Steroid responsive encephalopathy in cerebral amyloid angiopathy: a case report and review of evidence for immunosuppressive treatment.
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.
Kloppenborg
Raoul P
RP
Department of Neurology, Academic Medical Center, Amsterdam, The Netherlands.
Richard
Edo
E
Sprengers
Marieke E S
ME
Troost
Dirk
D
Eikelenboom
Piet
P
Nederkoorn
Paul J
PJ
D000368:Aged; D016657:Cerebral Amyloid Angiopathy / Q000150:complications* / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D013256:Steroids / Q000627:therapeutic use*
D013256:Steroids