Cerebral Amyloid Angiopathy-related Inflammation: A Spectrum of Presentation and Response to Therapy in Three Patients
Objective:
To describe the presentations, treatments, and outcomes of three female patients diagnosed with Cerebral amyloid angiopathy–related inflammation (CAA-rI) between 2021 and 2025.
Background:
CAA-rI is a rare but potentially treatable condition, with an estimated prevalence of 0.13 per 100,000. It presents with rapidly progressive cognitive decline, seizures, headache, or focal deficits, and is characterized radiologically by asymmetric white matter hyperintensities with lobar microbleeds or cortical superficial siderosis. Although biopsy is the gold standard, most cases are diagnosed using clinico-radiological criteria.
Results:
Patient 1, an 81-year-old woman with two years of cognitive decline and prior lobar hemorrhages, developed focal status epilepticus. MRI revealed new white matter changes and leptomeningeal enhancement. She improved after intravenous methylprednisolone and remained stable without maintenance immunosuppression.
Patient 2, an 81-year-old woman with a slowly progressive cognitive decline, was admitted for an acute confusional episode. MRI revealed diffuse vasogenic edema, scattered microhemorrhages, and minimal leptomeningeal enhancement. She was treated with intravenous methylprednisolone followed by a short oral steroid taper, resulting in clinical improvement. However, she developed recurrent symptoms requiring a longer steroid taper over a year, ultimately achieving clinical and radiological stability.
Patient 3, a 72-year-old woman with a fulminant course with recurrent subarachnoid hemorrhage, seizures, and encephalopathy. MRI revealed leptomeningeal enhancement and infarcts; biopsy confirmed CAA-rI. She deteriorated despite intravenous methylprednisolone, requiring cyclophosphamide, which was complicated by pancytopenia. After three cycles and a prolonged steroid taper, she showed modest functional improvement but remained severely disabled.
Conclusions:
Prompt diagnosis and early initiation of corticosteroid therapy are crucial in CAA-rI, as timely immunosuppression is strongly associated with improved clinical and radiological outcomes, resulting in reduced recurrence rates. Further studies are needed to identify effective second-line therapies for patients unresponsive to corticosteroids and to determine the ideal treatment duration. This underscores the importance of research to improve long-term outcomes in CAA-rI.
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