Skip to main content
AAN.com
×
Site maintenance Tuesday, May 28, 2024. Please note that new registrations and purchases will be unavailable on this date.
Articles
September 27, 2013

Aβ-related angiitis
Comparison with CAA without inflammation and primary CNS vasculitis

October 29, 2013 issue
81 (18) 1596-1603

Abstract

Objective:

To analyze the clinical findings, response to therapy, and outcomes of patients with cerebral vascular amyloid-β (Aβ) deposition with and without inflammatory vascular infiltration.

Methods:

We report 78 consecutive patients with cerebral vascular Aβ deposition examined at Mayo Clinic Rochester over 25 years (1987 through 2011). Specimens reviewed by a neuropathologist showed 40 with vascular Aβ peptide without inflammation (cerebral amyloid angiopathy [CAA]), 28 with granulomatous vasculitis (Aβ-related angiitis or ABRA), and 10 with perivascular CAA-related inflammation. We also matched findings in 118 consecutive patients with primary CNS vasculitis (PCNSV) without Aβ seen over 25 years (1983 through 2007).

Results:

Compared to the 40 with CAA, the 28 with ABRA were younger at diagnosis (p = 0.05), had less altered cognition (p = 0.02), fewer neurologic deficits (p = 0.02), and fewer intracranial hemorrhages (<0.001), but increased gadolinium leptomeningeal enhancement (p = 0.01) at presentation, and less mortality and disability at last follow-up (p < 0.001). Compared with PCNSV, the 28 patients with ABRA were older at diagnosis (p < 0.001), had a higher frequency of altered cognition (p = 0.05), seizures/spells (p = 0.006), gadolinium leptomeningeal enhancement (p < 0.001), and intracerebral hemorrhage (p = 0.02), lower frequency of hemiparesis (p = 0.01), visual symptoms (p = 0.04), and MRI evidence of cerebral infarction (p = 0.003), but higher CSF protein levels (p = 0.03). Results of treatment and outcomes in ABRA and PCNSV were similar.

Conclusions:

ABRA appears to represent a distinct subset of PCNSV.

Get full access to this article

View all available purchase options and get full access to this article.

Supplementary Material

File (table_e-1.doc)

