Sarah Coulette1, Dimitri Renard2, Sylvain Lehmann3, Nicolas Raposo4, Caroline Arquizan1, Mahmoud Charif1, Eric Thouvenot2, Anne Wacongne2, Alain Viguier4, Fabrice Bonneville5, Thibaut Allou6, Yassine Boukriche7, Laura Chiper7, Genevieve Blanchet Fourcade8, Audrey Gabelle1,9, Anne Ducros1, Claire Duflos10, Pierre Labauge1, Nicolas Menjot de Champfleur11, Xavier Ayrignac12. 1. Department of Neurology, Montpellier University Hospital, Montpellier, France. 2. Department of Neurology, Nimes University Hospital, Nimes, France. 3. IRMB, CRB, Inserm U11183, Montpellier University Hospital, Montpellier, France. 4. Department of Neurology, Toulouse University Hospital, Toulouse, France. 5. Department of Neuroradiology, Toulouse University Hospital, Toulouse, France. 6. Department of Neurology, Perpignan Hospital, Perpignan, France. 7. Department of Neurology, Beziers Hospital, Beziers, France. 8. Department of Neurology, Narbonne Hospital, Narbonne, France. 9. Memory Research and Resources Center, Montpellier University Hospital, Montpellier, France. 10. Economic Evaluation Unit, Montpellier University Hospital, Montpellier, France. 11. Neuroradiology, Montpellier University Hospital, Montpellier, France. 12. Department of Neurology, Montpellier University Hospital, Montpellier, France, xavier.ayrignac@yahoo.fr.
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.
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.
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
Authors: John M Ringman; Elizabeth Joe; Nasim Sheikh-Bahaei; Carol Miller; Harry V Vinters; Samuel Guzman; Helena C Chui Journal: Alzheimer Dis Assoc Disord Date: 2021 Jul-Sep 01 Impact factor: 2.703