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inflammatory cerebral amyloid angiopathy

Epidemiology: - rare Pathology: - autoimmune response to the vascular beta-amyloid deposits Genetics: - apoE4/apoE4 genotype common (70-80%) Clinical manifestations: - cognitive decline may be subacute - seizures - headache 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: - posterior reversible encephalopathy syndrome Management: - patients respond to a brief course of glucocorticoids - prednisone

General

cerebral amyloid angiopathy (CAA)

References

  1. Bernstein RA et al Clinical diagnosis and successful treatment of inflammatory cerebral amyloid angiopathy. Neurocrit Care. 2011 Jun;14(3):453-5. PMID: 21246307
  2. Eng JA et al Clinical manifestations of cerebral amyloid angiopathy-related inflammation. Ann Neurol. 2004 Feb;55(2):250-6. PMID: 14755729
  3. 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: 20935328
  4. 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: 20660406