REFERENCES

1.
Greenberg S. Cerebral amyloid angiopathy: prospects for clinical diagnosis and treatment. Neurology 1998;51:690–694.
2.
Vinters HV. Cerebral amyloid angiopathy: a critical review. Stroke 1987;18:311–324.
3.
Greenberg SM, Vonsattel JPG, Stakes JW, Gruber M, Finklestein SP. The clinical spectrum of cerebral amyloid angiopathy: presentations without lobar hemorrhage. Neurology 1993;43:2073–2079.
4.
Greenberg SM, Gurol ME, Rosand J, Smith EE. Amyloid angiopathy-related vascular cognitive impairment. Stroke 2004;35(suppl 1):2616–2619.
5.
Greenberg SM, Vonsattel JPG. Diagnosis of cerebral amyloid angiopathy: sensitivity and specificity of cortical biopsy. Stroke 1997;28:1418–1422.
6.
Ginsberg L, Geddes J, Valentine A. Amyloid angiopathy and granulomatous angiitis of the central nervous system: a case responding to corticosteroid treatment. J Neurol 1988;235:438–440.
7.
Fountain NB, Eberhard D. Primary angiitis of the central nervous system associated with cerebral amyloid angiopathy: report of two cases and review of the literature. Neurology 1996;46:190–197.
8.
Schwab P, Lidov HGW, Schwartz RB, Anderson RJ. Cerebral amyloid angiopathy associated with primary angiitis of the central nervous system: report of 2 cases and review of the literature. Arthritis Rheum 2003;49:421–427.
9.
Eng JA, Frosch MP, Choi K, Rebeck GW, Greenberg SM. Clinical manifestations of cerebral amyloid angiopathy-related inflammation. Ann Neurol 2004;55:250–256.
10.
Kinnecom C, Lev MH, Wendell L, et al. Course of cerebral amyloid angiopathy-related inflammation. Neurology 2007;68:1411–1416.
11.
Scolding NJ, Joseph F, Kirby PA, et al. Aβ-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy. Brain 2005;128:500–515.
12.
Salvarani C, Brown RD, Calamia KT, et al. Primary central nervous system vasculitis: comparison of patients with and without cerebral amyloid angiopathy. Rheumatology 2008;47:1671–1677.
13.
Salvarani C, Brown RD, Calamia KT, et al. Primary central nervous system vasculitis presenting with intracranial hemorrhage. Arthritis Rheum 2011;63:3598–3606.
14.
Giannini C, Salvarani C, Hunder G, Brown RD. Primary central nervous system vasculitis: pathology and mechanisms. Acta Neuropathol 2012;123:759–772.
15.
Salvarani C, Brown RD, Calamia KT, et al. Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol 2007;62:442–451.
16.
Sulter G, Steen C, De Keyser J. Use of the Barthel Index and modified Rankin Scale in acute stroke trials. Stroke 1999;30:1538–1541.
17.
Salvarani C, Brown RD, Hunder GG. Adult primary central nervous system vasculitis. Lancet 2012;380:767–777.
18.
Silbert PL, Bartleson JD, Miller GM, Parisi JE, Goldman MS, Meyer FB. Cortical petechial hemorrhage, leukoencephalopathy, and subacute dementia associated with seizures due to cerebral amyloid angiopathy. Mayo Clin Proc 1995;70:477–480.
19.
Oh U, Gupta R, Krakauer JW, Khandji AG, Chin SS, Elkind MS. Reversible leukoencephalopathy associated with cerebral amyloid angiopathy. Neurology 2004;62:494–497.
20.
DiFrancesco JC, Brioschi M, Brighina L, et al. Anti-Aβ autoantibodies in the CSF of a patient with CAA-related inflammation: a case report. Neurology 2011;76:842–844.
21.
Hermann DM, Keyvani K, van de Nes J, et al. Brain-reactive β-amyloid antibodies in primary CNS angiitis with cerebral amyloid angiopathy. Neurology 2011;77:503–505.
22.
Orgogozo JM, Gilman S, Dartigues JF, et al. Subacute meningoencephalitis in a subset of patients with AD after Abeta42 immunization. Neurology 2003;61:46–54.
23.
Sperling R, Salloway S, Brooks DJ, et al. Amyloid-related imaging abnormalities in patients with Alzheimer's disease treated with bapineuzumab: a retrospective analysis. Lancet Neurol 2012;11:241–249.

Information & Authors

Information

Published In

Neurology®
Volume 81Number 18October 29, 2013
Pages: 1596-1603
PubMed: 24078731

Publication History

Received: May 7, 2013
Accepted: August 2, 2013
Published online: September 27, 2013
Published in print: October 29, 2013

Permissions

Request permissions for this article.

Disclosure

The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.

Study Funding

No targeted funding reported.

