Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disorder of the central nervous system (CNS) mostly manifesting as optic neuritis and/or myelitis, which are frequently recurrent/bilateral or longitudinally extensive, respectively. As the autoantibody to aquaporin-4 (AQP4-Ab) can mediate the pathogenesis of NMOSD, testing for the AQP4-Ab in serum of patients can play a crucial role in diagnosing NMOSD. Nevertheless, the differential diagnosis of NMOSD in clinical practice is often challenging despite the phenotypical and serological characteristics of the disease because: (1) diverse diseases with autoimmune, vascular, infectious, or neoplastic etiologies can mimic these phenotypes of NMOSD; (2) patients with NMOSD may only have limited clinical manifestations, especially in their early disease stages; (3) test results for AQP4-Ab can be affected by several factors such as assay methods, serologic status, disease stages, or types of treatment; (4) some patients with NMOSD do not have AQP4-Ab; and (5) test results for the AQP4-Ab may not be readily available for the acute management of patients. Despite some similarity in their phenotypes, these NMOSD and NMOSD-mimics are distinct from each other in their pathogenesis, prognosis, and most importantly treatment. Understanding the detailed clinical, serological, radiological, and prognostic differences of these diseases will improve the proper management as well as diagnosis of patients.
BackgroundKey clinical features of chronic relapsing inflammatory optic neuropathy (CRION) include relapsing inflammatory optic neuritis (ON) and steroid dependency, both of which have been reported among patients with myelin oligodendrocyte glycoprotein antibodies (MOG-Abs). We investigated the relevance of the presence of serum MOG-IgG with the current diagnostic criteria for CRION among patients with idiopathic inflammatory optic neuritis (iON).MethodsRetrospective reviews of a database prospectively collated between 2011 and 2017 from the tertiary referral center for multiple sclerosis and neuromyelitis optica were performed. Sixty-four patients with iON, who did not meet the diagnostic criteria for multiple sclerosis, neuromyelitis optica (NMO) spectrum disorder with/without NMO-IgG, or acute disseminated encephalomyelitis and who had no symptomatic central nervous system (CNS) lesions other than on the optic nerve, were included from a cohort of 615 patients with inflammatory demyelinating diseases of the CNS. Fulfillment of the current diagnostic criteria for CRION, assay results for the serum IgG1 MOG-Ab, and characteristics of CRION patients with MOG-IgG were compared to those of non-CRION patients with MOG-IgG.ResultsTwelve iON patients fulfilled the current diagnostic criteria for CRION, 11 patients were positive for MOG-IgG, and one patient was borderline. Among the other 52 iON patients not meeting the criteria for CRION, 14 had relapsing disease courses and 38 had monophasic courses, of which MOG-IgG positivity were 0% and 29%, respectively. CRION patients with MOG-IgG had more relapsing disease courses (first steroid-dependent worsening/relapse in 2.3 months, range 0.4–7.0) and poorer optical coherence tomography outcomes at follow-up than non-CRION patients with MOG-IgG. However, patients in the two groups did not differ in terms of age of onset, sex, or steroid treatment duration after initial attack.ConclusionsCRION, according to the current diagnostic criteria, is a relapsing optic neuritis associated with MOG-IgG. Among iON patients with MOG-IgG, the absence of steroid-dependent attacks in the early stages of the disease may predict a long-term non-relapsing disease course and a more favorable outcome.Electronic supplementary materialThe online version of this article (10.1186/s12974-018-1335-x) contains supplementary material, which is available to authorized users.
There was a significant decrease in RNFL thickness over time in BRVO and significant thinning at 6 months compared with the normal eye. Retinal nerve fiber layer thinning needs to be differentiated from glaucoma or systemic disease, but it should be considered the natural course after BRVO.
Purpose: To evaluate the potential of the temporal raphe sign on the macular ganglion celleinner plexiform layer (mGCIPL) thickness map for discriminating glaucomatous from nonglaucomatous optic neuropathy (NGON) in eyes with mGCIPL thinning.Design: Cross-sectional study.Participants: A total of 175 eyes of 175 patients with mGCIPL thinning on Cirrus (Carl Zeiss Meditec, Dublin, CA) high-definition OCT were retrospectively included. Glaucoma specialists and neuro-ophthalmology specialists evaluated the patients' medical records for diagnosis of glaucomatous optic neuropathy (GON) or NGON. Finally, by consensus, 67 eyes with GON and 73 eyes with NGON were enrolled.Methods: A positive temporal raphe sign was declared in patients in whom there was a straight line longer than one-half of the length between the inner and outer annulus in the temporal elliptical area of the mGCIPL thickness map. Decision tree analysis was performed to formulate a diagnostic model.Main Outcome Measures: Area under receiver operating characteristic curve (AUC) with sensitivity and specificity.Results: The temporal raphe sign was observed in 61 of 67 GON eyes (91.0%), but in only 21 of 73 NGON eyes (28.8%) (P < 0.001; chi-square test). On this basis, the diagnostic ability of the temporal raphe sign for discriminating GON from NGON was judged to be good (AUC, 0.811; 95% confidence interval, 0.749e0.874; sensitivity, 91.0%; specificity, 71.2%). The diagnostic performance of the decision treeebased model (AUC 0.879; 95% confidence interval, 0.824e0.933; sensitivity, 88.1%; specificity, 87.7%) was better than that of the temporal raphe sign or the relative afferent pupillary defect (RAPD) alone (P ¼ 0.005, P < 0.001, respectively; DeLong's test). The decision tree model revealed the following: (1) If the temporal raphe sign is positive and the RAPD is absent, the case should be diagnosed as GON; (2) if the temporal raphe sign is absent regardless of the presence or absence of the RAPD, or both the temporal raphe sign and the RAPD are present, the case should be diagnosed as NGON.Conclusions: In clinical practice, determining whether the temporal raphe sign appears on OCT macular scans can be a useful tool for discrimination of glaucomatous from nonglaucomatous mGCIPL thinning.
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