College of Medicine and Life Sciences, The University of Toledo, Toledo, OH, USA.
Department of Pathology, The University of Toledo, Toledo, OH, USA.
Spinal Cord Ser Cases. 2021 Sep 28;7(1):89. doi: 10.1038/s41394-021-00450-1.
Sarcoidosis is a multisystem disease characterized histologically by noncaseating granulomas. Localization of sarcoidosis to the CNS is termed neurosarcoidosis, a complex and rare neuroinflammatory form of sarcoidosis. When the spinal cord is involved, lesions are often intradural. Here, we present a rare case of progressive myelopathy secondary to multifocal spinal extradural neurosarcoidosis with spinal cord compression and without pulmonary involvement.
A 29-year-old African American female presented to the emergency department with numbness and paresthesia of 2-month duration in her left lower extremity and 2-week duration in her right lower extremity. The patient reported difficulty ambulating, paresthesia below the umbilicus, and back pain radiating to bilateral lower extremities. She endorsed 9-month history of cough, subjective fevers, night sweats, and unintentional 15 kg weight loss. Examination revealed 4/5 strength in the left lower extremity. MRI of the brain and spinal cord revealed enhancing extradural lesions, with spinal cord compression at T8 measuring 1.3 × 1.9 cm. Lumbar puncture demonstrated oligoclonal bands and increased CSF neutrophils, lymphocytes, monocytes, and protein. T8 laminectomy with resection of the epidural lesion was performed. Histology showed granulomas, consistent with neurosarcoidosis. At follow-up, repeat spinal MRI revealed disease progression with intramedullary involvement. Long-term immunosuppressive treatment was eventually initiated with satisfactory response.
This is a rare case of myelopathy secondary to spinal extradural neurosarcoidosis. Spinal neurosarcoidosis is predominantly an intradural process. Our review of the literature identified only seven cases of extradural neurosarcoidosis presenting with compressive myelopathy. Additional insight into management and rehabilitation following pathological diagnosis is of clinical significance.
结节病是一种多系统疾病，组织学上表现为非干酪样肉芽肿。中枢神经系统受累的结节病称为神经结节病，是一种复杂且罕见的神经炎症性结节病形式。当脊髓受累时，病变通常位于硬脊膜内。在此，我们报告一例罕见的多灶性硬脊膜外神经结节病继发进行性脊髓病，伴有脊髓压迫而无肺部受累。
一名 29 岁的非裔美国女性因左下肢麻木和感觉异常就诊于急诊科，持续时间为 2 个月，右下肢麻木和感觉异常持续时间为 2 周。患者报告行走困难、脐以下感觉异常和双侧下肢放射痛。她有 9 个月的咳嗽史、主观发热、盗汗和非自愿性体重减轻 15 公斤。检查发现左下肢肌力为 4/5。脑和脊髓 MRI 显示增强的硬脊膜外病变，T8 处脊髓受压，大小为 1.3×1.9cm。进行了 T8 椎板切除术和硬膜外病变切除术。腰椎穿刺显示寡克隆带和 CSF 中性粒细胞、淋巴细胞、单核细胞和蛋白增加。在随访时，重复脊髓 MRI 显示疾病进展伴有髓内受累。最终开始长期免疫抑制治疗，反应良好。
这是一例罕见的由硬脊膜外神经结节病引起的脊髓病。脊柱神经结节病主要是硬脊膜内病变。我们对文献的回顾仅发现了 7 例表现为压迫性脊髓病的硬脊膜外神经结节病病例。对病理诊断后管理和康复的进一步了解具有临床意义。
