Abstract：
BACKGROUND: Retrospective studies suggest that spinal movement disorders, especially tonic spasms, are prevalent in NMOSD. However, there have been no prospective studies evaluating spinal movement disorders in NMOSD, MOGAD, and idiopathic transverse myelitis (ITM).
METHODS: Patients referred to a tertiary neuroimmunology clinic for spinal cord demyelination (excluding MS) were evaluated. All patients answered a movement disorders survey and underwent a movement disorder-focused exam. Movement disorders were compared among patients with NMOSD with and without AQP4-IgG, MOGAD, and ITM. Patients with and without involuntary movements were also compared to identify predictors of spinal movement disorders.
RESULTS: Sixty-three patients were evaluated from 2017 to 2021 (71% females, median age 49 years, range 18-72 years, median disease duration 12 months, range 1-408). Of the total, 49% had ITM, 21% had NMOSD without AQP4-IgG, 19% had NMOSD with AQP4-IgG, and 11% had MOGAD. Movement disorders were present in 73% of the total patients and were most frequent in NMOSD with AQP4-IgG (92%) and least frequent in MOGAD (57%). The most frequent spinal movement disorders were tonic spasms (57%), focal dystonia (25%), spinal tremor (16%), spontaneous clonus (9.5%), secondary restless limb syndrome (9.5%), and spinal myoclonus (8%). Multivariate analysis showed that longitudinally extensive myelitis and AQP4-IgG are independent risk factors for the development of spinal movement disorders, while MOG-IgG and African American race were associated with a lower risk of developing these movement disorders.
CONCLUSIONS: Spinal movement disorders are highly prevalent in non-MS demyelinating disorders of the spinal cord. Prevalence rates exceed those reported in MS and retrospective NMOSD studies.
METHODS: Patients referred to a tertiary neuroimmunology clinic for spinal cord demyelination (excluding MS) were evaluated. All patients answered a movement disorders survey and underwent a movement disorder-focused exam. Movement disorders were compared among patients with NMOSD with and without AQP4-IgG, MOGAD, and ITM. Patients with and without involuntary movements were also compared to identify predictors of spinal movement disorders.
RESULTS: Sixty-three patients were evaluated from 2017 to 2021 (71% females, median age 49 years, range 18-72 years, median disease duration 12 months, range 1-408). Of the total, 49% had ITM, 21% had NMOSD without AQP4-IgG, 19% had NMOSD with AQP4-IgG, and 11% had MOGAD. Movement disorders were present in 73% of the total patients and were most frequent in NMOSD with AQP4-IgG (92%) and least frequent in MOGAD (57%). The most frequent spinal movement disorders were tonic spasms (57%), focal dystonia (25%), spinal tremor (16%), spontaneous clonus (9.5%), secondary restless limb syndrome (9.5%), and spinal myoclonus (8%). Multivariate analysis showed that longitudinally extensive myelitis and AQP4-IgG are independent risk factors for the development of spinal movement disorders, while MOG-IgG and African American race were associated with a lower risk of developing these movement disorders.
CONCLUSIONS: Spinal movement disorders are highly prevalent in non-MS demyelinating disorders of the spinal cord. Prevalence rates exceed those reported in MS and retrospective NMOSD studies.
摘要：
背景：回顾性研究表明，脊柱运动障碍，尤其是强直痉挛,在NMOSD中普遍存在。然而,没有前瞻性研究评估NMOSD的脊柱运动障碍，MOGAD,特发性横贯性脊髓炎（ITM）。
方法：评估了因脊髓脱髓鞘（不包括MS）转诊到三级神经免疫学诊所的患者。所有患者都回答了运动障碍调查，并接受了以运动障碍为重点的检查。运动障碍在有和没有AQP4-IgG的NMOSD患者中进行比较。MOGAD,和ITM。还比较了有和没有不自主运动的患者，以确定脊柱运动障碍的预测因素。
结果：从2017年到2021年对63例患者进行了评估（71％为女性，中位年龄49岁，范围18-72年，中位病程12个月，范围1-408）。在总数中，49%有ITM,21%的NMOSD患者无AQP4-IgG，19%患有AQP4-IgG的NMOSD，11%有MOGAD。运动障碍存在于73％的患者中，在AQP4-IgG的NMOSD中最常见（92％），在MOGAD中最不常见（57％）。最常见的脊柱运动障碍是强直痉挛（57％），局灶性肌张力障碍（25%），脊髓震颤（16%），自发性阴部(9.5%)，继发性不宁肢综合征（9.5%），和脊髓肌阵挛症（8％）。多因素分析显示纵向广泛性脊髓炎和AQP4-IgG是脊柱运动障碍发展的独立危险因素。而MOG-IgG和非裔美国人种族与发展这些运动障碍的风险较低相关。
结论：脊髓运动障碍在非MS脊髓脱髓鞘疾病中非常普遍。患病率超过MS和回顾性NMOSD研究报告的患病率。
方法：评估了因脊髓脱髓鞘（不包括MS）转诊到三级神经免疫学诊所的患者。所有患者都回答了运动障碍调查，并接受了以运动障碍为重点的检查。运动障碍在有和没有AQP4-IgG的NMOSD患者中进行比较。MOGAD,和ITM。还比较了有和没有不自主运动的患者，以确定脊柱运动障碍的预测因素。
结果：从2017年到2021年对63例患者进行了评估（71％为女性，中位年龄49岁，范围18-72年，中位病程12个月，范围1-408）。在总数中，49%有ITM,21%的NMOSD患者无AQP4-IgG，19%患有AQP4-IgG的NMOSD，11%有MOGAD。运动障碍存在于73％的患者中，在AQP4-IgG的NMOSD中最常见（92％），在MOGAD中最不常见（57％）。最常见的脊柱运动障碍是强直痉挛（57％），局灶性肌张力障碍（25%），脊髓震颤（16%），自发性阴部(9.5%)，继发性不宁肢综合征（9.5%），和脊髓肌阵挛症（8％）。多因素分析显示纵向广泛性脊髓炎和AQP4-IgG是脊柱运动障碍发展的独立危险因素。而MOG-IgG和非裔美国人种族与发展这些运动障碍的风险较低相关。
结论：脊髓运动障碍在非MS脊髓脱髓鞘疾病中非常普遍。患病率超过MS和回顾性NMOSD研究报告的患病率。
