Park Brian J, Ray Emanuel, Bathla Girish, Bruch Leslie A, Streit Judy A, Cho Tracey A, Hitchon Patrick W
Department of Neurosurgery, University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA.
Department of Radiology, University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA.
World Neurosurg. 2021 Dec;156:e398-e407. doi: 10.1016/j.wneu.2021.09.089. Epub 2021 Sep 25.
Isolated spinal cord neurosarcoidosis is extremely rare. The potential implications of long-term immunosuppressant therapy make correct diagnosis imperative. However, there are challenges inherent in isolated spinal cord involvement that require a multidisciplinary approach. Here we present the largest series of definite and possible isolated spinal neurosarcoidosis and discuss our institutional experience in managing this rare but morbid condition.
A retrospective review was performed to identify all neurosarcoidosis cases starting from 2002 to 2020 at our institution. Patients were screened for cases of isolated spinal neurosarcoidosis. A descriptive analysis was performed for each case.
A total of 64 cases of neurosarcoidosis were identified. The spine was involved in 26 (40.6%) patients. Only 4 (6.3%) cases had isolated spinal cord involvement. A full medical and imaging workup was performed in determining isolated spinal cord involvement. Three patients subsequently underwent surgical biopsy, and 1 did not undergo biopsy because of patient preference. One of the patients who underwent biopsy had an initial nondiagnostic biopsy and had a repeat biopsy. Corticosteroids were employed in all cases with additional immunosuppressive agents for maintenance therapy and refractory cases. All showed radiographic improvement and were clinically stable to improved.
Isolated spinal cord involvement of neurosarcoidosis is rare and can present challenges in diagnosis. A biopsy can be performed when necessary. However, a biopsy of the spinal cord carries inherent risks and may not always be possible or result in a nondiagnostic sample. In the setting of high clinical suspicion, maximal medical therapy is still employed.
孤立性脊髓结节病极为罕见。长期免疫抑制治疗的潜在影响使得正确诊断至关重要。然而,孤立性脊髓受累存在一些固有挑战,需要多学科方法来应对。在此,我们报告了最大系列的明确和可能的孤立性脊髓结节病病例,并讨论我们机构在管理这种罕见但严重疾病方面的经验。
进行回顾性研究,以确定2002年至2020年在我们机构的所有结节病病例。对患者进行孤立性脊髓结节病病例筛查。对每个病例进行描述性分析。
共确定64例结节病病例。26例(40.6%)患者的脊柱受累。仅4例(6.3%)病例有孤立性脊髓受累。在确定孤立性脊髓受累时进行了全面的医学和影像学检查。3例患者随后接受了手术活检,1例因患者意愿未接受活检。其中1例接受活检的患者最初活检未明确诊断,随后进行了重复活检。所有病例均使用了皮质类固醇,并加用免疫抑制剂进行维持治疗和治疗难治性病例。所有患者影像学均有改善,临床状态稳定或改善。
结节病孤立性脊髓受累罕见,诊断可能存在挑战。必要时可进行活检。然而,脊髓活检存在固有风险,可能并不总是可行,或导致无法明确诊断的样本。在高度临床怀疑的情况下,仍采用最大程度的药物治疗。