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Case No. 752
2026-04-01~2026-04-30
출제자 : 임영희
Hit : 859
| Period | 2026-04-01~2026-04-30 |
|---|---|
| Diagnosis | CAA-related inflammation |
| Clinical information | 77/F C.C> Dizziness, headache, decrease in consciousness What is the diagnosis? |
| Discussion | <그림 설명> Microbleeds, superficial siderosis, with perilesional T2/FLAIR hyperintensity and leptomeningeal enhancement, involving the right parieto-temporo-occipital lobes <해설> Inflammatory cerebral amyloid angiopathy (iCAA), also termed CAA-related inflammation (CAA-ri) or amyloid-β–related angiitis (ABRA), is a treatable inflammatory subtype of cerebral amyloid angiopathy characterized by immune response to vascular amyloid deposition. 1. White matter changes: Patchy or confluent T2/FLAIR hyperintensities Typically asymmetric Predominantly subcortical ± cortical extension Represent vasogenic edema 2. Hemorrhage (CAA): Multiple strictly lobar cerebral microbleeds Cortical superficial siderosis ± Lobar macrohemorrhage 3. Leptomeningeal / Parenchymal enhancement: Supports inflammatory component and helps distinguish from non-inflammatory CAA 4. Distribution Pattern: Cortico-subcortical (often posterior predominance) Lesions correspond to areas of prior microhemorrhages Diagnostic category: Probable / possible / definite Probable CCA-ri 1. Age ≥40 years 2. ≥1 clinical features; headache, decreased consciousness, behavioral change, or focal neurological signs and seizure, not directly attributable to acute ICH 3. MRI ; white matter hyperintensities, asymmetric, extend to subcortical white matter 4. Cerebral microbleed, cortical superficial siderosis 5. Absence of neoplastic, infectious, or other caus Possible CAA-ri: Less specific imaging Definite CAA-ri: Brain biopsy (rarely required) Reference Wu JJ, et al. Cerebral amyloid angiopathy-related inflammation. Front Neurol. 2021. de Souza A, et al. Inflammatory cerebral amyloid angiopathy: a broad clinical spectrum. J Clin Neurol. 2023. Grasso D, et al. Cerebral amyloid angiopathy-related inflammation imaging features. Radiol Case Rep. 2021. Salvarani C, et al. Imaging findings of CAA-related inflammation. Medicine (Baltimore). 2016. |
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