Authors

Affiliations & Disclosures

Carlo Salvarani, MD
From the Unità Operativa di Reumatologia (C.S.), Azienda Ospedaliera ASMN, Istituto di Ricovero e Cura a Carattere Scientifico, Reggio Emilia, Italy; and Anatomic Pathology, Department of Laboratory Medicine and Pathology (C.G.), Departments of Radiology (J.M.M.) and Neurology (R.D.B.), and Divisions of Biostatistics (T.C.) and Rheumatology (G.G.H.), Mayo Clinic, Rochester, MN.
Disclosure
Scientific Advisory Boards:
1.
NONE
Gifts:
1.
NONE
Funding for Travel or Speaker Honoraria:
1.
NONE
Editorial Boards:
1.
(1) Clinical and Experimental Rheumtology, Editor Vasculitis Supplement, 2008-2013
Patents:
1.
NONE
Publishing Royalties:
1.
NONE
Employment, Commercial Entity:
1.
NONE
Consultancies:
1.
NONE
Speakers' Bureaus:
1.
NONE
Other Activities:
1.
NONE
Clinical Procedures or Imaging Studies:
1.
NONE
Research Support, Commercial Entities:
1.
NONE
Research Support, Government Entities:
1.
NONE
Research Support, Academic Entities:
1.
NONE
Research Support, Foundations and Societies:
1.
NONE
Stock/stock Options/board of Directors Compensation:
1.
NONE
License Fee Payments, Technology or Inventions:
1.
NONE
Royalty Payments, Technology or Inventions:
1.
NONE
Stock/stock Options, Research Sponsor:
1.
NONE
Stock/stock Options, Medical Equipment & Materials:
1.
NONE
Legal Proceedings:
1.
NONE
Gene G. Hunder, MD
From the Unità Operativa di Reumatologia (C.S.), Azienda Ospedaliera ASMN, Istituto di Ricovero e Cura a Carattere Scientifico, Reggio Emilia, Italy; and Anatomic Pathology, Department of Laboratory Medicine and Pathology (C.G.), Departments of Radiology (J.M.M.) and Neurology (R.D.B.), and Divisions of Biostatistics (T.C.) and Rheumatology (G.G.H.), Mayo Clinic, Rochester, MN.
Disclosure
Scientific Advisory Boards:
1.
NONE
Gifts:
1.
NONE
Funding for Travel or Speaker Honoraria:
1.
NONE
Editorial Boards:
1.
NONE
Patents:
1.
NONE
Publishing Royalties:
1.
NONE
Employment, Commercial Entity:
1.
NONE
Consultancies:
1.
NONE
Speakers' Bureaus:
1.
NONE
Other Activities:
1.
NONE
Clinical Procedures or Imaging Studies:
1.
NONE
Research Support, Commercial Entities:
1.
NONE
Research Support, Government Entities:
1.
NONE
Research Support, Academic Entities:
1.
NONE
Research Support, Foundations and Societies:
1.
NONE
Stock/stock Options/board of Directors Compensation:
1.
NONE
License Fee Payments, Technology or Inventions:
1.
NONE
Royalty Payments, Technology or Inventions:
1.
NONE
Stock/stock Options, Research Sponsor:
1.
NONE
Stock/stock Options, Medical Equipment & Materials:
1.
NONE
Legal Proceedings:
1.
NONE
Jonathan M. Morris, MD
From the Unità Operativa di Reumatologia (C.S.), Azienda Ospedaliera ASMN, Istituto di Ricovero e Cura a Carattere Scientifico, Reggio Emilia, Italy; and Anatomic Pathology, Department of Laboratory Medicine and Pathology (C.G.), Departments of Radiology (J.M.M.) and Neurology (R.D.B.), and Divisions of Biostatistics (T.C.) and Rheumatology (G.G.H.), Mayo Clinic, Rochester, MN.
Disclosure
Scientific Advisory Boards:
1.
NONE
Gifts:
1.
NONE
Funding for Travel or Speaker Honoraria:
1.
NONE
Editorial Boards:
1.
NONE
Patents:
1.
NONE
Publishing Royalties:
1.
NONE
Employment, Commercial Entity:
1.
NONE
Consultancies:
1.
NONE
Speakers' Bureaus:
1.
NONE
Other Activities:
1.
NONE
Clinical Procedures or Imaging Studies:
1.
NONE
Research Support, Commercial Entities:
1.
NONE
Research Support, Government Entities:
1.
NONE
Research Support, Academic Entities:
1.
NONE
Research Support, Foundations and Societies:
1.
NONE
Stock/stock Options/board of Directors Compensation:
1.
NONE
License Fee Payments, Technology or Inventions:
1.
NONE
Royalty Payments, Technology or Inventions:
1.
NONE
Stock/stock Options, Research Sponsor:
1.
NONE
Stock/stock Options, Medical Equipment & Materials:
1.
NONE
Legal Proceedings:
1.
NONE
Robert D. Brown, Jr, MD, MPH
From the Unità Operativa di Reumatologia (C.S.), Azienda Ospedaliera ASMN, Istituto di Ricovero e Cura a Carattere Scientifico, Reggio Emilia, Italy; and Anatomic Pathology, Department of Laboratory Medicine and Pathology (C.G.), Departments of Radiology (J.M.M.) and Neurology (R.D.B.), and Divisions of Biostatistics (T.C.) and Rheumatology (G.G.H.), Mayo Clinic, Rochester, MN.
