headache two months post‐teprotumumab initiation. Imaging showed cortical subarachnoid hemorrhage (SAH) with diffuse bilateral narrowing of distal MCAs. DSA showed distal segmental narrowing of MCA cortical branches (Figure 4). Verapamil led to clinical improvement, and the patient was discharged without residual deficits.ConclusionsThe temporal correlation between teprotumumab use and RCVS onset in both cases suggests a potential association. As teprotumumab antagonizes IGF‐1R, altering cerebrovascular modulation, we propose a hypothesis linking teprotumumab to RCVS pathogenesis. This report underscores the importance of clinical vigilance for rare complications and the necessity for further research to elucidate the causal relationship, aiding informed decision‐making for patients receiving teprotumumab.