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Background <p>Cerebral amyloid angiopathy-related inflammation (CAA-ri) is an uncommon but treatable cause of rapidly progressive dementia (RPD). Unlike prion or degenerative dementias, it can show partial reversibility with immunotherapy.</p> Case presentation <p>A 68-year-old man developed 3 months of fluctuating cognitive decline with disorientation, behavioural disturbances and memory disturbances. MRI revealed periventricular white matter hyperintensities and multiple cortical microbleeds, consistent with probable CAA-ri. He improved after intravenous methyl-prednisolone, with improvement in memory and behavioural domains, though minor memory deficits persisted.</p> Conclusion <p>CAA-ri should be considered in elderly patients with relapsing RPD. Recognition of its characteristic MRI features enables timely immunotherapy, which may stabilize or partially reverse cognitive decline.</p>

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Cerebral amyloid angiopathy-related inflammation presenting with relapsing rapidly progressive dementia

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  • 发表日期：

    2026年4月20日

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  • Vikas Lakhanpal,
  • Harkaran Singh Sahni,
  • Sarvpreet Singh Grewal

摘要

Background

Cerebral amyloid angiopathy-related inflammation (CAA-ri) is an uncommon but treatable cause of rapidly progressive dementia (RPD). Unlike prion or degenerative dementias, it can show partial reversibility with immunotherapy.

Case presentation

A 68-year-old man developed 3 months of fluctuating cognitive decline with disorientation, behavioural disturbances and memory disturbances. MRI revealed periventricular white matter hyperintensities and multiple cortical microbleeds, consistent with probable CAA-ri. He improved after intravenous methyl-prednisolone, with improvement in memory and behavioural domains, though minor memory deficits persisted.

Conclusion

CAA-ri should be considered in elderly patients with relapsing RPD. Recognition of its characteristic MRI features enables timely immunotherapy, which may stabilize or partially reverse cognitive decline.