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视神经脊髓炎谱系疾病伴水通道蛋白 4 免疫球蛋白 G 和髓鞘少突胶质细胞糖蛋白免疫球蛋白 G 相关自身免疫中的脊髓病变和萎缩。

Spinal cord lesions and atrophy in NMOSD with AQP4-IgG and MOG-IgG associated autoimmunity.

机构信息

NeuroCure Clinical Research Center, Charité - Universitätsmedizin Berlin, Berlin, Germany.

NeuroCure Clinical Research Center, Charité - Universitätsmedizin Berlin, Berlin, Germany/Department of Neuroradiology, Charité - Universitätsmedizin Berlin, Berlin, Germany.

出版信息

Mult Scler. 2019 Dec;25(14):1926-1936. doi: 10.1177/1352458518815596. Epub 2018 Nov 26.

DOI:10.1177/1352458518815596
PMID:30475082
Abstract

BACKGROUND

Spinal cord (SC) affection is a hallmark symptom of neuromyelitis optica spectrum disorders (NMOSD). Patients with aquaporin-4 (AQP4-IgG+) or myelin oligodendrocyte glycoprotein (MOG-IgG+) antibody seropositivity show this overlapping clinical phenotype.

OBJECTIVE

Quantitative comparison of SC lesions and atrophy in AQP4-IgG+ and MOG-IgG+ NMOSD.

METHODS

AQP4-IgG+ ( = 38), MOG-IgG+ ( = 15) NMOSD patients and healthy controls (HC,  = 24) were analysed for SC lesion (prevalence, length, location), atrophy as mean upper cervical cord area (MUCCA), Expanded Disability Status Scale (EDSS), timed 25-foot walk speed (T25FWS) and 9-hole peg test (9HPT) measures.

RESULTS

In total, 92% (35/38) of AQP4-IgG+ and 53% (8/15) of MOG-IgG+ patients had myelitis attacks (χ = 6.47,  = 0.011). 65.8%/26.7% of AQP4-/MOG-IgG+ patients had chronic SC lesions (χ = 5.16,  = 0.023), with similar proportions in cervical, upper thoracic and lower thoracic cord, and no length differences. MUCCA was decreased in AQP4-IgG+ ( = -2.27,  = 0.028), but not MOG-IgG+ patients ( = 0.58,  = 0.57) compared to HC. MUCCA associated with myelitis attacks (rho = -0.33,  = 0.016), EDSS (rho = -0.31,  = 0.030), pyramidal functional score (rho = -0.42,  = 0.003), T25FWS ( = 0.43,  = 0.010) and 9HPT Z-score ( = 0.32,  = 0.037), regardless of antibody status.

CONCLUSION

AQP4-IgG+ patients had more myelitis attacks, SC lesions and SC atrophy was more pronounced than in MOG-IgG+ patients. MUCCA is associated with clinical myelitis attacks and disability in all NMOSD patients.

摘要

背景

脊髓(SC)受累是视神经脊髓炎谱系疾病(NMOSD)的标志性症状。水通道蛋白 4(AQP4-IgG+)或髓鞘少突胶质细胞糖蛋白(MOG-IgG+)抗体阳性的患者表现出这种重叠的临床表型。

目的

定量比较 AQP4-IgG+和 MOG-IgG+NMOSD 患者的 SC 病变和萎缩。

方法

对 38 例 AQP4-IgG+(=38)、15 例 MOG-IgG+(=15)NMOSD 患者和 24 例健康对照者(HC,=24)进行 SC 病变(患病率、长度、部位)、萎缩(平均颈髓面积 MUCCA)、扩展残疾状况量表(EDSS)、25 英尺步行速度(T25FWS)和 9 孔钉测试(9HPT)测量分析。

结果

共有 92%(35/38)的 AQP4-IgG+和 53%(8/15)的 MOG-IgG+患者发生了脊髓炎发作(χ²=6.47,=0.011)。65.8%/26.7%的 AQP4-/MOG-IgG+患者有慢性 SC 病变(χ²=5.16,=0.023),在颈段、上胸段和下胸段的比例相似,长度无差异。与 HC 相比,AQP4-IgG+患者的 MUCCA 减少(=−2.27,=0.028),而 MOG-IgG+患者的 MUCCA 没有减少(=0.58,=0.57)。MUCCA 与脊髓炎发作(rho=−0.33,=0.016)、EDSS(rho=−0.31,=0.030)、锥体功能评分(rho=−0.42,=0.003)、T25FWS(=0.43,=0.010)和 9HPT Z 评分(=0.32,=0.037)相关,无论抗体状态如何。

结论

AQP4-IgG+患者的脊髓炎发作更多,SC 病变和 SC 萎缩更为明显。在所有 NMOSD 患者中,MUCCA 与临床脊髓炎发作和残疾有关。

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