Primary central nervous system vasculitis with prominent leptomeningeal enhancement: A subset with a benign outcome

Carlo Salvarani, Robert D Jr. Brown, Kenneth T. Calamia, Teresa J H Christianson, John III Huston, James F Meschia, Caterina Giannini, Dylan V. Miller, Gene G. Hunder

Research output: Contribution to journalArticle

62 Citations (Scopus)

Abstract

Objective. Primary central nervous system vasculitis (PCNSV) is an uncommon condition that affects the brain and spinal cord. This study was undertaken to evaluate the clinical features and outcomes among patients with PCNSV who presented with prominent gadolinium meningeal enhancement on magnetic resonance imaging (MRI). Methods. Through retrospective review using the Mayo Clinic medical records linkage system, we identified 101 consecutive patients with PCNSV based on brain biopsy or conventional angiography (or both) between January 1, 1983, and December 31, 2003. We evaluated data on demographics, clinical findings, laboratory studies, imaging, biopsy of brain or spinal cord (or both), treatment, and neurologic outcome. Results. MRIs showed prominent leptomeningeal enhancement in 8 of 101 patients with PCNSV. In 6 of those 8, cerebral angiography or magnetic resonance angiography results were normal, but biopsy of the brain or spinal cord showed vasculitis in all 8. Granulomatous vascular inflammation was found in 6 specimens and was associated in 4 cases with vascular deposits of β-amyloid peptide. All 8 patients had a prompt response to therapy, with resolution of the MRI meningeal enhancement. Although 3 of the 8 patients had relapses during followup, the overall outcome was favorable. Patients with meningeal enhancement, compared with patients without enhancement, more commonly had substantial abnormalities of cerebrospinal fluid (100% versus 58%; P = 0.02) and amyloid angiopathy (50% versus 12%; P = 0.03). Conclusion. Prominent gadolinium leptomeningeal enhancement on MRI may point to a distinct subtype of PCNSV with small leptomeningeal artery vasculitis and rapid response to therapy.

Original languageEnglish (US)
Pages (from-to)595-603
Number of pages9
JournalArthritis and Rheumatism
Volume58
Issue number2
DOIs
StatePublished - Feb 2008

Fingerprint

Central Nervous System Vasculitis
Spinal Cord
Magnetic Resonance Imaging
Gadolinium
Vasculitis
Biopsy
Blood Vessels
Medical Record Linkage
Brain
Cerebral Angiography
Magnetic Resonance Angiography
Amyloid Plaques
Neuroimaging
Nervous System
Cerebrospinal Fluid
Angiography
Arteries
Demography
Inflammation
Recurrence

ASJC Scopus subject areas

  • Immunology
  • Rheumatology

Cite this

Primary central nervous system vasculitis with prominent leptomeningeal enhancement : A subset with a benign outcome. / Salvarani, Carlo; Brown, Robert D Jr.; Calamia, Kenneth T.; Christianson, Teresa J H; Huston, John III; Meschia, James F; Giannini, Caterina; Miller, Dylan V.; Hunder, Gene G.

In: Arthritis and Rheumatism, Vol. 58, No. 2, 02.2008, p. 595-603.

Research output: Contribution to journalArticle

Salvarani, Carlo ; Brown, Robert D Jr. ; Calamia, Kenneth T. ; Christianson, Teresa J H ; Huston, John III ; Meschia, James F ; Giannini, Caterina ; Miller, Dylan V. ; Hunder, Gene G. / Primary central nervous system vasculitis with prominent leptomeningeal enhancement : A subset with a benign outcome. In: Arthritis and Rheumatism. 2008 ; Vol. 58, No. 2. pp. 595-603.
@article{57e025d06fd14136a101a3f64a9e7dbc,
title = "Primary central nervous system vasculitis with prominent leptomeningeal enhancement: A subset with a benign outcome",
abstract = "Objective. Primary central nervous system vasculitis (PCNSV) is an uncommon condition that affects the brain and spinal cord. This study was undertaken to evaluate the clinical features and outcomes among patients with PCNSV who presented with prominent gadolinium meningeal enhancement on magnetic resonance imaging (MRI). Methods. Through retrospective review using the Mayo Clinic medical records linkage system, we identified 101 consecutive patients with PCNSV based on brain biopsy or conventional angiography (or both) between January 1, 1983, and December 31, 2003. We evaluated data on demographics, clinical findings, laboratory studies, imaging, biopsy of brain or spinal cord (or both), treatment, and neurologic outcome. Results. MRIs showed prominent leptomeningeal enhancement in 8 of 101 patients with PCNSV. In 6 of those 8, cerebral angiography or magnetic resonance angiography results were normal, but biopsy of the brain or spinal cord showed vasculitis in all 8. Granulomatous vascular inflammation was found in 6 specimens and was associated in 4 cases with vascular deposits of β-amyloid peptide. All 8 patients had a prompt response to therapy, with resolution of the MRI meningeal enhancement. Although 3 of the 8 patients had relapses during followup, the overall outcome was favorable. Patients with meningeal enhancement, compared with patients without enhancement, more commonly had substantial abnormalities of cerebrospinal fluid (100{\%} versus 58{\%}; P = 0.02) and amyloid angiopathy (50{\%} versus 12{\%}; P = 0.03). Conclusion. Prominent gadolinium leptomeningeal enhancement on MRI may point to a distinct subtype of PCNSV with small leptomeningeal artery vasculitis and rapid response to therapy.",
author = "Carlo Salvarani and Brown, {Robert D Jr.} and Calamia, {Kenneth T.} and Christianson, {Teresa J H} and Huston, {John III} and Meschia, {James F} and Caterina Giannini and Miller, {Dylan V.} and Hunder, {Gene G.}",
year = "2008",
month = "2",
doi = "10.1002/art.23300",
language = "English (US)",
volume = "58",
pages = "595--603",
journal = "Arthritis and Rheumatology",
issn = "2326-5191",
publisher = "John Wiley and Sons Ltd",
number = "2",

