cerebral amyloid angiopathy (CAA)
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Etiology
- Alzheimer's disease, A4-40 form from APP (amyloid precursor protein) mostly in blood vessels
- Icelandic type, cystatin C derived
- familial British dementia, mutation in ITM2B gene
- familial Danish dementia, mutation in ITM2B gene
- high density lipoprotein deficiency associated, mutations in ABCA1 gene (600046 604091)
Epidemiology
- incidence increases progressively with age
- rarely evident in patients < 55 years of age
Pathology
- amyloid deposition in:
- small & medium-sized arteries
- leptomeninges (arachnoid & pia)
- affected vessels are generally superficial
- lobar hemorrhages
- subarachnoid hemorrhage may occur
- cortical superficial siderosis[5]
Clinical manifestations
- 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
- episodes of delirium
- 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]
Diagnostic procedures
- 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]
More general terms
More specific terms
- 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
References
- ↑ UCLA Intensive Course in Geriatric Medicine & Board Review, Marina Del Ray, CA, Sept 12-15, 2001
- ↑ Geriatrics Review Syllabus, American Geriatrics Society, 5th edition, 2002-2004
- ↑ 3.0 3.1 Johnson K et al, Imaging of amyloid burden and distribution in cerebral amyloid angiopathy. Ann Neurol 2007, 62:229 PMID: https://www.ncbi.nlm.nih.gov/pubmed/17683091
- ↑ 4.0 4.1 Ly JV et al. 11C-PIB binding is increased in patients with cerebral amyloid angiopathy-related hemorrhage. Neurology 2010 Feb 9; 74:487. PMID: https://www.ncbi.nlm.nih.gov/pubmed/20142615
- ↑ 5.0 5.1 Na HK et al. Cortical superficial siderosis: A marker of vascular amyloid in patients with cognitive impairment. Neurology 2015 Feb 24; 84:849 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/25632096 <Internet> http://www.neurology.org/content/84/8/849
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/22798323 Free Article
- ↑ 7.0 7.1 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
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/23625526
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/34779831 https://jamanetwork.com/journals/jamaneurology/fullarticle/2786344
- ↑ 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
- ↑ 11.0 11.1 11.2 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: https://www.ncbi.nlm.nih.gov/pubmed/36707720 https://onlinelibrary.wiley.com/doi/10.1002/ana.26610
Patient information
cerebral amyloid angiopathy patient information