title: Cerebral amyloid angiopathy-related inflammation: A potentially reversible cause of dementia with characteristic imaging findings.
abstract: OBJECTIVE
  Cerebral amyloid angiopathy with inflammation (CAA-I) is a less well-recognized clinically and radiologically distinct subtype of CAA. We aim to describe the imaging manifestations of this uncommon entity.
  
  
  METHODS
  A retrospective review of the medical records and imaging database yielded 9 patients with clinical and radiological findings compatible with CAA-I. The neurological findings at presentation, MRI findings including the presence of white matter involvement, mass effect, microhemorrhages and contrast enhancement, treatment provided and outcome were evaluated. Brain biopsy specimens, when available were also reviewed.
  
  
  RESULTS
  All patients presented with subacute cognitive decline. In all 9 patients, confluent white matter lesions with mass effect were observed. Eight out of 9 patients demonstrated foci of microhemorrhage, while in 1, the microhemorrhages appeared 12 weeks after the initial examination. No significant parenchymal or meningeal enhancement was present in any patient. In 4 patients, brain biopsy was consistent with CAA-I. Immunosuppressive therapy was initiated in all patients, leading to full recovery in 5.
  
  
  CONCLUSIONS
  CAA-I is characterized by the subacute onset of dementia, a distinct pattern of confluent white matter signal abnormality with mass effect and response to immunosuppressive therapy. Prompt recognition may help obviate brain biopsy and initiation of treatment.
authors.affiliation: Division of Neuroradiology, Department of Diagnostic Radiology and Nuclear Medicine, University of Maryland School of Medicine, 22 S Greene Street, Baltimore, MD 21201, United States. Electronic address: praghavan@umm.edu.
authors.forename: Prashant
authors.initials: P
authors.lastname: Raghavan
authors.affiliation: Department of Neuroradiology, Beaumont Hospital, Beaumont Road, Beaumont, Dublin 9, Ireland. Electronic address: seamuslooby@beaumont.ie.
authors.forename: Seamus
authors.initials: S
authors.lastname: Looby
authors.affiliation: Norton Hospital, 200 East Chestnut Street, Louisville, KY 40202, United States. Electronic address: david.bourne@nortonhealthcare.org.
authors.forename: T David
authors.initials: TD
authors.lastname: Bourne
authors.affiliation: Department of Radiology, Neuroradiology Division, Stanford University, 300 Pasteur Dr, Room S047, Stanford, CA 94305-5105, United States. Electronic address: Max.Wintermark@gmail.com.
authors.forename: Max
authors.initials: M
authors.lastname: Wintermark
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001921:Brain / Q000000981:diagnostic imaging* / Q000473:pathology*; D016657:Cerebral Amyloid Angiopathy / Q000000981:diagnostic imaging* / Q000473:pathology*; D003704:Dementia / Q000000981:diagnostic imaging / Q000473:pathology; D004660:Encephalitis / Q000000981:diagnostic imaging* / Q000473:pathology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies; D066127:White Matter / Q000000981:diagnostic imaging / Q000473:pathology
keywords: Amyloid; Biopsy; Cerebral; Immunosuppression; Inflammation