Document Detail

Course of cerebral amyloid angiopathy-related inflammation.
MedLine Citation:
PMID:  17452586     Owner:  NLM     Status:  MEDLINE    
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 epsilon4/epsilon4 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.
C Kinnecom; M H Lev; L Wendell; E E Smith; J Rosand; M P Frosch; S M Greenberg
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural    
Journal Detail:
Title:  Neurology     Volume:  68     ISSN:  1526-632X     ISO Abbreviation:  Neurology     Publication Date:  2007 Apr 
Date Detail:
Created Date:  2007-04-24     Completed Date:  2007-05-10     Revised Date:  2014-04-03    
Medline Journal Info:
Nlm Unique ID:  0401060     Medline TA:  Neurology     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1411-6     Citation Subset:  AIM; IM    
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MeSH Terms
Alzheimer Disease / immunology,  therapy
Anti-Inflammatory Agents / therapeutic use
Apolipoprotein E4 / genetics
Cerebral Amyloid Angiopathy / complications*,  drug therapy,  genetics,  pathology,  radiography
Cognition Disorders / etiology
Cohort Studies
Dementia, Vascular / etiology
Disease Progression
Follow-Up Studies
Headache / etiology
Magnetic Resonance Imaging
Middle Aged
Risk Factors
Seizures / etiology
Vasculitis / etiology*,  pathology
Grant Support
Reg. No./Substance:
0/Anti-Inflammatory Agents; 0/Apolipoprotein E4

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