Disclosure
Scientific Advisory Boards:
1.
NONE
Gifts:
1.
NONE
Funding for Travel or Speaker Honoraria:
1.
NONE
Editorial Boards:
1.
NONE
Patents:
1.
NONE
Publishing Royalties:
1.
LWW publisher, Handbook of Stroke, 2005 Mayo Clinic Press, Mayo Clinic Internal Medicine Board Review
Employment, Commercial Entity:
1.
NONE
Consultancies:
1.
NONE
Speakers' Bureaus:
1.
NONE
Other Activities:
1.
Neilsen Foundation, Board of Directors, 2007-current Brain Aneurysm Foundation, Medical Advisory Board, 2009- current Joe Neikro Foundation for Aneurysm Research, Medical Advisory Board, 2011-
Clinical Procedures or Imaging Studies:
1.
NONE
Research Support, Commercial Entities:
1.
NONE
Research Support, Government Entities:
1.
National Institutes of Health, NINDS -International Study of Unruptured Intracranial Aneurysms, ongoing, Principal Investigator -Familial Intracranial Aneurysm study, ongoing, Co-I -Siblings with Ischemic Stroke Study, ongoing, Co-I -Stroke Genetics Consortium, ongoing, Co-I Mayo Clinic-Czech Republic Research Collaboration -Stroke Project, Co-PI
Research Support, Academic Entities:
1.
NONE
Research Support, Foundations and Societies:
1.
NONE
Stock/stock Options/board of Directors Compensation:
1.
NONE
License Fee Payments, Technology or Inventions:
1.
NONE
Royalty Payments, Technology or Inventions:
1.
NONE
Stock/stock Options, Research Sponsor:
1.
NONE
Stock/stock Options, Medical Equipment & Materials:
1.
NONE
Legal Proceedings:
1.
NONE
Teresa Christianson
From the Unità Operativa di Reumatologia (C.S.), Azienda Ospedaliera ASMN, Istituto di Ricovero e Cura a Carattere Scientifico, Reggio Emilia, Italy; and Anatomic Pathology, Department of Laboratory Medicine and Pathology (C.G.), Departments of Radiology (J.M.M.) and Neurology (R.D.B.), and Divisions of Biostatistics (T.C.) and Rheumatology (G.G.H.), Mayo Clinic, Rochester, MN.
Disclosure
Scientific Advisory Boards:
1.
NONE
Gifts:
1.
NONE
Funding for Travel or Speaker Honoraria:
1.
NONE
Editorial Boards:
1.
NONE
Patents:
1.
NONE
Publishing Royalties:
1.
NONE
Employment, Commercial Entity:
1.
NONE
Consultancies:
1.
NONE
Speakers' Bureaus:
1.
NONE
Other Activities:
1.
NONE
Clinical Procedures or Imaging Studies:
1.
NONE
Research Support, Commercial Entities:
1.
NONE
Research Support, Government Entities:
1.
NONE
Research Support, Academic Entities:
1.
NONE
Research Support, Foundations and Societies:
1.
NONE
Stock/stock Options/board of Directors Compensation:
1.
NONE
License Fee Payments, Technology or Inventions:
1.
NONE
Royalty Payments, Technology or Inventions:
1.
NONE
Stock/stock Options, Research Sponsor:
1.
NONE
Stock/stock Options, Medical Equipment & Materials:
1.
NONE
Legal Proceedings:
1.
NONE
Caterina Giannini, MD, PhD
From the Unità Operativa di Reumatologia (C.S.), Azienda Ospedaliera ASMN, Istituto di Ricovero e Cura a Carattere Scientifico, Reggio Emilia, Italy; and Anatomic Pathology, Department of Laboratory Medicine and Pathology (C.G.), Departments of Radiology (J.M.M.) and Neurology (R.D.B.), and Divisions of Biostatistics (T.C.) and Rheumatology (G.G.H.), Mayo Clinic, Rochester, MN.
Disclosure
Scientific Advisory Boards:
1.
NONE
Gifts:
1.
NONE
Funding for Travel or Speaker Honoraria:
1.
NONE
Editorial Boards:
1.
NONE
Patents:
1.
NONE
Publishing Royalties:
1.
NONE
Employment, Commercial Entity:
1.
NONE
Consultancies:
1.
NONE
Speakers' Bureaus:
1.
NONE
Other Activities:
1.
NONE
Clinical Procedures or Imaging Studies:
1.
NONE
Research Support, Commercial Entities:
1.
NONE
Research Support, Government Entities:
1.
NONE
Research Support, Academic Entities:
1.
NONE
Research Support, Foundations and Societies:
1.
NONE
Stock/stock Options/board of Directors Compensation:
1.
NONE
License Fee Payments, Technology or Inventions:
1.
NONE
Royalty Payments, Technology or Inventions:
1.
NONE
Stock/stock Options, Research Sponsor:
1.
NONE
Stock/stock Options, Medical Equipment & Materials:
1.
NONE
Legal Proceedings:
1.
NONE

