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.
Neurol Neuroimmunol Neuroinflamm. 2021 Aug 4;8(6). doi: 10.1212/NXI.0000000000001063. Print 2021 Nov.
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).
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.
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.
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.
神经结节病（NS）中的脑血管表现以前被认为很少见，但现在越来越被认识到。我们报告了在接受高分辨率血管壁成像（VWI）的 NS 患者中的初步经验。
共对 13 例连续的 NS 患者进行了 VWI。由 2 位神经放射科医生进行共识分析。评估包括分段评估较大和中等大小的血管（颈内动脉、大脑中动脉 M1-M3 段；大脑前动脉 A1-A3 段；椎动脉 V4 段；基底动脉；和大脑后动脉 P1-P3 段）、纹状体穿通血管、脑髓质和深部脑静脉。由于与血流相关的伪影，皮质静脉未进行评估。6 例患者有脑活检发现，并对其进行了回顾。
患者平均年龄为 54.9 岁（33-71 岁），男女比例为 8:5。初始诊断和 VWI 研究之间的平均时间为 18 个月。总的来说，13 例中有 9 例（69%）有血管异常。13 例中有 3 例（23%）有环形大血管强化，6 例（46%）有穿通血管受累。6 例患者也有脑髓质和深部静脉受累。此外，13 例中有 7 例（54%）在磁敏感加权成像中有微出血，4 例（31%）有慢性梗死。在活检中，5 例中有 5 例显示血管周围肉芽肿，所有 5 例均有血管壁受累。
我们的初步发现表明，颅内血管结构的受累可能是 NS 患者的常见表现，应常规寻找。这些发现与先前报道的尸检文献一致，需要在更大范围内进行验证。
