Al-Hader Rami, Nofar Justin, Mohamedelkhair Ahmed, Affan Muhammad, Schultz Lonni R, Cerghet Mirela
Department of Neurology, Henry Ford Health, Detroit, MI 48202, USA.
Department of Neurology, University of Minnesota, Minneapolis, MN 55455, USA.
J Clin Med. 2024 Aug 27;13(17):5069. doi: 10.3390/jcm13175069.
To describe the clinical features and radiological outcomes of patients with spinal cord neurosarcoidosis, treatments, and long-term follow-up for this rare disorder. A cross-sectional, retrospective medical chart review was performed for all patients with spinal cord neurosarcoidosis treated at a single center between 01/1995 and 12/2020. Radiological imaging, laboratory test results, the type of immunosuppressive therapy, and function test scores were reviewed. We assessed 39 patients with spinal cord neurosarcoidosis (23 men, 16 women, mean age at presentation 46.4 years, SD 10.2 years). The mean (SD) duration of spinal cord neurosarcoidosis at data abstraction was 9.8 (6.3) years. There were 24 patients (62%) with extensive intramedullary lesions, 8 (21%) with multiple patchy intramedullary lesions, 12 (31%) with leptomeningeal involvement, and 7 (18%) with nerve root enhancement. The cervical spine was the most commonly affected region in 33 patients (85%). The most common presenting symptoms were paresthesia/neuropathic pain in 20 (51%) and weakness of extremities in 15 (38%) patients. Most patients ( = 37; 95%) had been treated with corticosteroids at symptom onset, and methotrexate was the most used immunosuppressive therapy ( = 19; 49%). Of 34 patients with follow-up magnetic resonance imaging (MRI) available, the median time to improvement per MRI was 10.8 months (95% CI, 6.1-17.0 months). Of 31 patients with MRI enhancement at presentation, 18 (58%) had complete enhancement resolution at follow-up, with a median time to resolution of 51.8 months (95% CI, 24.9-83.4 months). Patients had significantly lower pyramidal ( = 0.004) and sensory functional ( = 0.031) systems scores from presentation to the last clinic visit. Because spinal cord neurosarcoidosis is challenging to diagnose and no set treatment guidelines exist, clarifying patients' clinical parameters and responses to various treatments is needed to improve timely and efficient care. The incidence of spinal cord involvement in sarcoidosis in our cohort was higher than intracranial involvement and most patients had a long extensive intramedullary lesion. We also observed that most patients with spinal cord neurosarcoidosis improved clinically and radiologically after treatment; however, the resolution of MRI enhancement after immunosuppressive therapy may take years. Prospective studies of neurosarcoidosis will be crucial to address questions about effective treatment and long-term prognosis.
描述脊髓结节病患者的临床特征、放射学结果、治疗方法以及对这种罕见疾病的长期随访情况。对1995年1月至2020年12月在单一中心接受治疗的所有脊髓结节病患者进行了横断面回顾性病历审查。回顾了放射学影像、实验室检查结果、免疫抑制治疗类型和功能测试评分。我们评估了39例脊髓结节病患者(23例男性,16例女性,就诊时平均年龄46.4岁,标准差10.2岁)。数据提取时脊髓结节病的平均(标准差)病程为9.8(6.3)年。24例患者(62%)有广泛的髓内病变,8例(21%)有多发斑片状髓内病变,12例(31%)有软脑膜受累,7例(18%)有神经根强化。颈椎是最常受累的部位,有33例患者(85%)。最常见的首发症状是感觉异常/神经性疼痛,20例患者(51%)出现,肢体无力15例患者(38%)出现。大多数患者(n = 37;95%)在症状出现时接受了皮质类固醇治疗,甲氨蝶呤是最常用的免疫抑制治疗药物(n = 19;49%)。在有随访磁共振成像(MRI)的34例患者中,每次MRI改善的中位时间为10.8个月(95%可信区间,6.1 - 17.0个月)。在就诊时MRI有强化的31例患者中,18例(58%)在随访时强化完全消退,消退的中位时间为51.8个月(95%可信区间,24.9 - 83.4个月)。从就诊到最后一次门诊就诊,患者的锥体系统(P = 0.004)和感觉功能(P = 0.031)评分显著降低。由于脊髓结节病诊断具有挑战性且不存在既定的治疗指南,因此需要明确患者的临床参数以及对各种治疗的反应,以改善及时有效的护理。我们队列中结节病脊髓受累的发生率高于颅内受累,且大多数患者有广泛的长节段髓内病变。我们还观察到大多数脊髓结节病患者在治疗后临床和放射学上有所改善;然而,免疫抑制治疗后MRI强化的消退可能需要数年时间。对结节病进行前瞻性研究对于解决有效治疗和长期预后问题至关重要。