Notes

Correspondence to Dr. Salvarani: [email protected]
Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

Author Contributions

Dr. Carlo Salvarani: study design, acquisition of the data, interpretation of the data, drafting/revising the manuscript for content. Dr. Gene G. Hunder: study design, interpretation of the data, drafting/revising the manuscript for content. Dr. Jonathan M. Morris: acquisition of the data, interpretation of the data, revising the manuscript for content. Dr. Robert D. Brown, Jr.: study design, interpretation of the data, revising the manuscript for content. Teresa Christianson: statistical analysis, revising the manuscript for content. Dr. Caterina Giannini: study design, acquisition of the data, interpretation of the data, revising the manuscript for content.

Metrics & Citations

Metrics

Citations

Download Citations

If you have the appropriate software installed, you can download article citation data to the citation manager of your choice. Select your manager software from the list below and click Download.

Cited By
  1. Imaging of Amyloid-beta-related Arteritis, Neuroimaging Clinics of North America, 34, 1, (167-173), (2024).https://doi.org/10.1016/j.nic.2023.09.001
    Crossref
  2. Pathology of Primary Angiitis of the Central Nervous System, Neuroimaging Clinics of North America, 34, 1, (31-37), (2024).https://doi.org/10.1016/j.nic.2023.06.002
    Crossref
  3. Inflammatory Cerebral Amyloid Angiopathy: A Broad Clinical Spectrum, Journal of Clinical Neurology, 19, 3, (230), (2023).https://doi.org/10.3988/jcn.2022.0493
    Crossref
  4. Rare forms of cerebral amyloid angiopathy: pathogenesis, biological and clinical features of CAA-ri and iCAA, Frontiers in Neuroscience, 17, (2023).https://doi.org/10.3389/fnins.2023.1219025
    Crossref
  5. Cerebral amyloid angiopathy-related cardiac injury: Focus on cardiac cell death, Frontiers in Cell and Developmental Biology, 11, (2023).https://doi.org/10.3389/fcell.2023.1156970
    Crossref
  6. Neuropathology of Anti-Amyloid-β Immunotherapy: A Case Report, Journal of Alzheimer's Disease, 93, 2, (803-813), (2023).https://doi.org/10.3233/JAD-221305
    Crossref
  7. Primary Angiitis of the Central Nervous System in Adults: A Comprehensive Review of 76 Biopsy-Proven Case Reports, Journal of Inflammation Research, Volume 16, (5083-5094), (2023).https://doi.org/10.2147/JIR.S434126
    Crossref
  8. Clinical, Neuroimaging, and Genetic Markers in Cerebral Amyloid Angiopathy-Related Inflammation: A Systematic Review and Meta-Analysis, Stroke, 54, 1, (178-188), (2023).https://doi.org/10.1161/STROKEAHA.122.040671
    Crossref
  9. Convexity subarachnoid haemorrhage: a practical guide, Practical Neurology, 23, 5, (368-375), (2023).https://doi.org/10.1136/pn-2022-003572
    Crossref
  10. Incidence and risk factors associated with seizures in cerebral amyloid angiopathy, European Journal of Neurology, 30, 12, (3682-3691), (2023).https://doi.org/10.1111/ene.15903
    Crossref
  11. See more
Loading...

View Options

Get Access

Login options

Check if you have access through your login credentials or your institution to get full access on this article.

Personal login Institutional Login
Purchase Options

Purchase this article to get full access to it.

Purchase Access, $39 for 24hr of access

View options

Full Text

View Full Text

Full Text HTML

View Full Text HTML

Media

Figures

Other

Tables

Share

Share

Share article link

Share