Introduction Cerebral amyloid angiopathy (CAA) is generally a non‐inflammatory process more common in older patients. Rarely, the inflammatory subtype, Amyloid Beta Related Central Nervous System Angiitis (ABRA) occurs in relatively younger patients presenting with non‐specific symptoms of focal neurological deficits, encephalopathy, and seizures. We present a rare case of ABRA in a patient with a sudden New Onset of Refractory Status Epilepticus (NORSE) initially thought to be Posterior Reversible Encephalopathy Syndrome (PRES). Materials & Methods Consent was obtained. Case A 69‐year‐old female with a history of hypertension presented with new onset seizure with status epilepticus, for which she was intubated and admitted to the intensive care unit. Initial brain Magnetic Resonance Imaging (MRI) demonstrated confluent periventricular hyperintensities in the parietal‐occipital region with associated vasogenic edema, diffuse cortical and subcortical microhemorrhages and hemosiderin concerning for CAA versus PRES. MRA head demonstrated irregular vessels in the anterior and posterior circulation concerning for vasculitis. Cerebral spinal fluid (CSF) was notable for elevated protein 100 and glucose 71. CSF WBC2, negative meningitis/encephalitis panel. ESR 145, CRP 2. 3, elevated IL2 1548/IL‐6 22. 9/TNFa 1. 64, negative rheumatoid Factor, C3, C4, ANA MFIA, dsDNA, ANCA, AntiCardiolipin, SSA, SSB, ACE, AntiTG Ab, and vasculitis panel. The blood pressure was controlled and she was treated with intravenous immunoglobulin (IVIG) and high‐dose steroids. Continuous electroencephalograms showed an active left temporal occipital epileptogenic lesion. Repeat brain MRI demonstrated a small area of acute left temporal occipital infarct and persistent confluent T2/FLAIR hyperintensities with surrounding vasogenic edema. Multiple anti‐epileptic medications were therapeutically titrated including brivaracetam, valproate, lacosamide, levetiracetam, oxcarbazepine, phenobarbital, topiramate, and sedation with midazolam and propofol. Hospital course was complicated by pneumonia, hyperammonemia, hyponatremia, atrial fibrillation with rapid ventricular rate and, ultimately, a fatal cardiac arrest. Conclusion This case highlights the diagnostic and therapeutic challenges of ABRA, a rare and inflammatory subtype of CAA, which can present with severe neurological manifestations such as NORSE. To date, there is one similar case in the literature of ABRA presenting as NORSE complicated by psychosis, dysautonomia, and PRES. The initial presentation mimicking PRES underscores the difficulty in distinguishing ABRA from other neurovascular conditions. Despite aggressive immunotherapy and antiepileptic management, the patient's condition deteriorated, ultimately leading to a fatal outcome. This case emphasizes the importance of early recognition and tailored treatment strategies in managing ABRA, while also acknowledging the potential for poor prognosis despite optimal care. Further research is needed to better understand the pathophysiology and treatment approaches for this rare but serious condition. References 1. de Souza, A. , & Tasker, K. (2023). Inflammatory cerebral amyloid angiopathy: a broad clinical spectrum. Journal of Clinical Neurology (Seoul, Korea), 19 (3), 230. 2. Ishii, M. , Lavi, E. , Kamel, H. , Gupta, A. , Iadecola, C. , & Navi, B. B. (2014). Amyloid β‐related central nervous system angiitis presenting with an isolated seizure. The Neurohospitalist, 4 (2), 86‐89. 3. Prasad, A. , Sharma, S. , Schmidt, E. , & Latorre, J. (2016). (PRES) (P6. 219). Neurology, 86 (16ₛupplement), P6‐219.
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