Wingerchuk Dean M
Continuum (Minneap Minn). 2018 Apr;24(2, Spinal Cord Disorders):497-522. doi: 10.1212/CON.0000000000000582.
This article reviews the clinical presentation, diagnostic evaluation, and management of immune-mediated myelopathies.
The discovery of several neural autoantibodies and their antigenic targets has revolutionized the investigation and treatment of immune-mediated myelopathies. Detection of these serologic biomarkers can support or establish a diagnosis of an autoimmune myelopathy, and, in the case of paraneoplastic syndromes, indicate the likely presence of an underlying malignancy. Distinctive lesion patterns detected on spinal cord or brain MRI narrow the differential diagnosis in patients with acute or subacute inflammatory myelopathies, including those not associated with autoantibody markers.
Immune-mediated myelopathies usually present acutely or subacutely and have a broad differential diagnosis. A systematic diagnostic approach using data from the clinical setting and presentation, MRI lesion patterns, CSF data, and autoantibody markers can differentiate these disorders from noninflammatory myelopathies, often with precise disease classification. This, in turn, provides prognostic information, especially whether the disorder is likely to relapse, and facilitates therapeutic decision making. Diagnostic accuracy informs selection of acute immunotherapy aimed at arresting and reversing recent neurologic injury and, when necessary, selection of long-term treatment for prevention of disease progression or relapse.
本文综述免疫介导性脊髓病的临床表现、诊断评估及治疗。
多种神经自身抗体及其抗原靶点的发现彻底改变了免疫介导性脊髓病的研究与治疗。检测这些血清生物标志物可辅助或确诊自身免疫性脊髓病，对于副肿瘤综合征患者，还可提示潜在恶性肿瘤的可能存在。脊髓或脑部MRI检测到的独特病变模式可缩小急性或亚急性炎症性脊髓病患者的鉴别诊断范围，包括那些与自身抗体标志物无关的患者。
免疫介导性脊髓病通常急性或亚急性起病，鉴别诊断范围广泛。采用来自临床情况及表现、MRI病变模式、脑脊液数据和自身抗体标志物的数据进行系统的诊断方法，可将这些疾病与非炎症性脊髓病区分开来，且常能进行精确的疾病分类。这进而提供预后信息，尤其是疾病是否可能复发，并有助于治疗决策。诊断准确性有助于选择旨在阻止和逆转近期神经损伤的急性免疫疗法，必要时还可选择预防疾病进展或复发的长期治疗方法。
