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脱髓鞘疾病:横贯性脊髓炎的最新进展

Demyelinating disorders: update on transverse myelitis.

作者信息

Krishnan Chitra, Kaplin Adam I, Pardo Carlos A, Kerr Douglas A, Keswani Sanjay C

机构信息

Department of Neurology, Johns Hopkins University School of Medicine, Pathology 627, 600 North Wolfe Street, Baltimore, MD 21287-6965, USA.

出版信息

Curr Neurol Neurosci Rep. 2006 May;6(3):236-43. doi: 10.1007/s11910-006-0011-1.

Abstract

Transverse myelitis (TM) is a focal inflammatory disorder of the spinal cord. Perivascular monocytic and lymphocytic infiltration, demyelination, and axonal injury are prominent histopathogic features of TM. The clinical manifestations of TM are consequent to dysfunction of motor, sensory, and autonomic pathways. At peak deficit, 50% of patients with TM are completely paraplegic (with no volitional movements of legs), virtually all have some degree of bladder dysfunction, and 80% to 94% have numbness, paresthesias, or band-like dysesthesias. Longitudinal case series of TM reveal that approximately one third of patients recover with little to no sequelae, one third are left with a moderate degree of permanent disability, and one third have severe disability. Recent studies have shown that the cytokine interleukin-6 may be a useful biomarker, as the levels of interleukin-6 in the cerebrospinal fluid of acute TM patients strongly correlate with and are highly predictive of disability. Clinical trials testing the efficacy of promising axonoprotective agents in combination with intravenous steroids in the treatment of TM are currently underway.

摘要

横贯性脊髓炎(TM)是一种脊髓的局灶性炎症性疾病。血管周围单核细胞和淋巴细胞浸润、脱髓鞘以及轴突损伤是TM突出的组织病理学特征。TM的临床表现是运动、感觉和自主神经通路功能障碍的结果。在功能缺损高峰期,50%的TM患者完全截瘫(腿部无自主运动),几乎所有患者都有一定程度的膀胱功能障碍,80%至94%的患者有麻木、感觉异常或带状感觉障碍。TM的纵向病例系列显示,约三分之一的患者恢复后几乎没有后遗症,三分之一的患者留有中度永久性残疾,三分之一的患者有严重残疾。最近的研究表明,细胞因子白细胞介素-6可能是一种有用的生物标志物,因为急性TM患者脑脊液中白细胞介素-6的水平与残疾程度密切相关且具有高度预测性。目前正在进行临床试验,测试有前景的轴突保护剂与静脉注射类固醇联合治疗TM的疗效。

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