Probable Cerebral Amyloid Angiopathy-Related Inflammation: A Case Report
Tianheng Zheng, Kangqing Zhang, Junjie Hao, Yibin Fang, Huazheng LiangBackground
Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare, treatable inflammatory variant of cerebral amyloid angiopathy characterized by heterogeneous clinical manifestations and ambiguous imaging features, frequently leading to delayed diagnosis.
Case Description
This report describes a 70-year-old hypertensive male who presented with subacute progressive cognitive decline, severe headache, vomiting, and blurred vision. Neurological examination revealed left homonymous hemianopia and mild cognitive impairment (MMSE 21/30). Brain non-contrast MRI demonstrated extensive asymmetric T2/FLAIR white matter hyperintensities with mild mass effect, multiple strictly lobar microbleeds on susceptibility-weighted imaging (SWI), and patchy subcortical DWI-positive ischemic lesions with corresponding ADC changes without appreciable parenchymal or meningeal enhancement. Lumbar puncture revealed isolated mild elevation of cerebrospinal fluid (CSF) protein (0.8 g/L) without pleocytosis. ApoE genotyping confirmed an ε4/ε4 homozygous genotype. The patient was recommended corticosteroid therapy but declined and transferred to another institution, where he received methylprednisolone . His symptoms resolved dramatically , and the two-year follow-up MRI showed significant resolution of the previously extensive white matter lesions , with only mild residual Fazekas grade 2 leukoaraiosis.
Conclusion
This case fulfills the validated Auriel clinicoradiological criteria for probable CAA-RI without pathological confirmation. ApoE ε4/ε4 genotyping may provide supportive auxiliary evidence rather than a definitive diagnostic index. Combined interpretation of clinical symptoms, characteristic multimodal MRI findings, and genetic susceptibility evidence can substantially improve the diagnostic accuracy of probable CAA-RI in clinical practice.