Revised diagnostic criteria for neuromyelitis optica

Abstract

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.

Neuromyelitis optica spectrum disorder in patients with connective tissue disease and myelitis

“…All patients with suspected SS (primary or overlap) were offered a minor salivary gland (lip) biopsy, as were patients with SSA antibody positivity. The diagnosis of NMO was based on revised clinical criteria (12) ( Figure 1). The designation NMOSD was applied to cases thought to be at high risk for progression to NMO, as described by Wingerchuk et al (13) (Figure 1).…”

Neuromyelitis optica in Portugal (NEMIPORT) – A multicentre study

“…It is more prevalent in females (9:1) [1]. In the past 10 years, different diagnostic criteria for NMO have been proposed, including those of Wingerchuk [1,3] and United States National Multiple Sclerosis Society -NMSS [4].…”

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

“…The diagnosis of NMO requires the appropriate constellation of clinical symptoms (optic neuritis and transverse myelitis) plus 2 of the following: MRI imaging demonstrating extensive longitudinal involvement of the spinal cord; MRI imaging of the brain not meeting diagnostic criteria for MS; identification of AQP4 antibodies in the serum or cerebrospinal fluid (CSF) [43]. The AQP4 antibodies are fairly specific for NMO but have limited sensitivity, and thus seronegative patients can be diagnosed with NMO [44].…”