Contents

Search

cerebral amyloid angiopathy (CAA)

Etiology: 1) Alzheimer's disease, A4-40 form from APP (amyloid precursor protein) mostly in blood vessels 2) Icelandic type, cystatin C derived 3) familial British dementia, mutation in ITM2B gene 4) familial Danish dementia, mutation in ITM2B gene 5) high density lipoprotein deficiency associated, mutations in ABCA1 gene (600046 604091) Epidemiology: 1) incidence increases progressively with age 2) rarely evident in patients < 55 years of age Pathology: 1) amyloid deposition in: a) small & medium-sized arteries b) leptomeninges (arachnoid & pia) 2) affected vessels are generally superficial a) lobar hemorrhages b) subarachnoid hemorrhage may occur 3) cortical superficial siderosis [5] Clinical manifestations: 1) recurrent cerebral hemorrhages - repeated episodes of transient neurologic dysfunction may occur (cortical microhemorrhages) - headaches, focal neurologic symptoms, seizures, & recurrent intracranial microbleeds suggest inflammation in amyloid-affected arteries 2) episodes of delirium 3) dementia develops in 10-30% of patients Laboratory: - all CSF amyloid-beta isoforms lower in patients with CAA than in controls [11] - CSF Abeta42 decreased in patients with spontaneous cerebral amyloid angiopathy vs Alzheimer's disease [11] Special laboratory: - consider brain biopsy prior to innitiation of immunosuppression Radiology: - MRI neuroimaging - Carbon 11-labeled Pittsburgh Compound B (PiB) positron emission tomgraphy (PET) imaging is a research tool [3,4] Complications: - vascular inflammation in amyloid-affected arteries Management: - consider high-dose glucocorticoids for 5 days followed by a rapid taper - treat hypertension to a target of < 140/90 mm Hg - reconsider anticoagulation &/or anti-platelet therapy - reconsider statin therapy - consider levetiracetam or topiramate to reduce frequency & severity of transient focal neurologic signs - immunosuppression with cyclophosphamide, methotrexate or mycophenolate [7]

Interactions

disease interactions

Specific

cerebral amyloid microangiopathy (Alzheimer type) Dutch-type cerebral amyloid angiopathy familial British dementia (cerebral amyloid angiopathy ITM2B-related type 1, CAA-ITM2B1) familial Danish dementia (cerebral amyloid angiopathy ITM2B-related type 2, CAA-ITM2B2, heredopathia ophthalmo-oto-encephalica) Icelandic-type cerebral amyloid angiopathy inflammatory cerebral amyloid angiopathy

General

amyloidosis cerebrovascular disease/disorder

References

  1. UCLA Intensive Course in Geriatric Medicine & Board Review, Marina Del Ray, CA, Sept 12-15, 2001
  2. Geriatrics Review Syllabus, American Geriatrics Society, 5th edition, 2002-2004
  3. Johnson K et al, Imaging of amyloid burden and distribution in cerebral amyloid angiopathy. Ann Neurol 2007, 62:229 PMID: 17683091
  4. Ly JV et al. 11C-PIB binding is increased in patients with cerebral amyloid angiopathy-related hemorrhage. Neurology 2010 Feb 9; 74:487. PMID: 20142615
  5. Na HK et al. Cortical superficial siderosis: A marker of vascular amyloid in patients with cognitive impairment. Neurology 2015 Feb 24; 84:849 PMID: 25632096 http://www.neurology.org/content/84/8/849
  6. Charidimou A, Peeters A, Fox Z et al Spectrum of transient focal neurological episodes in cerebral amyloid angiopathy: multicentre magnetic resonance imaging cohort study and meta-analysis. Stroke. 2012 Sep;43(9):2324-30. Review. PMID: 22798323 Free Article
  7. Bergman C, Okhravi HR Cerebral Amyloid Angiopathy-Related Inflammation: A Case of Unexpected Cognitive Recovery. Ann Longterm Care. 2019;27(12):e13-e19 https://www.managedhealthcareconnect.com/articles/cerebral-amyloid-angiopathy-related-inflammation-case-unexpected-cognitive-recovery
  8. Piazza F, Greenberg SM, Savoiardo M et al Anti-amyloid beta autoantibodies in cerebral amyloid angiopathy- related inflammation: implications for amyloid-modifying therapies. Ann Neurol. 2013 Apr;73(4):449-58. PMID: 23625526
  9. Maria Sanchez-Caro J, de Lorenzo Martinez de Ubago I, de Celis Ruiz E et al Transient Focal Neurological Events in Cerebral Amyloid Angiopathy and the Long-term Risk of Intracerebral Hemorrhage and Death. A Systematic Review and Meta-analysis. JAMA Neurol. Published online November 15, 2021 PMID: 34779831 https://jamanetwork.com/journals/jamaneurology/fullarticle/2786344
  10. Bergman C, Okhravi HR Cerebral Amyloid Angiopathy-Related Inflammation: A Case of Unexpected Cognitive Recovery. Annals of Long-Term Care. 2019;27(12):e13-e19. https://www.hmpgloballearningnetwork.com/site/altc/articles/cerebral-amyloid-angiopathy-related-inflammation-case-unexpected-cognitive-recovery
  11. De Kort AM et al. Decreased cerebrospinal fluid amyloid beta 38, 40, 42, and 43 levels in sporadic and hereditary cerebral amyloid angiopathy. Ann Neurol 2023 Jan 27; [e-pub] PMID: 36707720 https://onlinelibrary.wiley.com/doi/10.1002/ana.26610

Databases & Images

OMIM 176500 image related to cerebral amyloid angiopathy