A 32 year old man with COPA syndrome characterized by interstitial lung disease, rheumatoid arthritis, and glomerulonephritis, presented with headache, right hemibody paresthesias, and visual scotoma.  COPA syndrome is an autosomal dominant immune dysregulatory disease which has not historically been associated with intracranial pathology. 

On presentation, neurologic exam demonstrated a left homonymous visual deficit.   Initial MRI revealed acute infarcts in the right occipital lobe.  EEG was without epileptiform findings.  Expansion of scotoma prompted repeat MRI with new right occipital lobe infarcts and wall enhancement on vessel imaging.   Angiogram was performed and demonstrated a right posterior cerebral artery fusiform aneurysm; he lacked other vascular risk factors.  Intervention was not recommended because of the concern for causing further infarcts given the fusiform shape.  Since then, he has had no further infarcts on dual antiplatelet therapy and immunosuppression. 

Written informed consent was obtained from the patient for access to clinical files and imaging for research purposes and for publication. 

COPA syndrome is an autosomal dominant mutation causing primary immunodefiencies that lead to immune dysregulation and autoinflammation.  This case is the first description in the literature of intracranial aneurysm with vessel enhancement supporting autoinflammation as the cause of ischemic stroke in COPA syndrome.