視神経脊髄炎

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a rare autoimmune demyelinating disorder of the central nervous system that can present with varied neurological symptoms. While commonly mistaken for multiple sclerosis or neuromyelitis optica spectrum disorders, MOGAD can also mimic cerebrovascular events, posing a diagnostic challenge. We report a case of a 30-year-old male who initially presented with acute-onset left-sided hemiplegia and right facial palsy, raising suspicion of an ischemic stroke. Despite thrombolysis, his recurrent neurological symptoms, including dysarthria and cerebellar ataxia, prompted further evaluation. Neuroimaging revealed hyperintense lesions in the right hemi-pons and bilateral middle cerebellar peduncles, raising the possibility of a demyelinating disorder. MOG-IgG seropositivity confirmed the diagnosis of MOG encephalitis. The patient responded well to pulse corticosteroid therapy, followed by maintenance immunosuppression with mycophenolate mofetil, and remained asymptomatic on follow-up. This case underscores the importance of considering autoimmune demyelinating disorders in young patients with recurrent neurological deficits and clinico-radiological dissociation. Early recognition and appropriate immunotherapy can prevent unnecessary thrombolysis and improve patient outcomes. Clinicians should maintain a high index of suspicion for MOGAD as a potential stroke mimic in atypical presentations.

BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is a relapsing autoimmune disease in which disability is largely relapse-driven. The effect of relapse burden on post-relapse recovery remains unclear. METHODS: This retrospective cohort study included adult patients with NMOSD who experienced clinical relapse at a tertiary care center in Northern Thailand between 2015 and 2024. Relapse episodes were stratified by prior relapse burden into no prior relapse, low relapse burden (1-2 prior relapses), and high relapse burden (>2 prior relapses). Outcomes included Expanded Disability Status Scale (EDSS) at relapse nadir and at 90 and 180 days. Unfavorable outcome was defined as EDSS ≥6. Recovery was assessed using the Recovery Index (RI). RESULTS: A total of 148 patients contributed 493 relapse episodes. Higher relapse burden was associated with progressively greater proportions of unfavorable outcomes at 90 and 180 days (P<0.001). At 90 days, unfavorable outcomes occurred in 4.7%, 12.0%, and 34.9% of no-prior-relapse, low-relapse-burden, and high-relapse-burden episodes, respectively. At 180 days, corresponding proportions were 2.0%, 8.3%, and 27.8%. EDSS at nadir and follow-up increased stepwise with higher relapse burden, whereas RI was progressively lower at both time points (all P<0.001). Each additional prior relapse and delayed intravenous methylprednisolone initiation >14 days after relapse onset were independently associated with unfavorable outcomes at both 90 and 180 days. CONCLUSION: Higher prior relapse burden was associated with more severe attacks, greater post-relapse disability, and less complete recovery. Relapse prevention, early attack recognition, and timely acute treatment may reduce disability accumulation in NMOSD.

BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease characterized by optic neuritis and myelitis, often misdiagnosed as multiple sclerosis (MS). Optical coherence tomography (OCT) provides noninvasive quantification of retinal layer damage, offering potential for NMOSD diagnosis and differentiation from MS. METHOD: Fifty-five articles were included in this meta-analysis. All patients underwent OCT examination, with at least one measurement recorded for retinal assessment. This review is reported according to the Meta-analysis of Observational Studies in Epidemiology guidelines and assessed the risk of bias of the included studies. Subgroup analysis assessed the clinical utility of OCT biomarkers across predefined subgroups. Sensitivity analysis was used to evaluate stability of results. Begg's and Egger's test and funnel plot were applied to assess publication bias. RESULTS: The results showed that the thickness of peripapillary retinal nerve fiber layer (pRNFL) (WMD = -17.73, p < 0.001), macular RNFL (WMD = -5.35, p < 0.001), superior RNFL (RNFL-S) (WMD = -32.91, p < 0.001), inferior RNFL (RNFL-I) (WMD = -39.21, p < 0.001), temporal RNFL (RNFL-T) (WMD = -17.74, p < 0.001), nasal RNFL (RNFL-N) (WMD = -13.59, p < 0.001), superotemporal RNFL (WMD = -25.60, p < 0.001), inferotemporal RNFL (WMD = -26.97, p = 0.001), superonasal RNFL (WMD = -19.84, p < 0.001), inferonasal RNFL (WMD = -23.31, p < 0.001), ganglion cell and inner plexiform layer (GCIPL) (WMD = -14.03, p < 0.001), ganglion cell layer (WMD = -9.34, p < 0.001), inner plexiform layer (WMD = -6.02, p < 0.001), outer nuclear layer (WMD = -2.03, p < 0.001), foveal (WMD = -12.95, p < 0.001) and GCIPL volume (WMD = -0.11, p < 0.001) in NMOSD were significantly thinner than those in the normal population, but no significant changes were found in the outer plexiform layer and photorreceptor layer. On the other hand, compared with MS, NMOSD had significantly thinner pRNFL (WMD = -11.68, p < 0.001), RNFL-S (WMD = -18.09, p = 0.017) /RNFL-I (WMD = -21.25, p = 0.008)/ RNFL-T (WMD = -6.47, p < 0.001)/RNFL-N (WMD = -2.38, p = 0.048) and GCIPL (WMD = -4.22, p < 0.001). Similarly, NMOSD patients had reduced macular volume compared with NC and MS (WMD = -0.32, p < 0.001), with no difference in inner nuclear layer thickness. CONCLUSION: OCT serves as a validated imaging biomarker for detecting NMOSD-specific intraretinal neurodegeneration, delineating pathological mechanisms and enhancing differential diagnosis, prognostication, and therapeutic monitoring.

