PDF(Pubmed)
Abstract：
Sarcoidosis is a multisystemic inflammatory granulomatosis disease that rarely involves the central nervous system (CNS) and is even more so rarely isolated to the intramedullary thoracic spine. In isolated CNS sarcoidosis cases, surgical treatment is debated. We present here a case report and literature review on intramedullary thoracic spine sarcoidosis to evaluate potential portents of spine involvement and indications for surgical intervention. A 47-year-old female with a prior history of renal cell carcinoma presented with a week-long history of urinary retention and bilateral lower extremity numbness, and a 24-hour history of left lower extremity (LLE) weakness with saddle anesthesia. Magnetic resonance imaging demonstrated a syrinx spanning the spinal cord to the conus medullaris and a contrast-enhancing, expansile intramedullary thoracic lesion at T6-T7 with a non-enhancing, cystic right paraspinal lesion at T5. Given the patient\'s history of a kidney neoplasm, a metastatic work-up was completed. Biopsy of the T5 lesion was consistent with endometriosis. The patient underwent a T6-8 laminectomy with excisional biopsy and gross total resection of the intramedullary mass. Initial pathology was notable for lymphohistiocytic infiltrate with coagulative necrosis and rare multinucleated giant cells. At the one-month follow-up, the patient had improving LLE weakness and continued impairment of gait, balance, and coordination, but her symptoms of urinary retention, paresthesia, and numbness were resolved. Final pathology supported a diagnosis of sarcoidosis. At the three-month follow-up, the patient reported intermittent surgical site pain, but no other symptoms. She is followed up by her primary care consultant for symptom management and recurrence monitoring. Apart from the presented case, only one case of isolated intramedullary thoracic spine sarcoidosis was identified in the literature. The only case, of both review and presented, without significant symptom improvement did not undergo surgery. The available literature is limited; however, early surgical intervention may be indicated in isolated thoracic spine sarcoidosis.
摘要：
结节病是一种多系统的炎性肉芽肿病，很少涉及中枢神经系统（CNS），甚至很少隔离到髓内胸椎。在孤立的中枢神经系统结节病病例中，手术治疗有争议。我们在此提供有关髓内胸椎结节病的病例报告和文献综述，以评估脊柱受累的潜在预兆和手术干预的适应症。一名47岁女性，既往有肾细胞癌病史，有一周的尿潴留和双侧下肢麻木病史，和24小时的左下肢（LLE）无力与鞍式麻醉的历史。磁共振成像显示，从脊髓到延髓圆锥的syrinx和对比增强，T6-T7时的扩张性胸膜髓内病变，无增强，T5时右椎旁囊性病变。鉴于患者的肾脏肿瘤病史，完成了转移检查。T5病变的活检与子宫内膜异位症一致。该患者接受了T6-8椎板切除术，并进行了活检和髓内肿块的总切除。最初病理为淋巴组织细胞浸润伴凝固性坏死和罕见多核巨细胞。在为期一个月的随访中，患者LLE无力改善，步态持续受损，balance,协调,但她的尿潴留症状,感觉异常,麻木解决了。最终病理支持结节病的诊断。在三个月的随访中，患者报告间歇性手术部位疼痛，但没有其他症状.她由她的初级保健顾问进行症状管理和复发监测。除了提出的案件，文献中仅发现1例孤立性髓内胸椎结节病。唯一的案例,无论是审查还是提交，症状无明显改善,未接受手术治疗.现有文献有限；然而，早期手术干预可能适用于孤立性胸椎结节病。
