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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