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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.

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Supplementary Material

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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

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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.

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