Prodromal Alzheimer's Disease Presenting as Cerebral Amyloid Angiopathy-Related Inflammation with Spontaneous Amyloid-Related Imaging Abnormalities and High Cerebrospinal Fluid Anti-Aβ Autoantibodies

Article type: Short Communication

Authors: Boncoraglio, Giorgio B.^{a; 1; *} | Piazza, Fabrizio^{b; c; 1} | Savoiardo, Mario^d | Farina, Laura^d | DiFrancesco, Jacopo C.^{b; e} | Prioni, Sara^f | Tagliavini, Fabrizio^f | Parati, Eugenio A.^a | Giaccone, Giorgio^f

Affiliations: [a] Department of Cerebrovascular Diseases, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milano, Italy | [b] Department of Surgery and Translational Medicine, University of Milano-Bicocca, Monza, Italy | [c] The inflammatory Cerebral Amyloid Angiopathy and Alzheimer's disease Biomarkers International Network (iCAB) Coordinator | [d] Department of Neuroradiology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milano, Italy | [e] Department of Neurology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milano, Italy | [f] Department of Neurodegenerative Diseases, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milano, Italy

Correspondence: [*] Correspondence to: Dr. Giorgio Boncoraglio, Department of Cerebrovascular Diseases, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milano, via Celoria 11 – 20133 Milano, Italy. Tel.: +39 0223941; Fax: +39 0270638217; E-mail: [email protected].

Note: [1] These authors contributed equally to this work.

Abstract: Cerebral amyloid angiopathy-related inflammation (CAA-ri), a rare form of vasculitis associated with amyloid-β (Aβ) deposition in vessel walls, has been proposed as a spontaneous human model of the amyloid-related imaging abnormalities (ARIA) occurring after anti-Aβ immunotherapy for the treatment of Alzheimer's disease (AD). We describe a case of a patient with biopsy-proven CAA-ri and prodromal AD, confirmed by means of neuropsychological examination after 20 months follow-up, presenting with ARIA and high levels of cerebrospinal fluid anti-Aβ autoantibodies. This case further supports the analogies between the inflammatory response driven by anti-Aβ immunotherapy and that spontaneously occurring in CAA-ri.

Keywords: Alzheimer's disease, amyloid-related imaging abnormalities, anti-Aβ autoantibodies, cerebral amyloid angiopathy, cerebral amyloid angiopathy-related inflammation, subarachnoid hemorrhage

DOI: 10.3233/JAD-142376

Journal: Journal of Alzheimer's Disease, vol. 45, no. 2, pp. 363-367, 2015