Revised diagnostic criteria for neuromyelitis optica

Background: The authors previously proposed diagnostic criteria for neuromyelitis optica (NMO) that facilitate its distinction from prototypic multiple sclerosis ( MS). However, some patients with otherwise typical NMO have additional symptoms not attributable to optic nerve or spinal cord inflammation or have MS-like brain MRI lesions. Furthermore, some patients are misclassified as NMO by the authors' earlier proposed criteria despite having a subsequent course indistinguishable from prototypic MS. A serum autoantibody marker, NMO-IgG, is highly specific for NMO. The authors propose revised NMO diagnostic criteria that incorporate NMO-IgG status. Methods: Using final clinical diagnosis (NMO or MS) as the reference standard, the authors calculated sensitivity and specificity for each criterion and various combinations using a sample of 96 patients with NMO and 33 with MS. The authors used likelihood ratios and logistic regression analysis to develop the most practical and informative diagnostic model. Results: Fourteen patients with NMO (14.6%) had extra-optic-spinal CNS symptoms. NMO-IgG seropositivity was 76% sensitive and 94% specific for NMO. The best diagnostic combination was 99% sensitive and 90% specific for NMO and consisted of at least two of three elements: longitudinally extensive cord lesion, onset brain MRI nondiagnostic for MS, or NMO-IgG seropositivity. Conclusions: The authors propose revised diagnostic criteria for definite neuromyelitis optica ( NMO) that require optic neuritis, myelitis, and at least two of three supportive criteria: MRI evidence of a contiguous spinal cord lesion 3 or more segments in length, onset brain MRI nondiagnostic for multiple sclerosis, or NMO-IgG seropositivity. CNS involvement beyond the optic nerves and spinal cord is compatible with NMO.

背景：作者此前提出了视神经脊髓炎（NMO）的诊断标准，有助于将其与典型的多发性硬化症（MS）相区分。然而，一些原本具有典型NMO症状的患者还出现了并非由视神经或脊髓炎症导致的其他症状，或者存在类似MS的脑部磁共振成像（MRI）病变。此外，一些患者虽依据作者早期提出的标准被误诊为NMO，但随后的病程却与典型MS并无区别。一种血清自身抗体标志物——NMO-IgG，对NMO具有高度特异性。作者提出了纳入NMO-IgG状态的修订版NMO诊断标准。 方法：以最终临床诊断（NMO或MS）作为参考标准，作者对96例NMO患者和33例MS患者的样本，计算了每个标准及各种组合的敏感性和特异性。作者运用似然比和逻辑回归分析，构建出最实用且信息丰富的诊断模型。 结果：14例NMO患者（14.6%）出现了视神经脊髓以外的中枢神经系统（CNS）症状。NMO-IgG血清阳性对NMO诊断的敏感性为76%，特异性为94%。最佳诊断组合对NMO诊断的敏感性为99%，特异性为90%，该组合包含以下三个要素中的至少两个：纵向广泛的脊髓病变、发病时脑部MRI不能诊断为MS，或NMO-IgG血清阳性。 结论：作者提出了明确的视神经脊髓炎（NMO）修订诊断标准，要求具备视神经炎、脊髓炎，以及以下三个支持性标准中的至少两个：MRI显示脊髓连续病变长度达3个节段或以上、发病时脑部MRI不能诊断为多发性硬化症，或NMO-IgG血清阳性。中枢神经系统累及范围超出视神经和脊髓与NMO相符。