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脊髓结节病:7例报告

Spinal cord sarcoidosis: report of seven cases.

作者信息

Varron L, Broussolle C, Candessanche J-P, Marignier R, Rousset H, Ninet J, Sève P

机构信息

Hospices Civils de Lyon, Université de Lyon 1, Lyon, France.

出版信息

Eur J Neurol. 2009 Mar;16(3):289-96. doi: 10.1111/j.1468-1331.2008.02409.x.

Abstract

BACKGROUND

Spinal cord involvement in sarcoidosis is rare, occurring in <1% of patients with sarcoidosis.

METHODS

We report seven cases of spinal cord sarcoidosis, seen in two French hospitals over a 13-year period. Presentation of disease, methods of diagnosis and response to treatment, with quantification according to the reduction of the modified Rankin scale (MRS), were noted.

RESULTS

Six patients presented insidious paresthesias or weakness and one a sudden paraplegia. Average MRS at diagnosis was to 2. Spine MRI showed one or several intramedullary lesions in all cases. Diagnosis was confirmed by extra-neural tissue biopsies in all cases, including mediastinoscopy (two patients), coelioscopy (one patient), bronchoscopy (one patient), salivary gland biopsy (one patient) and skin biopsy (two patients). The average follow-up for the group was 49.4 months. All patients responded to corticosteroid therapy with a median reduction of MRS of one point. Five patients received immunosuppressive therapy: cyclophosphamide (two patients), methotrexate (two patients), azathioprine (one patient), mycophenolate mofetyl (one patient), with an inconstant benefit. Patients who received cyclophosphamide presented severe fungaemia.

CONCLUSION

Based on our study and literature analysis, we propose an algorithm for treatment of spinal cord sarcoidosis.

摘要

背景

结节病累及脊髓较为罕见,在结节病患者中的发生率低于1%。

方法

我们报告了在13年期间于两家法国医院诊治的7例脊髓结节病病例。记录了疾病表现、诊断方法及治疗反应,并根据改良Rankin量表(MRS)评分的降低情况进行量化。

结果

6例患者表现为隐匿性感觉异常或肌无力,1例突发截瘫。诊断时平均MRS评分为2分。所有病例的脊柱MRI均显示一个或多个脊髓内病变。所有病例均通过神经外组织活检确诊,包括纵隔镜检查(2例患者)、腹腔镜检查(1例患者)、支气管镜检查(1例患者)、唾液腺活检(1例患者)和皮肤活检(2例患者)。该组患者的平均随访时间为49.4个月。所有患者对皮质类固醇治疗均有反应,MRS评分中位数降低1分。5例患者接受了免疫抑制治疗:环磷酰胺(2例患者)、甲氨蝶呤(2例患者)、硫唑嘌呤(1例患者)、霉酚酸酯(1例患者),疗效不一。接受环磷酰胺治疗的患者出现了严重的真菌血症。

结论

基于我们的研究和文献分析,我们提出了一种脊髓结节病的治疗方案。

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