Dimitri Renard1, Lavinia Tatu1, Laurent Collombier2, Anne Wacongne1, Xavier Ayrignac3, Mahmoud Charif3, Yassine Boukriche4, Laura Chiper4, Genevieve Fourcade5, Souhayla Azakri3, Nicolas Gaillard6, Erick Mercier7, Sylvain Lehmann8, Eric Thouvenot1,9. 1. Department of Neurology, Nîmes University Hospital, Nîmes, France. 2. Department of Nuclear Medicine, Nîmes University Hospital, Nîmes, France. 3. Department of Neurology, Montpellier University Hospital, Montpellier, France. 4. Department of Neurology, CH Beziers, France. 5. Department of Neurology, CH Narbonne, France. 6. Department of Neurology, CH Perpignan, France. 7. Department of Hematology, Nîmes University Hospital, Nîmes, France. 8. Laboratoire de Biochimie-Protéomique Clinique - IRMB - CRB - Inserm U11183, CHU Montpellier, Hôpital St-Eloi - Université Montpellier, France. 9. Institut de Génomique Fonctionnelle, UMR5203, Université Montpellier, Montpellier, France.
Abstract
BACKGROUND: Cerebral amyloid angiopathy (CAA) can be associated with primary vasculitis of small/medium-sized leptomeningeal and cortical arteries, called CAA-related inflammation (CAA-ri). OBJECTIVE: To compare hemorrhagic and diffusion-weighted imaging (DWI) MRI features in CAA and CAA-ri. METHODS: We prospectively scored in a consecutive CAA and CAA-ri cohort: presence/number of chronic intracerebral hemorrhage (ICH), cerebral microbleeds (CMB), and cortical superficial siderosis (CSS) on initial T2*-weighted imaging, and DWI lesions on both initial and follow-up imaging. In a subgroup, ApoE, CSF, and 18F-florbetaben-positron emission tomography (FBB-PET) were also analyzed. RESULTS: In CAA-ri, CMB presence was more frequent (100% versus 40%, p < 0.001) and CMB numbers higher (mean 137 versus 8, p < 0.001). No difference was observed for chronic ICH or CSS. DWI lesions were more frequent in acute compared to chronic CAA-ri (p = 0.025), whereas no such difference was observed between acute and chronic CAA (p = 0.18). Both ApoE4 (genotyping available in 22 CAA-ri and 48 CAA patients) carriers and homozygosity were more frequent in CAA-ri (48% versus 19% [p = 0.014] and 32% versus 2% [p < 0.001] respectively). CSF biomarker analyses (performed in 20 CAA-ri and 45 CAA patients) showed lower Aβ42 levels in CAA-ri compared to CAA (median 312 versus 422 pg/mL, p = 0.0032). FBB-PET (performed in 11 CAA-ri and 20 CAA patients) showed higher standardized uptake value ratios in CAA-ri compared with CAA, only significant when the pons was used as reference (p = 0.037). CONCLUSION: Compared to CAA, CAA-ri was associated with higher CMB numbers, more frequent ApoE4 carriers and homozygotes, lower CSF Aβ42 levels, and more severe amyloid load on FBB-PET.
BACKGROUND:Cerebral amyloid angiopathy (CAA) can be associated with primary vasculitis of small/medium-sized leptomeningeal and cortical arteries, called CAA-related inflammation (CAA-ri). OBJECTIVE: To compare hemorrhagic and diffusion-weighted imaging (DWI) MRI features in CAA and CAA-ri. METHODS: We prospectively scored in a consecutive CAA and CAA-ri cohort: presence/number of chronic intracerebral hemorrhage (ICH), cerebral microbleeds (CMB), and cortical superficial siderosis (CSS) on initial T2*-weighted imaging, and DWI lesions on both initial and follow-up imaging. In a subgroup, ApoE, CSF, and 18F-florbetaben-positron emission tomography (FBB-PET) were also analyzed. RESULTS: In CAA-ri, CMB presence was more frequent (100% versus 40%, p < 0.001) and CMB numbers higher (mean 137 versus 8, p < 0.001). No difference was observed for chronic ICH or CSS. DWI lesions were more frequent in acute compared to chronic CAA-ri (p = 0.025), whereas no such difference was observed between acute and chronic CAA (p = 0.18). Both ApoE4 (genotyping available in 22 CAA-ri and 48 CAA patients) carriers and homozygosity were more frequent in CAA-ri (48% versus 19% [p = 0.014] and 32% versus 2% [p < 0.001] respectively). CSF biomarker analyses (performed in 20 CAA-ri and 45 CAA patients) showed lower Aβ42 levels in CAA-ri compared to CAA (median 312 versus 422 pg/mL, p = 0.0032). FBB-PET (performed in 11 CAA-ri and 20 CAA patients) showed higher standardized uptake value ratios in CAA-ri compared with CAA, only significant when the pons was used as reference (p = 0.037). CONCLUSION: Compared to CAA, CAA-ri was associated with higher CMB numbers, more frequent ApoE4 carriers and homozygotes, lower CSF Aβ42 levels, and more severe amyloid load on FBB-PET.
Authors: L Grangeon; G Quesney; X Ayrignac; D Wallon; M Verdalle-Cazes; S Coulette; D Renard; A Wacongne; T Allou; N Olivier; Y Boukriche; G Blanchet-Fourcade; P Labauge; C Arquizan; S Canaple; O Godefroy; O Martinaud; P Verdure; M Quillard-Muraine; J Pariente; E Magnin; G Nicolas; C Charbonnier; D Maltête; M Formaglio; N Raposo Journal: J Neurol Date: 2022-06-26 Impact factor: 6.682