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Comparative Study
. 2024 Jul 23;103(2):e209548.
doi: 10.1212/WNL.0000000000209548. Epub 2024 Jun 20.

Cerebral Amyloid Angiopathy-Related Inflammation and Biopsy-Positive Primary Angiitis of the CNS: A Comparative Study

Lou Grangeon  1 Grégoire Boulouis  1 Jean Capron  1 Fouzi Bala  1 Dimitri Renard  1 Nicolas Raposo  1 Ozlem Ozkul-Wermester  1 Aude Triquenot-Bagan  1 Xavier Ayrignac  1 David Wallon  1 Emmanuel Gerardin  1 Philippe Kerschen  1 Denis Sablot  1 Maïté Formaglio  1 Fernando Pico  1 Guillaume Turc  1 Marc Verny  1 Lisa Humbertjean  1 Marie Gaudron  1 Stéphane Vannier  1 Nelly Dequatre  1 Benoit Guillon  1 Clothilde Isabel  1 Caroline Arquizan  1 Olivier Detante  1 Sophie Godard  1 Barbara Casolla  1 Michael Levraut  1 Cédric Gollion  1 Mathieu Gerfaud-Valentin  1 Laurent Kremer  1 Laure Daelman  1 Nicolas Lambert  1 Sylvain Lanthier  1 Alexandre Poppe  1 Alexis Régent  1 David Weisenburger-Lile  1 Pierre Verdure  1 Gérald Quesney  1 Mathieu Vautier  1 Anne Wacongne  1 Eric Thouvenot  1 Jérémie Pariente  1 Sarah Coulette  1 Pierre M Labauge  1 Nadège Olivier  1 Thibaut Allou  1 Hélène Zephir  1 Antoine Néel  1 Saskia Bresch  1 Benjamin Terrier  1 Olivier Martinaud  1 Romain Schneckenburger  1 Thomas Papo  1 Chloé Comarmond-Ortoli  1 Eric Jouvent  1 Marie Subréville  1 Louis Poncet-Megemont  1 Muhammad Amer Khatib  1 François Lun  1 Carole Henry  1 Eloi Magnin  1 Quentin Thomas  1 Mathilde Graber  1 Yassine Boukriche  1 Geneviève Blanchet-Fourcade  1 Diana Ratiu  1 Christian Pagnoux  1 Emmanuel Touzé  1 Hubert de Boysson  1 Sonia Alamowitch  1 Ahmad Nehme  1
Affiliations
Comparative Study

Cerebral Amyloid Angiopathy-Related Inflammation and Biopsy-Positive Primary Angiitis of the CNS: A Comparative Study

Lou Grangeon et al. Neurology. .

Abstract

Background and objectives: Cerebral amyloid angiopathy-related inflammation (CAA-RI) and biopsy-positive primary angiitis of the CNS (BP-PACNS) have overlapping clinicoradiologic presentations. It is unknown whether clinical and radiologic features can differentiate CAA-RI from BP-PACNS and whether both diseases have different relapse rates. The objectives of this study were to compare clinicoradiologic presentations and relapse rates in patients with CAA-RI vs BP-PACNS.

Methods: Patients with CAA-RI and BP-PACNS were enrolled from 2 retrospective multicenter cohorts. Patients with CAA-RI were biopsy-positive or met probable clinicoradiologic criteria. Patients with BP-PACNS had histopathologic confirmation of CNS angiitis, with no secondary etiology. A neuroradiologist read brain MRIs, blinded to the diagnosis of CAA-RI or BP-PACNS. Clinicoradiologic features were compared using univariable logistic regression models. Relapse rates were compared using a univariable Fine-Gray subdistribution hazard model, with death as a competing risk.

Results: This study enrolled 104 patients with CAA-RI (mean age 73 years, 48% female sex) and 52 patients with BP-PACNS (mean age 45 years, 48% female sex). Patients with CAA-RI more often had white matter hyperintense lesions meeting the probable CAA-RI criteria (93% vs 51%, p < 0.001), acute subarachnoid hemorrhage (15% vs 2%, p = 0.02), cortical superficial siderosis (27% vs 4%, p < 0.001), ≥1 lobar microbleed (94% vs 26%, p < 0.001), past intracerebral hemorrhage (17% vs 4%, p = 0.04), ≥21 visible centrum semiovale perivascular spaces (34% vs 4%, p < 0.01), and leptomeningeal enhancement (70% vs 27%, p < 0.001). Patients with BP-PACNS more often had headaches (56% vs 31%, p < 0.01), motor deficits (56% vs 36%, p = 0.02), and nonischemic parenchymal gadolinium enhancement (82% vs 16%, p < 0.001). The prevalence of acute ischemic lesions was 18% in CAA-RI and 22% in BP-PACNS (p = 0.57). The features with the highest specificity for CAA-RI were acute subarachnoid hemorrhage (98%), cortical superficial siderosis (96%), past intracerebral hemorrhage (96%), and ≥21 visible centrum semiovale perivascular spaces (96%). The probable CAA-RI criteria had a 71% sensitivity (95% CI 44%-90%) and 91% specificity (95% CI 79%-98%) in differentiating biopsy-positive CAA-RI from BP-PACNS. The rate of relapse in the first 2 years after remission was lower in CAA-RI than in BP-PACNS (hazard ratio 0.46, 95% CI 0.22-0.96, p = 0.04).

Conclusion: Clinicoradiologic features differed between patients with CAA-RI and those with BP-PACNS. Specific markers for CAA-RI were hemorrhagic signs of subarachnoid involvement, past intracerebral hemorrhage, ≥21 visible centrum semiovale perivascular spaces, and the probable CAA-RI criteria. A biopsy remains necessary for diagnosis in some cases of CAA-RI. The rate of relapse in the first 2 years after disease remission was lower in CAA-RI than in BP-PACNS.

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