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Course of cerebral amyloid angiopathy–related inflammation

From the Hemorrhagic Stroke Research Program (C.K., L.W., E.E.S., J.R., S.M.G.), Department of Neurology, Department of Radiology (M.H.L.), and C.S. Kubik Laboratory of Neuropathology (M.P.F.), Massachusetts General Hospital, Boston.

Address correspondence and reprint requests to Dr Greenberg, MGH Stroke Research Center, 55 Fruit St., CPZ 175, Suite 300, Boston, MA 02114 sgreenberg{at}partners.org

Background: A subset of patients with cerebral amyloid angiopathy (CAA) present with cognitive symptoms, seizures, headaches, T2-hyperintense MRI lesions, and neuropathologic evidence of CAA-associated vascular inflammation.

Objective: To analyze the risk factors, diagnostic characteristics, and long-term course of this disorder.

Methods: We assessed 14 consecutive patients with pathologically diagnosed CAA-related inflammation, 12 with available neuroimaging and follow-up data. Patients were evaluated for MRI appearance, APOE genotype, and clinical course over a 46.8 ± 29.1-month follow-up.

Results: Baseline MRI scans were characterized by asymmetric T2-hyperintense lesions extending to the subcortical white matter and occasionally the overlying gray matter, with signal properties suggesting vasogenic edema. Subjects could be divided into three groups based on response to immunosuppressive treatment: monophasic improvement (7/12), initial improvement followed by symptomatic relapse (3/12), and no evident response to treatment (2/12). The volume of MRI hyperintensities correlated with the severity of clinical symptoms. One patient experienced symptomatic intracerebral hemorrhage within a region of recurrent MRI hyperintensity. The APOE 4/4 genotype was strongly associated with CAA-related inflammation, present in 76.9% (10/13) of subjects vs 5.1% (2/39) with symptomatic but noninflammatory CAA (p < 0.0001).

Conclusion: Cerebral amyloid angiopathy–related inflammation represents a clinically, pathologically, radiographically, and genetically distinct disease subtype with implications for clinical practice and ongoing immunotherapeutic approaches to Alzheimer disease.

Received September 7, 2006. Accepted in final form December 22, 2006.

Disclosure: The authors report no conflicts of interest.

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