Cerebral Amyloid Angiopathy-Related Inflammation: Report of a Case with Very Difficult Therapeutic Management.
Background. Cerebral amyloid angiopathy-related inflammation (CAA-ri) results from autoimmune response to beta-amyloid deposits in cerebral vessels. Its clinical course and complications have seldom been described in literature. Case Report. In a patient presenting with delirium and left hemiparesis the diagnosis of CAA-ri was supported by the finding of elevated anti-amyloid autoantibodies in the cerebrospinal fluid (CSF). Steroid therapy produced significant improvements in clinical and investigational assessments, but after two months, it caused Acute Respiratory Distress Syndrome. After steroid therapy discontinuation the patient presented a rapidly progressive dementia, Guillain-Barré syndrome, new cerebral ischemic lesions, and thrombosis of the right cephalic and subclavian veins that were treated with subcutaneous heparin. After a week the patient died because of brain hemorrhage. Conclusion. This case suggests caution in steroid therapy discontinuation and antithrombotic therapy administration in patients with CAA-ri. The CSF search of anti-amyloid autoantibodies could be helpful to support the diagnosis.
Geriatric Unit, Department of Life, Health & Environmental Sciences, University of L'Aquila, 67100 L'Aquila, Italy.
Francesca
F
Crosta
Neurology Unit, Avezzano's Hospital, 67051 L'Aquila, Italy.
Berardino
B
Orlandi
Neurology Unit, Avezzano's Hospital, 67051 L'Aquila, Italy.
Federica
F
De Santis
Radiology Unit, Avezzano's Hospital, 67051 L'Aquila, Italy.
Gianni
G
Passalacqua
Department of Neurology, San Gerardo Hospital, 20900 Monza, Italy ; The Inflammatory Cerebral Amyloid Angiopathy and Alzheimer's Disease Biomarkers (iCA β ) International Network, Department of Surgery and Translational Medicine, Milan Center for Neuroscience (NeuroMi), University of Milano-Bicocca, 20900 Monza, Italy.
Jacopo C
JC
DiFrancesco
The Inflammatory Cerebral Amyloid Angiopathy and Alzheimer's Disease Biomarkers (iCA β ) International Network, Department of Surgery and Translational Medicine, Milan Center for Neuroscience (NeuroMi), University of Milano-Bicocca, 20900 Monza, Italy.
Fabrizio
F
Piazza
Radiology Unit, L'Aquila's Hospital, 67100 L'Aquila, Italy.
Alessia
A
Catalucci
Geriatric Unit, Department of Life, Health & Environmental Sciences, University of L'Aquila, 67100 L'Aquila, Italy.
Giovambattista
G
Desideri
Neurology Unit, Department of Life, Health & Environmental Sciences, University of L'Aquila, 67100 L'Aquila, Italy.
Carmine
C
Marini