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[脊髓受累揭示系统性结节病]

[Spinal cord involvement revealing systemic sarcoidosis].

作者信息

Cacoub P, Sbaï A, Hausfater P, Dormont D, Capelle L, Fohanno D, Piette J C

机构信息

Service de Médecine Interne, La Pitié-Salpêtrière, Paris.

出版信息

Rev Neurol (Paris). 2000 Oct;156(10):896-900.

Abstract

Spinal neurosarcoidosis is rare (0.43 p. 100 of all sarcoidosis) and can be the initial manifestation of the disease. A 43-year-old right handed African man developed a progressive dorsal neck pain, slowly worsened paresthesia weakness in the legs and a gait disturbance. Magnetic resonance imaging (MRI) scan of the cervical and thoracic spinal cord (sagittal T(1)- weighted image) revealed diffuse enlargement of the cord from C2 to T7 with intense intramedullary enhancement from C2 to T3 after administration of contrast material on sagittal T(1)- weighted image. Cranial MRI scan was normal. Radiographs of the chest revealed bilateral symmetric hilar mediastinal lymphadenopathy with no pulmonary infiltrates. Bronchial biopsy demonstrated non caseating granulomas with langerhans giant cells. The serum angiotensin converting enzyme level was elevated. The patient received corticosteroid with good progressive response. His neurologic symptoms improved markedly and twenty months post-treatment MRI showed no abnormality on enhanced T(1)- weighted images. Two years later he had no relapse. Only 6 to 10 p. 100 of patients with neurosarcoidosis have spinal cord involvement, which may account for the first clinical manifestation of the disease. Most patients with neurosarcoidosis have associated extraneurologic abnormalities. The diagnosis is supported usually by extraneurologic biopsies. Spinal cord biopsy needs to be considered on case-by-case basis.

摘要

脊髓结节病较为罕见(占所有结节病的0.43%),且可能是该疾病的首发表现。一名43岁的右利手非洲男性出现进行性颈背部疼痛,双腿感觉异常性无力逐渐加重,并伴有步态障碍。颈椎和胸椎脊髓的磁共振成像(MRI)扫描(矢状位T1加权像)显示,在矢状位T1加权像上注射造影剂后,脊髓从C2至T7弥漫性增粗,C2至T3髓内强化明显。头颅MRI扫描正常。胸部X线片显示双侧对称性肺门纵隔淋巴结肿大,无肺部浸润。支气管活检显示非干酪样肉芽肿伴朗汉斯巨细胞。血清血管紧张素转换酶水平升高。该患者接受了皮质类固醇治疗,病情呈良好的进展性缓解。其神经症状明显改善,治疗后20个月的MRI增强T1加权像显示无异常。两年后他未复发。仅有6%至10%的神经结节病患者有脊髓受累,这可能是该疾病的首发临床表现。大多数神经结节病患者伴有神经系统外的异常。诊断通常依靠神经系统外的活检来支持。脊髓活检需要根据具体情况考虑。

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