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. 2022 Sep;269(9):4972-4984.
doi: 10.1007/s00415-022-11145-4. Epub 2022 Jun 26.

Different clinical outcomes between cerebral amyloid angiopathy-related inflammation and non-inflammatory form

L Grangeon #  1 , G Quesney #  2 , M Verdalle-Cazes  3 , S Coulette  4 , D Renard  5 , A Wacongne  5 , T Allou  6 , N Olivier  6 , Y Boukriche  7 , G Blanchet-Fourcade  8 , P Labauge  4 , C Arquizan  4 , S Canaple  9 , O Godefroy  9 , O Martinaud  10   11 , P Verdure  12 , M Quillard-Muraine  13 , J Pariente  14 , E Magnin  15 , G Nicolas  16 , C Charbonnier  16 , D Maltête  2 , M Formaglio  17 , N Raposo  14 , X Ayrignac #  4 , D Wallon #  2   15
Affiliations

Different clinical outcomes between cerebral amyloid angiopathy-related inflammation and non-inflammatory form

L Grangeon et al. J Neurol. 2022 Sep.

Abstract

Objective: Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a rare manifestation related to CAA, thought to be more severe. We aimed to compare the clinical and radiological outcomes of CAA-ri and non-inflammatory CAA.

Materials and methods: We retrospectively included all patients with CAA-ri from 13 French centers. We constituted a sex- and age-matched control cohort with non-inflammatory CAA and similar disease duration. Survival, autonomy and cognitive evolution were compared after logistic regression. Cerebral microbleeds (CMB), intracerebral hemorrhage, cortical superficial siderosis and hippocampal atrophy were analyzed as well as CSF biomarker profile and APOE genotype when available. Outcomes were compared using Kaplan-Meier curves and log-rank tests.

Results: Data from 48 CAA-ri patients including 28 already reported and 20 new patients were analyzed. Over a mean of 3.1 years, 11 patients died (22.9%) and 18 (37.5%) relapsed. CAA-ri patients were more frequently institutionalized than non-inflammatory CAA patients (30% vs 8.3%, p < 0.001); mortality rates remained similar. MMSE and modified Rankin scale scores showed greater severity in CAA-ri at last follow-up. MRI showed a higher number of CMB at baseline and last follow-up in CAA-ri (p < 0.001 and p = 0.004, respectively). CSF showed lower baseline levels of Aß42 in CAA-ri than non-inflammatory CAA (373.3 pg/ml vs 490.8 pg/ml, p = 0.05). CAA-ri patients more likely carried at least one APOE ε4 allele (76% vs 37.5%, adjusted p = 0.05) particularly as homozygous status (56% vs 6.2%, p < 0.001).

Interpretation: CAA-ri appears to be more severe than non-inflammatory CAA with a significant loss of autonomy and global higher amyloid burden, shown by more CMB and a distinct CSF profile. This burden may be partially promoted by ε4 allele.

Keywords: APOE genotype; CSF biomarkers; Cerebral MRI; Cerebral amyloid angiopathy; Cerebral amyloid angiopathy-related inflammation; Clinical outcome.

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References

    1. Greenberg SM, Bacskai BJ, Hernandez-Guillamon M et al (2020) Cerebral amyloid angiopathy and Alzheimer disease—one peptide, two pathways. Nat Rev Neurol 16(1):30–42. https://doi.org/10.1038/s41582-019-0281-2 - DOI - PubMed
    1. Chung KK, Anderson NE, Hutchinson D et al (2011) Cerebral amyloid angiopathy related inflammation: three case reports and a review. J Neurol Neurosurg Psychiatry 82:20–26. https://doi.org/10.1136/jnnp.2009.204180 - DOI - PubMed
    1. Castro Caldas A, Silva C, Albuquerque L et al (2015) Cerebral amyloid angiopathy associated with inflammation: report of 3 cases and systematic review. J Stroke Cerebrovasc Dis 24:2039–2048. https://doi.org/10.1016/j.jstrokecerebrovasdis.2015.04.015 - DOI - PubMed
    1. Auriel E, Charidimou A, Gurol ME et al (2016) Validation of clinicoradiological criteria for the diagnosis of cerebral amyloid angiopathy-related inflammation. JAMA Neurol 73:197–202. https://doi.org/10.1001/jamaneurol.2015.4078 - DOI - PubMed
    1. Charidimou A, Imaizumi T, Moulin S et al (2017) Brain hemorrhage recurrence, small vessel disease type, and cerebral microbleeds: a meta-analysis. Neurology 89:820–829. https://doi.org/10.1212/WNL.0000000000004259 - DOI - PubMed - PMC

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