BACKGROUND AND OBJECTIVES: Neuromyelitis optica spectrum disorder (NMOSD) is a severe condition usually associated with aquaporin-4 (AQP4) antibodies. A clinical presentation suggestive of NMOSD can also be associated with myelin oligodendrocyte glycoprotein (MOG) antibodies (MOGAD). NMOSD can be diagnosed in the absence of autoantibodies (double-negative NMOSD [DN-NMOSD]), but this subgroup has been poorly investigated. We conducted a systematic review and meta-analysis to define the clinical spectrum, prognosis, and treatment response in DN-NMOSD vs AQP4-NMOSD/MOGAD. METHODS: We searched on PubMed, Scopus, Embase, Google Scholar, Cochrane Library, and ClinicalTrials.gov databases of studies on patients fulfilling inclusion criteria. Patient characteristics, outcome measures, and treatment regimens were extracted. RESULTS: We included 41 of 1,027 articles screened and analyzed 671 patients with DN-NMOSD (median age 38.6 years [range IQR: 32.5-42.85]; female-to-male ratio 1.5:1; median follow-up 44.4 months [range 1-600]), 73.6% of which relapsed. In the meta-analysis, mean annualized relapse rate (ARR) was higher, albeit not significantly, in DN-NMOSD (1.08; 95% CI 0.73-1.43) vs AQP4-NMOSD (0.84; 95% CI 0.45-1.23) and MOGAD (0.61; 95% CI 0.39-0.83, p = 0.08). Administration of maintenance immunosuppression in DN-NMOSD led to a significant ARR reduction (pooled rate ratio 0.19, 95% CI 0.07-0.49; p = 0.001), with high heterogeneity (I2 = 90%, p < 0.0001). In meta-regression, no covariates were associated with ARR reduction, including the administration of specific drugs (rituximab, p = 0.288; azathioprine, p = 0.291; mycophenolate, p = 0.918). The pooled mean difference in pre‑ and post‑maintenance treatment Expanded Disability Status Scale values indicated a significant change in disability in MOGAD (-0.93, 95% CI -1.67 to -0.19, p = 0.02) but not in AQP4-NMOSD (-0.62, 95% CI -1.85 to 0.61, p = 0.27) or DN-NMOSD (-0.52 (95% CI -1.30 to 0.25, p = 0.16). DISCUSSION: DN-NMOSD is a heterogenous, severe and highly relapsing disease, where attacks lead to irreversible dysfunction. The administration of maintenance immunotherapy reduces the relapse risk and should be considered early to prevent further disability.

BACKGROUND: An elevated risk of cardiovascular disease (CVD) and cerebrovascular disease (CBD) has been observed in patients with ICD-defined Neuromyelitis Optica (hereinafter referred to as NMO). However, population-based research on this topic remains limited. In this study, the risk of macrovascular events was compared between individuals with NMO and a matched population without NMO in Taiwan. METHODS: Data for this retrospective cohort study were collected from a nationwide database in Taiwan. A total of 1,376 patients with new-onset NMO between 2003 to 2020 were enrolled. A Cox proportional hazards model was constructed to investigate CVD and CBD risk in patients with NMO and controlled for relevant variables. RESULTS: After relevant variables were controlled for, patients with NMO exhibited a significantly higher risk of CVD (adjusted hazard ratio [aHR] = 1.40; 95% confidence interval [CI] = 1.11-1.77) and CBD (aHR = 3.37; 95% CI = 2.69-4.22) than matched controls. Significant associations were observed between NMO and ischemic stroke, hemorrhagic stroke, and transient ischemic attack but not acute myocardial infarction, atrial fibrillation, coronary artery disease, or heart failure. CONCLUSION: Individuals with NMO exhibited an elevated risk of CBD. Conversely, NMO was not associated with an elevated risk of certain CVDs.