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脊髓结节病的临床特征及预后因素:一项对20例经活检证实患者的多中心观察性研究

Clinical features and prognostic factors of spinal cord sarcoidosis: a multicenter observational study of 20 BIOPSY-PROVEN patients.

作者信息

Durel Cécile-Audrey, Marignier Romain, Maucort-Boulch Delphine, Iwaz Jean, Berthoux Emilie, Ruivard Marc, André Marc, Le Guenno Guillaume, Pérard Laurent, Dufour Jean-François, Turcu Alin, Antoine Jean-Christophe, Camdessanche Jean-Philippe, Delboy Thierry, Sève Pascal

机构信息

Département de Médicine Interne, Hôpital de la Croix-Rousse, Hospices Civils de Lyon, 69004, Lyon, France.

Université de Lyon, Université Lyon 1, 69100, Villeurbanne, France.

出版信息

J Neurol. 2016 May;263(5):981-990. doi: 10.1007/s00415-016-8092-5. Epub 2016 Mar 23.

Abstract

Sarcoidosis of the spinal cord is a rare disease. The aims of this study are to describe the features of spinal cord sarcoidosis (SCS) and identify prognostic markers. We analyzed 20 patients over a 20-year period in 8 French hospitals. There were 12 men (60 %), mostly Caucasian (75 %). The median ages at diagnosis of sarcoidosis and myelitis were 34.5 and 37 years, respectively. SCS revealed sarcoidosis in 12 patients (60 %). Eleven patients presented with motor deficit (55 %) and 9 had sphincter dysfunction (45 %). The median initial Edmus Grading Scale (EGS) score was 2.5. The cerebrospinal fluid (CSF) showed elevated protein level (median: 1.00 g/L, interquartile range (IQR) 0.72-1.97), low glucose level (median 2.84 mmol/L, IQR 1.42-3.45), and elevated white cell count (median 22/mm(3), IQR 6-45). The cervical and thoracic cords were most often affected (90 %). All patients received steroids and an immunosuppressive drug was added in 10 cases (50 %). After a mean follow-up of 52.1 months (range 8-43), 18 patients had partial response (90 %), 7 displayed functional impairment (35 %), and the median final EGS score was 1. Six patients experienced relapse (30 %). There was an association between the initial and the final EGS scores (p = 0.006). High CSF protein level showed a trend toward an association with relapse (p = 0.076). The spinal cord lesion was often the presenting feature of sarcoidosis. Most patients experienced clinical improvement with corticosteroids and/or immunosuppressive treatment. The long-term functional prognosis was correlated with the initial severity.

摘要

脊髓结节病是一种罕见疾病。本研究的目的是描述脊髓结节病(SCS)的特征并确定预后标志物。我们在20年期间对法国8家医院的20例患者进行了分析。有12名男性(60%),大多为白种人(75%)。结节病和脊髓炎诊断时的中位年龄分别为34.5岁和37岁。12例患者(60%)的SCS显示有结节病。11例患者出现运动功能障碍(55%),9例有括约肌功能障碍(45%)。初始的埃德蒙斯分级量表(EGS)中位评分为2.5。脑脊液(CSF)显示蛋白水平升高(中位值:1.00g/L,四分位间距(IQR)0.72 - 1.97),葡萄糖水平降低(中位值2.84mmol/L,IQR 1.42 - 3.45),白细胞计数升高(中位值22/mm³,IQR 6 - 45)。颈段和胸段脊髓最常受累(90%)。所有患者均接受了类固醇治疗,10例(50%)加用了免疫抑制药物。平均随访52.1个月(范围8 - 43个月)后,18例患者有部分缓解(90%),7例有功能障碍(35%),最终EGS中位评分为1。6例患者复发(30%)。初始和最终EGS评分之间存在关联(p = 0.006)。脑脊液蛋白水平高显示出与复发相关的趋势(p = 0.076)。脊髓病变常为结节病的首发特征。大多数患者经皮质类固醇和/或免疫抑制治疗后临床症状改善。长期功能预后与初始严重程度相关。

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