Neutrophil to lymphocyte ratio may be a useful marker in distinguishing MOGAD and MS and platelet to lymphocyte ratio associated with MOGAD activity.

BACKGROUND AND OBJECTIVE: Clinical overlap is observed between multiple sclerosis (MS) and myelin oligodendrocyte glycoprotein immunoglobulin-G (MOG-IgG) associated disease (MOGAD) and the difficulty in distinguishing between the two diseases. Here, we measured and compared the readily available neutrophil to lymphocyte ratio (NLR), platelet to lymphocyte ratio (PLR), and monocyte to lymphocyte ratio (MLR) to determine whether these three biomarkers can help to distinguish MOGAD and MS at disease onset. The impact of these three biomarkers on MOGAD and MS relapse also needs to be explored. METHODS: In this retrospective analysis, we obtained clinical and paraclinical data from the first attacks of MOGAD (N = 31) and MS (N = 50). Electronic medical records were used to collect demographic data (gender, age at onset), clinical symptoms, EDSS at onset, and medical treatments. The primary outcome was relapse within one year of onset. Four hematological parameters were recorded, including neutrophil count, platelet count, lymphocyte count, and monocyte count. NLR, PLR, and MLR were calculated and compared between MOGAD, MS, and HC. Receiver operator curve (ROC) analysis was performed to assess the ability of NLR, PLR, and MLR to distinguish between MOGAD and MS, MOGAD and HC, respectively. A logistic regression analysis was performed to determine the impact of NLR/PLR/MLR on MOGAD/MS relapse within one year of onset. RESULTS: Compared to HC, NLR is significantly higher in MOGAD and MS (p<0.001, p = 0.04, respectively). The PLR and MLR are elevated in MOGAD compared to HC (p<0.001, p<0.001, respectively), and MLR in MS are also statistically higher than in HC (p = 0.023). It is worth noting that NLR and PLR were much higher in MOGAD compared to MS (p<0.001, p = 0.001, respectively), but a significant difference regarding MLR has not been found between MOGAD and MS. Based on ROC curve analyses, we found that using NLR, PLR, and MLR to discriminate between MOGAD and MS yielded a ROC-plot area under the curve (AUC) value of 0.794, 0.727, and 0.681, respectively. Meanwhile, the AUC of NLR, PLR, and MLR to discriminate between MOGAD and HC were 0.926, 0.772, and 0.786. Furthermore, the logistics analysis revealed a significant positive association between PLR and MOGAD relapse. CONCLUSION: NLR helps differentiate MOGAD and MS in disease onset, and higher PLR was related to MOGAD relapse.

Plasma Complement 3 and Complement 4 Are Promising Biomarkers for Distinguishing NMOSD From MOGAD and Are Associated With the Blood-Brain-Barrier Disruption in NMOSD.

Background and Objective: Neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody (MOG-IgG) associated disease (MOGAD) are autoimmune inflammatory demyelinating diseases of the central nervous system (CNS). As the clinical features of NMOSD are similar to MOGAD, diagnostic confusion exists between the two diseases. To better discriminate NMOSD from MOGAD, we investigated whether the plasma levels of complement 3 (C3) and complement 4 (C4) are different in NMOSD and MOGAD during the acute attacks of the diseases. We sought to determine whether C3 or C4 has an influence on the features of NMOSD. Methods: In this observational study, data from 73 aquaporin-4 antibodies (AQP4-IgG) positive NMOSD patients and 22 MOG-IgG positive MOGAD patients were collected retrospectively. Demographics, clinical characteristics, plasma parameters, and cerebrospinal fluid (CSF) findings will be analyzed for comparability between the two groups. Immunoglobulin-G (IgG) and albumin were measured in both plasma and CSF. Plasma levels of C3 and C4 were measured and compared between the NMOSD, MOGAD, and 42 healthy controls (HC). The correlations between plasma C3, C4, and NMOSD clinical parameters were analyzed. Results: The ages of onset were later in the AQP4-IgG positive NMOSD group and females predominated, which differed from the MOGAD group, whose ages were younger and with a slight male preponderance. The AQP4-IgG positive NMOSD patients presented with the clinical symptoms of optic neuritis (ON) and transverse myelitis (TM), whereas encephalitis symptoms were more prevalent in MOGAD patients. CSF analysis shows that slight but not significantly higher white cell count (WCC) and protein were observed in the MOGAD group than in the AQP4-IgG positive NMOSD group. The plasma levels of IgG in MOGAD patients are significantly lower (p = 0.027) than in NMOSD patients. On the contrary, the plasma levels of albumin in MOGAD were higher than in NMOSD, which reached statistical significance (p = 0.039). Both the plasma C3 and C4 levels in the NMOSD group were significantly lower than in MOGAD and HC. The receiver operating characteristic (ROC) curve of the prediction model comprises C3 and C4 to distinguish NMOSD from MOGAD [area under the curve (AUC): 0.731, 0.645], which are considered to have discriminatory values. The results of Spearman's analysis revealed that there was a significant positive correlation between the plasma C3 and the CSF WCC (r = 0.383, p = 0.040). There was an inverse correlation between plasma C4 and plasma IgG (r = -0.244, p = 0.038). Plasma C3 or C4 was significantly positively correlated with CSF albumin and Q-Alb, which is considered a measure of blood-brain barrier (BBB) disruption. Conclusion: During the acute phase of NMOSD and MOGAD, plasma C3 and C4 may become potential biomarkers for distinguishing the two diseases and reflecting the NMOSD BBB damage.

