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Cerebral Amyloid Angiopathy-Related Inflammation and Biopsy-Positive Primary Angiitis of the CNS: A Comparative Study.
Grangeon, Lou; Boulouis, Grégoire; Capron, Jean; Bala, Fouzi; Renard, Dimitri; Raposo, Nicolas; Ozkul-Wermester, Ozlem; Triquenot-Bagan, Aude; Ayrignac, Xavier; Wallon, David; Gerardin, Emmanuel; Kerschen, Philippe; Sablot, Denis; Formaglio, Maïté; Pico, Fernando; Turc, Guillaume; Verny, Marc; Humbertjean, Lisa; Gaudron, Marie; Vannier, Stéphane; Dequatre, Nelly; Guillon, Benoit; Isabel, Clothilde; Arquizan, Caroline; Detante, Olivier; Godard, Sophie; Casolla, Barbara; Levraut, Michael; Gollion, Cédric; Gerfaud-Valentin, Mathieu; Kremer, Laurent; Daelman, Laure; Lambert, Nicolas; Lanthier, Sylvain; Poppe, Alexandre; Régent, Alexis; Weisenburger-Lile, David; Verdure, Pierre; Quesney, Gérald; Vautier, Mathieu; Wacongne, Anne; Thouvenot, Eric; Pariente, Jérémie; Coulette, Sarah; Labauge, Pierre M; Olivier, Nadège; Allou, Thibaut; Zephir, Hélène; Néel, Antoine; Bresch, Saskia.
Affiliation
  • Grangeon L; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Boulouis G; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Capron J; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Bala F; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Renard D; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Raposo N; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Ozkul-Wermester O; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Triquenot-Bagan A; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Ayrignac X; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Wallon D; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Gerardin E; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Kerschen P; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Sablot D; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Formaglio M; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Pico F; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Turc G; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Verny M; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Humbertjean L; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Gaudron M; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Vannier S; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Dequatre N; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Guillon B; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Isabel C; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Arquizan C; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Detante O; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Godard S; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Casolla B; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Levraut M; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Gollion C; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Gerfaud-Valentin M; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Kremer L; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Daelman L; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Lambert N; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Lanthier S; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Poppe A; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Régent A; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Weisenburger-Lile D; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Verdure P; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Quesney G; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Vautier M; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Wacongne A; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Thouvenot E; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Pariente J; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Coulette S; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Labauge PM; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Olivier N; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Allou T; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Zephir H; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Néel A; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
  • Bresch S; From Neurology (L.G., O.O.-W., A.T.-B., D.W., P.V., G.Q.), CHU de Rouen; Radiology (G.B., F.B.), CHRU de Tours; Service des Urgences Neurovasculaires (J.C., S.A.), Hôpital Pitié-Salpêtrière, Sorbonne Université, AP-HP, Stare Team, iCRIN, Institut du Cerveau, Paris; Neurology (D. Renard, A.W., E. Tho
Neurology ; 103(2): e209548, 2024 Jul 23.
Article in En | MEDLINE | ID: mdl-38900992
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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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Cerebral Amyloid Angiopathy / Vasculitis, Central Nervous System Limits: Adult / Aged / Aged80 / Female / Humans / Male / Middle aged Language: En Journal: Neurology Year: 2024 Document type: Article Country of publication: United States
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Cerebral Amyloid Angiopathy / Vasculitis, Central Nervous System Limits: Adult / Aged / Aged80 / Female / Humans / Male / Middle aged Language: En Journal: Neurology Year: 2024 Document type: Article Country of publication: United States