October 29, 2015: Cerebral amyloid angiopathy-related inflammation

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October 29, 2015

Cerebral amyloid angiopathy-related inflammation

Cerebral amyloid angiopathy (CAA) is a disorder of amyloid deposition in the cerebral vasculature most often presenting in the elderly as an accumulation of hemorrhagic infarctions that eventually leads to dementia. Hemorrhages are visible on susceptibility-weighted MRI as macrohemorrhages (>5mm) and punctuate “microhemorrhages” (≤5mm). CAA-related inflammation (CAARI) is a subset of CAA that presents with reversible acute/subacute encephalopathy, headache, seizures, or stroke-like signs (e.g. focal neurologic deficits) 1. In addition to the MRI findings typical of CAA, those characteristic of CAARI include symmetric or asymmetric, patchy or confluent T2 hyperintense lesions in the cortical and subcortical white matter. Recognition of this syndrome is important as treatment with corticosteroids or alternative immunosuppression is the current standard and is associated with a good prognosis.

  1. Kirshner HS, Bradshaw M. The Inflammatory Form of Cerebral Amyloid Angiopathy or "Cerebral Amyloid Angiopathy-Related Inflammation" (CAARI). Current neurology and neuroscience reports 2015; 15: 54.

Submitted by Michael J. Bradshaw, MD, and Howard S. Kirshner, MD, Department of Neurology, Vanderbilt University Medical Center.

Disclosures: Drs. Bradshaw and Kirshner report no disclosures.

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