Probable Cerebral Amyloid Angiopathy-Related Inflammation: A Case Report
Abstract
Background 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.
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BibTeX
@article{zheng2026probable,
title = {{Probable Cerebral Amyloid Angiopathy-Related Inflammation: A Case Report}},
author = {Tianheng Zheng and Kangqing Zhang and Junjie Hao and Yibin Fang and Huazheng Liang},
journal = {Journal of Central Nervous System Disease},
year = {2026},
month = oct,
doi = {10.1177/11795735261502255},
url = {https://doi.org/10.1177/11795735261502255},
note = {CC BY},
}
RIS
TY - JOUR
TI - Probable Cerebral Amyloid Angiopathy-Related Inflammation: A Case Report
AU - Tianheng Zheng
AU - Kangqing Zhang
AU - Junjie Hao
AU - Yibin Fang
AU - Huazheng Liang
PY - 2026
DA - 2026/10/29
JO - Journal of Central Nervous System Disease
DO - 10.1177/11795735261502255
UR - https://doi.org/10.1177/11795735261502255
AB - Background 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.
SN - 1179-5735
ER -