Immune-related adverse events (irAEs) are a frequent complication of immunotherapy, but neurological irAEs are rare and varied. Here, we present a case of cerebral amyloid angiopathy-related inflammation (CAA-ri) attributable to nivolumab monotherapy, which has not been previously reported. The context of immunotherapy and availability of serial imaging also provide unique insights into the pathogenesis and evolution of CAA-ri. Routine surveillance neuroimaging in a patient with metastatic melanoma, in remission after treatment with nivolumab, demonstrated progressive microhaemorrhages and associated oed ema, suspicious for CAA-ri. These changes progressed despite cessation of nivolumab. The patient was initially asymptomatic, but later developed an acute confusional state, warranting brain biopsy, which confirmed the diagnosis of CAA-ri. Treatment with methylprednisolone resulted in resolution of the oedema, and a marked decrease in the subsequent accumulation of microhaemorrhages. The temporal evolution prior to symptom development and subsequently related to treatment suggests that inflammation may be an important component of the pathogenesis of CAA-ri, rather than simply a secondary response. Given that immunotherapy is in its relative infancy, it is important to consider rare irAEs in patients exhibiting unusual imaging findings.
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