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A Clinico-Radiological Study of Cerebral Amyloid Angiopathy-Related Inflammation

Abstract : OBJECTIVE: To describe the clinico-radiological features and long-term prognosis in patients with cerebral amyloid angiopathy-related inflammation (CAA-ri). METHODS: Twenty-eight CAA-ri patients were recruited retrospectively from 6 neurological centers. We recorded the clinico-radiological and biological data, at baseline and during follow-up. Baseline characteristics associated with relapse risk and prognosis were assessed. RESULTS: Five patients had pathologically confirmed CAA-ri whereas 23 had probable (n = 21) or possible (n = 2) CAA-ri. The mean age was 72 years; main clinical symptoms included confusion (54%), hemiparesis (36%), and aphasia (29%). Cerebral MRI disclosed a brain parenchymal lesion (89%), which was usually multifocal (82%) and bilateral (89%). It was associated with gadolinium enhancement (84%), small ischemic lesions (39%), cortical superficial siderosis (CSS; 50%), and a high number of microbleeds (mean 240 ± 277). An isolated leptomeningeal involvement was observed in 3 patients with pathological confirmation. Despite a favorable initial evolution after treatment, we observed a 42% risk of relapse, mostly within the first year (83%). After a mean follow-up of 2 years, 29% died and 25% had a marked disability. Disseminated CSS was associated with death. CONCLUSION: Despite an apparently favorable initial evolution, CAA-ri is characterized by a poor prognosis. Diagnostic criteria should consider patients with isolated leptomeningeal involvement.
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https://hal.umontpellier.fr/hal-02558996
Contributeur : Cécile Nowak <>
Soumis le : jeudi 30 avril 2020 - 09:18:32
Dernière modification le : vendredi 15 mai 2020 - 12:22:09

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Sarah Coulette, Dimitri Renard, Sylvain Lehmann, Nicolas Raposo, Caroline Arquizan, et al.. A Clinico-Radiological Study of Cerebral Amyloid Angiopathy-Related Inflammation. Cerebrovascular Diseases, Karger, 2019, 48 (1-2), pp.38-44. ⟨10.1159/000502832⟩. ⟨hal-02558996⟩

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