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November 2021; 8 (6) ArticleOpen Access

Vascular Involvement in Neurosarcoidosis

Early Experiences From Intracranial Vessel Wall Imaging

View ORCID ProfileGirish Bathla, View ORCID ProfileLama Abdel-Wahed, Amit Agarwal, Tracey A. Cho, Sarika Gupta, Karra A. Jones, Sarv Priya, Neetu Soni, Bruce A. Wasserman
First published August 4, 2021, DOI: https://doi.org/10.1212/NXI.0000000000001063
Girish Bathla
From the Department of Radiology (G.B., S.P., N.S.), University of Iowa Hospitals and Clinics; Department of Neurology (L.A.-W., T.A.C.), University of Iowa Hospitals and Clinics, IA; Department of Radiology (A.A.), University of Texas Southwestern Medical Center; Department Pathology (S.G., K.A.J.), University of Iowa Hospitals and Clinics, IA; and Department of Radiology (B.A.W.), Johns Hopkins School of Medicine, Baltimore, MD.
MBBS, FRCR, MMeD, DABR
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  • ORCID record for Girish Bathla
Lama Abdel-Wahed
From the Department of Radiology (G.B., S.P., N.S.), University of Iowa Hospitals and Clinics; Department of Neurology (L.A.-W., T.A.C.), University of Iowa Hospitals and Clinics, IA; Department of Radiology (A.A.), University of Texas Southwestern Medical Center; Department Pathology (S.G., K.A.J.), University of Iowa Hospitals and Clinics, IA; and Department of Radiology (B.A.W.), Johns Hopkins School of Medicine, Baltimore, MD.
MD
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  • ORCID record for Lama Abdel-Wahed
  • For correspondence: lama-abdelwahed@uiowa.edu
Amit Agarwal
From the Department of Radiology (G.B., S.P., N.S.), University of Iowa Hospitals and Clinics; Department of Neurology (L.A.-W., T.A.C.), University of Iowa Hospitals and Clinics, IA; Department of Radiology (A.A.), University of Texas Southwestern Medical Center; Department Pathology (S.G., K.A.J.), University of Iowa Hospitals and Clinics, IA; and Department of Radiology (B.A.W.), Johns Hopkins School of Medicine, Baltimore, MD.
MD
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  • For correspondence: amit.agarwal@utsouthwestern.edu
Tracey A. Cho
From the Department of Radiology (G.B., S.P., N.S.), University of Iowa Hospitals and Clinics; Department of Neurology (L.A.-W., T.A.C.), University of Iowa Hospitals and Clinics, IA; Department of Radiology (A.A.), University of Texas Southwestern Medical Center; Department Pathology (S.G., K.A.J.), University of Iowa Hospitals and Clinics, IA; and Department of Radiology (B.A.W.), Johns Hopkins School of Medicine, Baltimore, MD.
MD
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  • For correspondence: tracey-cho@uiowa.edu
Sarika Gupta
From the Department of Radiology (G.B., S.P., N.S.), University of Iowa Hospitals and Clinics; Department of Neurology (L.A.-W., T.A.C.), University of Iowa Hospitals and Clinics, IA; Department of Radiology (A.A.), University of Texas Southwestern Medical Center; Department Pathology (S.G., K.A.J.), University of Iowa Hospitals and Clinics, IA; and Department of Radiology (B.A.W.), Johns Hopkins School of Medicine, Baltimore, MD.
MD, FRCP
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  • For correspondence: sarika-gupta@uiowa.edu
Karra A. Jones
From the Department of Radiology (G.B., S.P., N.S.), University of Iowa Hospitals and Clinics; Department of Neurology (L.A.-W., T.A.C.), University of Iowa Hospitals and Clinics, IA; Department of Radiology (A.A.), University of Texas Southwestern Medical Center; Department Pathology (S.G., K.A.J.), University of Iowa Hospitals and Clinics, IA; and Department of Radiology (B.A.W.), Johns Hopkins School of Medicine, Baltimore, MD.
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  • For correspondence: karra-jones@uiowa.edu
Sarv Priya
From the Department of Radiology (G.B., S.P., N.S.), University of Iowa Hospitals and Clinics; Department of Neurology (L.A.-W., T.A.C.), University of Iowa Hospitals and Clinics, IA; Department of Radiology (A.A.), University of Texas Southwestern Medical Center; Department Pathology (S.G., K.A.J.), University of Iowa Hospitals and Clinics, IA; and Department of Radiology (B.A.W.), Johns Hopkins School of Medicine, Baltimore, MD.
MD
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  • For correspondence: sarv-priya@uiowa.edu
Neetu Soni
From the Department of Radiology (G.B., S.P., N.S.), University of Iowa Hospitals and Clinics; Department of Neurology (L.A.-W., T.A.C.), University of Iowa Hospitals and Clinics, IA; Department of Radiology (A.A.), University of Texas Southwestern Medical Center; Department Pathology (S.G., K.A.J.), University of Iowa Hospitals and Clinics, IA; and Department of Radiology (B.A.W.), Johns Hopkins School of Medicine, Baltimore, MD.
MD, DNB
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  • For correspondence: neetu-soni@uiowa.edu
Bruce A. Wasserman
From the Department of Radiology (G.B., S.P., N.S.), University of Iowa Hospitals and Clinics; Department of Neurology (L.A.-W., T.A.C.), University of Iowa Hospitals and Clinics, IA; Department of Radiology (A.A.), University of Texas Southwestern Medical Center; Department Pathology (S.G., K.A.J.), University of Iowa Hospitals and Clinics, IA; and Department of Radiology (B.A.W.), Johns Hopkins School of Medicine, Baltimore, MD.
MD
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  • For correspondence: bwasser@jhmi.edu
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Citation
Vascular Involvement in Neurosarcoidosis
Early Experiences From Intracranial Vessel Wall Imaging
Girish Bathla, Lama Abdel-Wahed, Amit Agarwal, Tracey A. Cho, Sarika Gupta, Karra A. Jones, Sarv Priya, Neetu Soni, Bruce A. Wasserman
Neurol Neuroimmunol Neuroinflamm Nov 2021, 8 (6) e1063; DOI: 10.1212/NXI.0000000000001063