Association between clinical factors and orofacial dyskinesias in anti-N-methyl-D-aspartate receptor encephalitis.

BACKGROUND AND PURPOSE: We aimed to determine whether demographic information, clinical characteristics, laboratory tests, and imaging features are associated with orofacial dyskinesias (OFLD) in patients with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. METHODS: In this retrospective study, patients who were diagnosed with anti-NMDAR encephalitis were enrolled. All patients' factors, including demographic information, clinical characteristics, laboratory tests, and imaging features, were obtained at the time of hospitalization. The neurological function was assessed using the modified Rankin scale (mRS). Univariate and multivariate logistic regressions were used to examine the associations between clinical factors and OFLD. RESULTS: In total, 119 patients (median age: 28.0 [19.0-41.0] years; 67 females) were recruited. Of 119 patients, 44 (37.0%) had OFLD. OFLD was associated with increased mRS at admission, serum sodium, lumbar puncture pressure, female biologic sex, fever, psychiatric symptoms, seizures, impaired consciousness, autonomic dysfunction, and central hypoventilation in univariate logistic regression, respectively. Multivariate regression analysis revealed that female biologic sex (odds ratios [OR], 4.73; 95% confidence interval [CI], 1.27-17.64; p = .021), increased mRS at admission (OR, 2.09; 95% CI, 1.18-3.71; p = .011), psychiatric symptoms (OR, 7.27; 95% CI, 1.20-43.91; p = .031), and seizures (OR, 5.11; 95% CI, 1.22-21.43; p = .026) were associated with OFLD, after adjusting for confounding factors. CONCLUSIONS: Our analysis suggests that the following clinical factors are associated with OFLD: female biologic sex, increased mRS at admission, psychiatric symptoms, and seizures.

Clinical significance of anti-SSA/Ro antibody in Neuromyelitis optica spectrum disorders.

BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune inflammatory disease of the central nervous system (CNS), also described as CNS autoimmune astrocytopathy, due to the production of pathogenic antibodies against aquaporin-4 (AQP4) expressed on the foot of astrocytes. NMOSD coexists with autoimmune diseases and related autoantibodies [anti-Sjogren's syndrome A (anti-SSA)/Ro antibody, anti-Sjogren's syndrome B (anti-SSB)/La antibody, anti-nuclear (anti-ANA) antibodies, anti-double-stranded DNA (anti-dsDNA) antibody, anti-thyroglobulin antibody, and anti-thyroid peroxidase antibody]. OBJECTIVES: No precise conclusion has been drawn on the role of the anti-SSA/Ro antibody in NMOSD. Therefore, the aim of this work was to evaluate whether the anti-SSA/Ro antibody has an impact on the clinical manifestation or prognosis of NMOSD. METHODS: Data were retrospectively collected from 102 patients with NMOSD diagnosed by experienced neurologists. The study population was divided into two groups based on the serum anti-SSA/Ro antibody status: NMOSD with or without anti-SSA/Ro antibody. The clinical, neuroimaging and laboratory parameters were compared between the two groups, including the neurological symptoms, MRI results, frequency of systemic autoantibodies, Expanded Disability Status Scale (EDSS), and NMOSD relapse rate. The EDSS and relapse were applied as measures of the NMOSD patient prognostic value. Cox regression analysis was used to evaluate the prognostic impact of anti-SSA/Ro antibody on NMOSD. RESULTS: Among the 102 NMOSD patients, striking differences were observed in the positive rate of AQP4-IgG (89.2% vs. 72.3%, p = 0.046) between those patients with and without the anti-SSA/Ro antibody. In addition, NMOSD patients with anti-SSA/Ro antibody showed the presence of more frequent anti-ANA antibodies (p = 0.002), anti-SSB/La antibody (p < 0.001), anti-dsDNA antibody (p < 0.002), Sjogren's syndrome (SS, p < 0.001) and systemic lupus erythematosus (SLE, p = 0.045). Univariate and multivariate Cox regression analysis were performed to confirm that the anti-SSA/Ro antibody affected the EDSS score and the relapse of NMOSD patients. The analysis of the survival curve revealed that the EDSS score in the NMOSD patients positive for the anti-SSA/Ro antibody reached 4.0 (p = 0.035) and relapsed (p = 0.039) earlier than in the negative group. CONCLUSION: The anti-SSA/Ro antibody could be associated with disease activity and severe disability in NMOSD.

Clinical characteristic of myelin oligodendrocyte glycoprotein antibody associated cortical encephalitis in adults and outcomes following glucocorticoid therapy.

Objective: To describe the clinical and radiological features, as well as outcomes following glucocorticoid therapy and recurrence in adults suffering from cortical encephalitis associated with myelin oligodendrocyte glycoprotein (MOG) antibody. Methods: The clinical information of nine adult patients suffering from cortical encephalitis associated with MOG antibody admitted to the Affiliated Brain Hospital of Nanjing Medical University from 2020 to 2022 was systematically reviewed. The clinical symptoms, laboratory data, imaging results, outcomes following glucocorticoid therapy and recurrence were evaluated. Result: A total of 9 patients positive for MOG antibody and suffering from cortical encephalitis were included in our study (55.6% men, median age 29 years, 15-57 years). The most common clinical symptoms included headache (77.8%), fever (66.7%), and generalized seizures (55.6%). Some patients also experienced limb shaking (22.2%), leg numbness (22.2%), transient motor aphasia (11.1%), and vision loss (11.1%). The main features of cerebrospinal fluid () examination were increased intracranial pressure, pleocytosis, and elevated cerebrospinal fluid (CSF) protein. In addition, N-methyl-D-aspartate receptor (NMDAR) and MOG antibodies were found in the CSF of 3 patients, and NMDAR, MOG, and glial fibrillary acidic protein antibodies were found in the CSF of 1 patient. All patients were subjected to magnetic resonance imaging (MRI) and the images of eight of them showed T2 and/flair image hyperintense lesions, three showed meningeal or lesion enhancement and four showed white matter lesions, which were mostly located in the midline structures (75%). All patients received glucocorticoid therapy in the acute phase and in remission, and eight of them received an intravenous high dose of methylprednisolone, including one patient who received a simultaneous immunoglobulin therapy. One patient was treated with low-dose prednisolone tablets. Seven (77.8%) patients were wholly recovered at discharge, and 2 (22.2%) patients were left with slight symptoms. During the median 9-month follow-up (range: 2-36 months), 2 (22.2%) patients developed recurrence. Conclusion: The clinical manifestations of adult MOG antibody-associated cortical encephalitis were significantly different from those of the typical MOG antibody-associated disease (MOGAD). Patients in the acute phase of the disease were prone to show signs similar to central nervous system infection, requiring clinicians to have the ability to recognize the disease to avoid misdiagnosis. In addition, seizures were common in MOG antibody-related encephalitis, and the type of seizures was age-related. Brain MRI results showed that the distribution of cerebral cortex lesions was closely related to the classification of cortical encephalitis. Based on the patient's response to the treatment, glucocorticoid therapy was effective against MOG antibody-associated cortical encephalitis, which is consistent with the treatment response and clinical prognosis of MOGAD. Therefore, our opinion was that MOG antibody might be the "responsible antibody" in MOG antibody-associated cortical encephalitis, although further studies are needed to confirm this hypothesis.