Hemorrhagic Leukoencephalopathy Attributable to Cerebral Amyloid Angiopathy– Related Inflammation (CAA-ri): Therapeutic Lessons from Immunosuppressive Response
Siddhant Arora1, Supreet Kaur1, Simranpreet Singh2
1Barrow Neurological Institute, 2Tower Health Reading Hospital
Objective:
To present a case of hemorrhagic leukoencephalopathy due to cerebral amyloid angiopathy–related inflammation (CAA-ri) and highlight the critical role of gradual corticosteroid tapering following high-dose pulse therapy to prevent relapses and ensure optimal clinico-radiologic recovery.
Background:
CAA-ri is an immune-mediated, reversible variant of cerebral amyloid angiopathy presenting with subacute cognitive decline, focal deficits, asymmetric vasogenic edema, cortical microbleeds, and/or leptomeningeal enhancement on imaging. Large cohort studies confirm a transient, treatment-responsive yet relapsing course, with rapid tapering of corticosteroids being a major risk factor for relapse and worsening neurological function. The largest longitudinal cohort registry of patients with CAA-ri showed that slow oral tapering after intravenous corticosteroid pulse therapy effectively prevents recurrences. Antolini et al. (2021) reported 70% clinical recovery with IV corticosteroids, but relapse occurred in 38% after abrupt discontinuation; slow tapering markedly reduced recurrence (HR 4.68; p = 0.006).
Design/Methods:
A 74-year-old man with chronic lymphocytic leukemia in remission presented with subacute cognitive decline and left-sided weakness. MRI Brain showed asymmetric hemorrhagic leukoencephalopathy with cortical microbleeds and leptomeningeal enhancement. CSF revealed mild lymphocytic pleocytosis (17 cells/μL) and negative flow cytometry/ infectious/ paraneoplastic and autoimmune workup. Brain biopsy demonstrated nonspecific inflammation and was negative for amyloid; however, the clinico-radiological findings fulfilled the criteria. He improved after IV methylprednisolone (1 g/day × 5 days) but relapsed twice following abrupt discontinuation. A slow oral prednisone taper (≈1 mg/kg/day, reduced by 5–10 mg/ month, then more gradually at lower doses) led to sustained clinical and radiologic recovery.
Conclusions:
CAA-ri is an inflammatory variant of Cerebral amyloid angiopathy that responds well to early recognition and prompt corticosteroid treatment. While high-dose pulse steroid therapy leads to clinico-radiological improvement, relapses are common after rapid steroid withdrawal, highlighting the importance of a gradual taper to prevent recurrence, reduce hospitalizations, and improve neurological outcomes.
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