Steroid responsive encephalopathy in cerebral amyloid angiopathy: a case report and review of evidence for immunosuppressive treatment |
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Authors: | Raoul P Kloppenborg Edo Richard Marieke ES Sprengers Dirk Troost Piet Eikelenboom and Paul J Nederkoorn |
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Institution: | (1) Department of Neurology, Academic Medical Center, Amsterdam, The Netherlands;(2) Department of Radiology, Academic Medical Center, Amsterdam, The Netherlands;(3) Department of Neuropathology, Academic Medical Center, Amsterdam, The Netherlands |
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Abstract: | Cerebral amyloid angiopathy (CAA) is a common but often asymptomatic disease, characterized by deposition of amyloid in cerebral
blood vessels. We describe the successful treatment of CAA encephalopathy with dexamethasone in a patient with CAA-related
inflammation causing subacute progressive encephalopathy and seizures, which is an increasingly recognized subtype of CAA.
The two pathological subtypes of CAA-related inflammation are described and a review of the literature is performed concerning
immunosuppressive treatment of CAA-related inflammation with special attention to its pathological subtypes. Immunosuppressive
therapy appears to be an appropriate treatment for CAA encephalopathy. |
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