Development Of Neuromyelitis Optica Research Articles

Neuromyelitis optica spectrum disorder after presumed coronavirus (COVID-19) infection: A case report

Neuromyelitis optica spectrum disorder is an inflammatory autoimmune condition, predominantly affecting the optic nerves and spinal cord. It has been stated that viral infections play a role in the development of neuromyelitis optica. Several murine coronaviruses can cause inflammatory demyelinating diseases, including optic neuritis. Here we report, to the best of our knowledge, the first human case linking a presumed SARS-CoV-2 infection to the development of NMOSD.

Systemic lupus erythematosus with the development of neuromyelitis optica (Devic's syndrome) is a rare combination of autoimmune diseases

Neuromyelitis optica ((NMO), Devic's syndrome) is an immune-mediated inflammatory demyelinating disease characterized by transverse myelitis and optic neuritis. Determination of the level of antibodies to aquaporin 4 (NMO-IgG) is presently one of the key methods for the diagnosis and assessment of the activity of ONM, which allows this disease to be differentiated from multiple sclerosis and other demyelinating CNS lesions. ONM can occur not only as an independent disease, but also as a syndrome in different systemic diseases, such as: systemic lupus erythematosus (SLE), antineutrophilic cytoplasmic antibody-associated vasculitides, Sjögren's disease, etc. (up to 50–70%). In such situations, the clinician is always confronted with a question as whether the patient can have two rare autoimmune diseases or develop ONM as a systemic manifestation of rheumatic disease.The paper describes a clinical case of a young female patient with SLE concurrent with a CNS lesion, the manifestations of which corresponded to ONM. The patient had focal changes in the substance of the brain and spinal cord, as evidenced by magnetic resonance imaging, as well as high NMO-IgG titers. The development of ONM worsens SLE prognosis and requires active immunosuppressive therapy. The patient received three plasmapheresis sessions, ultrahigh-dose glucocorticoid and cyclophosphamide therapy, followed by replacement with azathioprine, causing a stable clinical and laboratory disease remission to be achieved.

Differentiating Neuromyelitis Optica-Related and Multiple Sclerosis-Related Acute Optic Neuritis Using Conventional Magnetic Resonance Imaging Combined With Readout-Segmented Echo-Planar Diffusion-Weighted Imaging.

In clinical practice, acute optic neuritis (ON) associated with the development of neuromyelitis optica (NMO) after the first attack is often indistinguishable from that associated with multiple sclerosis (MS). We aimed to determine the optimal combination of features derived from conventional magnetic resonance imaging (MRI) and diffusion-weighted imaging using readout-segmented echo-planar imaging (RESOLVE-DWI) for the differentiation of these conditions. Orbital conventional MRI and RESOLVE-DWI were performed using a 3.0-T scanner on 54 patients with acute ON (26 NMO-related and 28 MS-related). The features detected by conventional MRI (including laterality, the enhancement pattern, and the extent and position of involvement) and the apparent diffusion coefficient (ADC) measurements were retrospectively compared between the NMO-related and MS-related groups. A multivariate logistic regression analysis was used to identify the most significant variables, and receiver operating characteristic curve analyses were performed to determine the ability of a combined diagnostic model based on the qualitative and quantitative characteristics identified in this study to differentiate the 2 conditions. The multivariate logistic regression analyses indicated that the presence of chiasm involvement and lower ADC values were significantly associated with NMO-related acute ON compared with MS-related acute ON (P = 0.037 and 0.008, respectively). The diagnostic criterion of chiasm involvement or "ADC < 791 × 10 mm/s and chiasm involvement" had the highest specificity (96.9%), and "ADC < 791 × 10 mm/s or chiasm involvement" showed the optimal sensitivity (77.8%) for differentiating NMO-related from MS-related acute ON. Conventional MRI RESOLVE-DWI is helpful for differentiating NMO-related acute ON from MS-related acute ON. The combination of the ADC value chiasm involvement appears to be effective for discriminating these 2 types of acute ON.

Protective environmental factors for neuromyelitis optica.

