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Front. Neurol. | doi: 10.3389/fneur.2019.00984

Cerebral amyloid angiopathy related inflammation with prominent meningeal involvement. A report of 2 cases

 Agnès Aghetti1, 2, Damien Sène1, 3, Marc Polivka1, Natalia Shor4, Sarah Lechtman1, 3,  Hugues Chabriat1, 2, 3,  Eric Jouvent1, 2, 3 and  Stéphanie Guey1, 2, 3*
  • 1Groupe Hospitalier Lariboisière Fernand Widal, France
  • 2INSERM U1141 Neuroprotection du cerveau en développement, France
  • 3Université Sorbonne Paris Cité, France
  • 4Hôpitaux Universitaires Pitié Salpêtrière, France

Cerebral amyloid angiopathy related inflammation (CAA-RI) is a rare form of CAA characterized by subacute encephalitic symptoms (cognitive decline, seizures, focal deficits) characterized on brain MRI by extensive and confluent white matter lesions colocalizing with lobar microbleeds. We report two cases of unusual CAA-RI mimicking meningoencephalitis without typical brain lesions on FLAIR and T2* sequences, which diagnoses were delayed. These 2 cases may extend the spectrum of CAA-RI by suggesting the possible occurrence of quite purely meningeal forms of CAA-RI.

Keywords: Cerebral Amyloid Angiopathy (CAA), Cerebral amyloid angiopathy-related inflammation, xanthochromia, Subarachanoid hemorrhage, meningeal inflammation

Received: 17 Apr 2019; Accepted: 28 Aug 2019.

Copyright: © 2019 Aghetti, Sène, Polivka, Shor, Lechtman, Chabriat, Jouvent and Guey. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Mx. Stéphanie Guey, Groupe Hospitalier Lariboisière Fernand Widal, Paris, France, stephanie.guey@aphp.fr