Cerebral Amyloid Angiopathy-Related Inflammation: Report of a Case with Very Difficult Therapeutic Management

Francesca Crosta,Federica De Santis,Carmine Marini,Fabrizio Piazza,Gianni Passalacqua,Jacopo C Difrancesco,Alessia Catalucci,Berardino Orlandi,Giovambattista Desideri

doi:10.1155/2015/483020

Abstract

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.

Highlights

Cerebral amyloid angiopathy-related inflammation (CAA-ri) represents the coexistence of cerebral amyloid angiopathy (CAA) and inflammation and is thought to be due to an autoimmune response to beta-amyloid deposits [1,2,3,4]
Cerebral spinal fluid (CSF) anti-amyloid-β autoantibodies have recently been demonstrated to be elevated during the acute phase of CAA-ri, returning to control level following clinical and radiological remission [4, 5]
CAAri is a reversible cause of progressive dementia but the pattern of cortical and subcortical symptoms, severity, and progression has not been extensively assessed in the available reports

Summary

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. 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-Barresyndrome, new cerebral ischemic lesions, and thrombosis of the right cephalic and subclavian veins that were treated with subcutaneous heparin. 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

Introduction

Case Report

Discussion

Conclusion