inflammatory cerebral amyloid angiopathy
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Epidemiology
- rare
Pathology
- autoimmune response to the vascular beta-amyloid deposits
Genetics
- apoE4/apoE4 genotype common (70-80%)
Clinical manifestations
Laboratory
- CSF analysis:
- increased CSF protein
- possibly elevated CSF lymphocytes
- brain & leptomeningeal biopsy needed for definitive diagnosis
Radiology
- diffuse white matter edema
- microhemorrhages
Differential diagnosis
Management
- patients respond to a brief course of glucocorticoids
More general terms
References
- ↑ Bernstein RA et al Clinical diagnosis and successful treatment of inflammatory cerebral amyloid angiopathy. Neurocrit Care. 2011 Jun;14(3):453-5. PMID: https://www.ncbi.nlm.nih.gov/pubmed/21246307
- ↑ Eng JA et al Clinical manifestations of cerebral amyloid angiopathy-related inflammation. Ann Neurol. 2004 Feb;55(2):250-6. PMID: https://www.ncbi.nlm.nih.gov/pubmed/14755729
- ↑ Chung KK et al Cerebral amyloid angiopathy related inflammation: three case reports and a review. J Neurol Neurosurg Psychiatry. 2011 Jan;82(1):20-6 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20935328
- ↑ Greenberg SM, Rapalino O, Frosch MP. Case 22-2010: An 87-year-old woman with dementia and a seizure. N Engl J Med 2010; 363:373-381. PMID: https://www.ncbi.nlm.nih.gov/pubmed/20660406