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Cerebral amyloid angiopathy-related inflammation (CAA-ri) is an unusual cause of encephalopathy, seizures and focal neurological deficits.1 2 We report three cases of CAA-ri with minimal symptoms but striking and dynamically evolving brain MRI findings.
A 62-year-old man presented with a moderately severe non-radiating frontal headache. Brain MRI 9 months later showed multiple discrete regions of abnormal signal and mild swelling involving white matter and overlying cortex. Susceptibility-weighted imaging (SWI) demonstrated numerous cortical lobar microbleeds throughout both cerebral hemispheres. Repeat MRI another 9 months later showed resolution of many of the parenchymal abnormalities, but with several new regions containing more peripheral microbleeds. Amyloid-PET (using 18F-florbetapir) demonstrated moderate widespread amyloid deposition; CSF analysis showed reduced amyloid-beta 1–42 and high-normal total tau. Formal neuropsychological testing suggested mild compromise in frontal functioning only. The patient was treated with 5 days of intravenous methylprednisolone (1 g daily), followed by an oral taper from prednisolone 60 mg over 8 weeks. Follow-up MRI after 8 months showed almost complete resolution of the parenchymal abnormalities, but with persisting lobar microbleeds. At 24 months following symptom onset, he remains asymptomatic, with stable brain imaging.
A 74-year-old man presented with mild subjective memory difficulties only, with no objective neuropsychological deficits. MRI demonstrated a substantial region of abnormal signal in the right temporal and occipital white matter, with no enhancement. Repeat imaging after a few weeks showed partial regression. Over the following 4 years, three further MRIs showed multiple areas of abnormal white matter (sometimes involving cortex as well) within the temporal, parietal and occipital lobes, which …
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