To determine whether early environmental factors, such as cesarean delivery, breastfeeding, and exposure to smoking or herpes viruses, are associated with neuromyelitis optica (NMO) risk in children. This is a case-control study of pediatric NMO, multiple sclerosis (MS), and healthy subjects. Early-life exposures were obtained by standardized questionnaire. Epstein-Barr virus, cytomegalovirus, and herpes simplex virus 1 antibody responses were determined by ELISA. Multivariate logistic regression models were used to adjust for age at sampling, sex, race, and ethnicity. Early-life exposures were obtained from 36 pediatric subjects with NMO, 491 with MS, and 224 healthy controls. Daycare (odds ratio [OR] 0.33, 95% confidence interval [CI] 0.14, 0.78; p < 0.01) and breastfeeding (OR 0.42, 95% CI 0.18, 0.99; p = 0.05) were associated with lower odds of having NMO compared with healthy subjects. Cesarean delivery tended to be associated with 2-fold-higher odds of NMO compared with having MS/clinically isolated syndrome (OR 1.98, 95% CI 0.88, 4.59; p = 0.12) or with being healthy (OR 1.95, 95% CI 0.81, 4.71; p = 0.14). Sera and DNA were available for 31 subjects with NMO, 189 with MS, and 94 healthy controls. Epstein-Barr virus, herpes simplex virus 1, cytomegalovirus exposure, and being HLA-DRB1*15 positive were not associated with odds of having NMO compared with healthy subjects. Exposure to other young children may be an early protective factor against the development of NMO, as previously reported for MS, consistent with the hypothesis that infections contribute to disease risk modification. Unlike MS, pediatric NMO does not appear to be associated with exposures to common herpes viruses.

A serological analysis of viral and bacterial infections associated with neuromyelitis optica

To evaluate the role of infections in the development of neuromyelitis optica (NMO), 19 patients positive for anti-aquaporin-4 antibody were screened for 24 viral and bacterial infections. Serological evidence of recent viral infection was found in 7 of 15 patients screened during the acute phase of the neurologic illness, which was a significantly more frequent rate of infection than seen in the control group of 33 patients with neurodegenerative, metabolic, or vertebral diseases (47% versus 15%). Mumps virus and human herpes viruses were the frequent causal agents, although there was no statistical difference in frequency between the two groups. Most patients with identified recent infection had monophasic or recurrent myelitis without evidence of optic nerve involvement and small number of total clinical relapses. Disease history tended to be shorter in patients with identified recent infection than those without, and an expanded long spinal cord lesion in magnetic resonance imaging was rarely found in patients with identified recent infection, although statistical significance could not be shown. These findings indicate that, not single, but various viral infections, can be associated with the development of NMO during the early stages of the illness, although the exact pathogenesis of NMO has yet to be clarified.

NMO-IgG predicts the outcome of recurrent optic neuritis

To determine the prognostic value of neuromyelitis optica (NMO)-immunoglobulin G (IgG) in patients with recurrent optic neuritis (ON). The aquaporin-4-specific serum autoantibody, NMO-IgG, is a biomarker for NMO and relapsing transverse myelitis. Recurrent ON may herald multiple sclerosis (MS) or NMO, or it may occur as an isolated syndrome. The prognosis and response to therapy differs in each of these contexts. We evaluated 34 patients who were tested for NMO-IgG between 2000 and 2007 and who had two or more episodes of ON without satisfying a diagnosis of MS or NMO prior to serologic testing. Clinical data were available for 25 Mayo Clinic patients (5 NMO-IgG positive and 20 NMO-IgG negative) and for an additional 9 seropositive patients whose serum was referred to the Mayo Clinic Neuroimmunology laboratory for testing. Twenty percent of the patients with recurrent ON seen at Mayo Clinic were seropositive. All NMO-IgG-positive patients (vs 65% NMO-IgG-negative patients) had at least one attack with visual acuity in the affected eye worse than 20/200 (p = 0.05). In seropositive patients for whom long-term follow-up was possible (median 8.9 years after the initial ON), 6 of 12 (50%) experienced an episode of myelitis and fulfilled criteria for NMO. In contrast, 1 of 15 seronegative patients (6.7%) fulfilled McDonald criteria for MS (p = 0.03). Seropositive patients had a final visual score which was worse than that of seronegative patients (p = 0.02). Neuromyelitis optica (NMO)-immunoglobulin G seropositivity predicts poor visual outcome and development of NMO. Seropositive recurrent optic neuritis is a limited form of NMO.