}

TY - JOUR

T1 - Primary central nervous system vasculitis with prominent leptomeningeal enhancement

T2 - A subset with a benign outcome

AU - Salvarani, Carlo

AU - Brown, Robert D Jr.

AU - Calamia, Kenneth T.

AU - Christianson, Teresa J H

AU - Huston, John III

AU - Meschia, James F

AU - Giannini, Caterina

AU - Miller, Dylan V.

AU - Hunder, Gene G.

PY - 2008/2

Y1 - 2008/2

N2 - Objective. Primary central nervous system vasculitis (PCNSV) is an uncommon condition that affects the brain and spinal cord. This study was undertaken to evaluate the clinical features and outcomes among patients with PCNSV who presented with prominent gadolinium meningeal enhancement on magnetic resonance imaging (MRI). Methods. Through retrospective review using the Mayo Clinic medical records linkage system, we identified 101 consecutive patients with PCNSV based on brain biopsy or conventional angiography (or both) between January 1, 1983, and December 31, 2003. We evaluated data on demographics, clinical findings, laboratory studies, imaging, biopsy of brain or spinal cord (or both), treatment, and neurologic outcome. Results. MRIs showed prominent leptomeningeal enhancement in 8 of 101 patients with PCNSV. In 6 of those 8, cerebral angiography or magnetic resonance angiography results were normal, but biopsy of the brain or spinal cord showed vasculitis in all 8. Granulomatous vascular inflammation was found in 6 specimens and was associated in 4 cases with vascular deposits of β-amyloid peptide. All 8 patients had a prompt response to therapy, with resolution of the MRI meningeal enhancement. Although 3 of the 8 patients had relapses during followup, the overall outcome was favorable. Patients with meningeal enhancement, compared with patients without enhancement, more commonly had substantial abnormalities of cerebrospinal fluid (100% versus 58%; P = 0.02) and amyloid angiopathy (50% versus 12%; P = 0.03). Conclusion. Prominent gadolinium leptomeningeal enhancement on MRI may point to a distinct subtype of PCNSV with small leptomeningeal artery vasculitis and rapid response to therapy.

AB - Objective. Primary central nervous system vasculitis (PCNSV) is an uncommon condition that affects the brain and spinal cord. This study was undertaken to evaluate the clinical features and outcomes among patients with PCNSV who presented with prominent gadolinium meningeal enhancement on magnetic resonance imaging (MRI). Methods. Through retrospective review using the Mayo Clinic medical records linkage system, we identified 101 consecutive patients with PCNSV based on brain biopsy or conventional angiography (or both) between January 1, 1983, and December 31, 2003. We evaluated data on demographics, clinical findings, laboratory studies, imaging, biopsy of brain or spinal cord (or both), treatment, and neurologic outcome. Results. MRIs showed prominent leptomeningeal enhancement in 8 of 101 patients with PCNSV. In 6 of those 8, cerebral angiography or magnetic resonance angiography results were normal, but biopsy of the brain or spinal cord showed vasculitis in all 8. Granulomatous vascular inflammation was found in 6 specimens and was associated in 4 cases with vascular deposits of β-amyloid peptide. All 8 patients had a prompt response to therapy, with resolution of the MRI meningeal enhancement. Although 3 of the 8 patients had relapses during followup, the overall outcome was favorable. Patients with meningeal enhancement, compared with patients without enhancement, more commonly had substantial abnormalities of cerebrospinal fluid (100% versus 58%; P = 0.02) and amyloid angiopathy (50% versus 12%; P = 0.03). Conclusion. Prominent gadolinium leptomeningeal enhancement on MRI may point to a distinct subtype of PCNSV with small leptomeningeal artery vasculitis and rapid response to therapy.

UR - http://www.scopus.com/inward/record.url?scp=39449094984&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=39449094984&partnerID=8YFLogxK

U2 - 10.1002/art.23300

DO - 10.1002/art.23300

M3 - Article

C2 - 18240248

AN - SCOPUS:39449094984

VL - 58

SP - 595

EP - 603

JO - Arthritis and Rheumatology

JF - Arthritis and Rheumatology

SN - 2326-5191

IS - 2

ER -