RT Journal Article
SR Electronic
T1 Vascular Involvement in Neurosarcoidosis
JF Neurology - Neuroimmunology Neuroinflammation
JO Neurol Neuroimmunol Neuroinflamm
FD Lippincott Williams & Wilkins
SP e1063
DO 10.1212/NXI.0000000000001063
VO 8
IS 6
A1 Girish Bathla
A1 Lama Abdel-Wahed
A1 Amit Agarwal
A1 Tracey A. Cho
A1 Sarika Gupta
A1 Karra A. Jones
A1 Sarv Priya
A1 Neetu Soni
A1 Bruce A. Wasserman
YR 2021
UL http://nn.neurology.org/content/8/6/e1063.abstract
AB 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.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