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Abstract

Background and Objectives Cerebrovascular manifestations in neurosarcoidosis (NS) were previously considered rare but are being increasingly recognized. We report our preliminary experience in patients with NS who underwent high-resolution vessel wall imaging (VWI).

Methods A total of 13 consecutive patients with NS underwent VWI. Images were analyzed by 2 neuroradiologists in consensus. The assessment included segment-wise evaluation of larger- and medium-sized vessels (internal carotid artery, M1-M3 middle cerebral artery; A1-A3 anterior cerebral artery; V4 segments of vertebral arteries; basilar artery; and P1-P3 posterior cerebral artery), lenticulostriate perforator vessels, and medullary and deep cerebral veins. Cortical veins were not assessed due to flow-related artifacts. Brain biopsy findings were available in 6 cases and were also reviewed.

Results Mean patient age was 54.9 years (33–71 years) with an M:F of 8:5. Mean duration between initial diagnosis and VWI study was 18 months. Overall, 9/13 (69%) patients had vascular abnormalities. Circumferential large vessel enhancement was seen in 3/13 (23%) patients, whereas perforator vessel involvement was seen in 6/13 (46%) patients. Medullary and deep vein involvement was also seen in 6/13 patients. In addition, 7/13 (54%) patients had microhemorrhages in susceptibility-weighted imaging, and 4/13 (31%) had chronic infarcts. On biopsy, 5/6 cases showed perivascular granulomas with vessel wall involvement in all 5 cases.

Discussion Our preliminary findings suggest that involvement of intracranial vascular structures may be a common finding in patients with NS and should be routinely looked for. These findings appear concordant with previously reported autopsy literature and need to be validated on a larger scale.

Glossary

AZA=
azathioprine;
CAD=
coronary artery disease;
CNE=
cranial nerve enhancement;
DM-2=
type 2 diabetes;
DWI=
diffusion-weighted imaging;
EE=
ependymal enhancement;
FLAIR=
fluid-attenuated inversion recovery;
HC=
hydrocephalus;
HL=
hyperlipidemia;
HT=
hypertension;
INX=
infliximab;
LME=
leptomeningeal enhancement;
LSP=
lenticulostriate perforator;
MMF=
mycophenolate mofetil;
MinIP=
minimum intensity projection;
MTX=
methotrexate;
NEWM=
nonenhancing white matter;
NS=
neurosarcoidosis;
NT=
not tested;
PGE=
parenchymal granulomatous enhancement;
PME=
pachymeningeal enhancement;
PVE=
perivascular enhancement;
RTX=
rituximab;
SPACE=
sampling perfection with application-optimized contrasts using different flip angle evolution;
SWI=
susceptibility-weighted imaging;
VWI=
vessel wall imaging;
FSR=
Foundation of Sarcoidosis Research

Footnotes

  • Go to Neurology.org/NN for full disclosures. Funding information is provided at the end of the article.

  • The Article Processing Charge was funded by the authors.

  • Received March 9, 2021.
  • Accepted in final form June 28, 2021.
  • Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

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Neurology - Neuroimmunology Neuroinflammation: 10 (6)

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