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Background. Acute sensorineural hearing loss (SNHL) is a rare development in the central nervous system (CNS) demyelinating diseases such as aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Methods. We retrospectively reviewed consecutive patients with seropositive NMOSD or MOGAD in the CNS Inflammatory and Demyelinating Disease Registry at Samsung Medical Center from January 2015 to December 2020. After the medical chart review, the demographic data and the results of brain magnetic resonance imaging (MRI) and audiometry of patients with hearing loss were collected. Results. Five patients (NMOSD, n=3; MOGAD, n=2) were diagnosed with SNHL, two developed SNHL before the first core clinical symptom, and another two patients who underwent brain MRI at the timing of hearing loss showed lesions. Only three patients received high-dose steroids; however, hearing loss did not improve in any patients. Conclusion. SNHL was observed in a small number of patients with seropositive NMOSD and MOGAD; however, it could be underrecognized. Further large cohort prospective studies are helpful to elucidate the clinical implication of SNHL in NMOSD and MOGAD.

Fatigue is a prevalent symptom and major burden in neuroimmunological diseases. In neuromyelitis optica spectrum disorder (NMOSD), a severe autoimmune central nervous system (CNS) inflammatory disease with autoantibodies reactive to aquaporin-4, there are few reports about fatigue and quality of life (QOL). We aimed to evaluate the severity of fatigue and its relationship with QOL in patients with NMOSD. We prospectively studied patients with NMOSD who were in remission and seropositive for anti-aquaporin-4 antibody, and they were divided into 2 groups based on the presence of fatigue assessed using the Functional Assessment of Chronic Illness Therapy-fatigue score. Sleep quality, depression, pain, and QOL were also evaluated. A total of 35 patients were enrolled (mean age, 46.5 ± 14.1 years; female: male = 29:6), and the median Expanded Disability Status Scale (EDSS) score was 2.0 (range, 0 to 8.0). The patients with fatigue (N = 25, 71.4%) had poorer sleep quality and more severe depression than those without fatigue (p = 0.009 and p = 0.001). Both the physical and mental QOL scores were lower in patients with fatigue than in those without fatigue (p = 0.033 and p = 0.004). Multiple linear regression analyses showed that the degree of fatigue with EDSS score and pain were independent predictors of physical aspects of QOL (B = 0.382, p = 0.001), whereas depression was the only predictor of the mental components of QOL (B = -0.845, p = <0.001). Fatigue is a common symptom and an important predictor of QOL in patients with NMOSD.

Multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) are autoimmune inflammatory disorders of the central nervous system (CNS) with similar characteristics. The differential diagnosis between MS and NMOSD is critical for initiating early effective therapy. In this study, we developed a deep learning model to differentiate between multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) using brain magnetic resonance imaging (MRI) data. The model was based on a modified ResNet18 convolution neural network trained with 5-channel images created by selecting five 2D slices of 3D FLAIR images. The accuracy of the model was 76.1%, with a sensitivity of 77.3% and a specificity of 74.8%. Positive and negative predictive values were 76.9% and 78.6%, respectively, with an area under the curve of 0.85. Application of Grad-CAM to the model revealed that white matter lesions were the major classifier. This compact model may aid in the differential diagnosis of MS and NMOSD in clinical practice.

The association between allergic diseases and optic neuritis (ON) remains insufficiently characterized. This nationwide cohort study investigated the relationship between major allergic diseases (atopic dermatitis, asthma, and allergic rhinitis) and the risk of ON development. We performed a population-based cohort study utilizing data from the Korean National Health Insurance Service database. The study included 4,052,827 individuals aged 20 years or older who participated in health screenings in 2009. All participants were monitored until December 2018 or until ON diagnosis. Cox proportional hazards models were applied to estimate hazard ratios (HRs) for ON, adjusting for relevant confounders. Over a median follow-up duration of 8.2 years, 3,640 individuals (0.09%) were diagnosed with ON. After adjusting for confounders, individuals with allergic diseases exhibited a statistically significant increased ON risk compared to those without allergic diseases (adjusted HR 1.317, 95% confidence interval [CI] 1.216–1.426, p  < 0.0001). Among the specific allergic diseases, allergic rhinitis showed the highest association (adjusted HR 1.335, 95% CI 1.229–1.450, p  < 0.0001), whereas asthma and atopic dermatitis did not demonstrate statistically significant associations after adjustment. These results highlight potential common pathogenic mechanisms and underscore the importance of future studies to clarify the biological pathways connecting allergic diseases and ON.

BackgroundPeople with multiple sclerosis (MS) are more likely to develop stroke than those without. However, little is known about the association between neuromyelitis optica spectrum disorder (NMOSD) and the risk of stroke. We aimed to estimate the risk of stroke in patients with MS and NMOSD in South Korea.MethodsData from the Korean National Health Insurance between January 2010 and December 2017 were analysed. A total of 1541/1687 adult patients with MS/NMOSD, who were free of stroke were included. Matched controls were selected based on age, sex and the presence of hypertension, diabetes mellitus and dyslipidaemia.ResultsThe risk of developing stroke was 2.78 times higher (adjusted HR (aHR), 95% CI 1.91 to 4.05) in patients with MS compared with controls matched by age, sex, hypertension, diabetes mellitus and dyslipidaemia. The risk of stroke in NMOSD was also higher than that in matched controls (aHR=1.69, 95% CI 1.10 to 2.61) and not statistically different from that of MS (p=0.216). The patients with MS had a higher risk for either of ischaemic or haemorrhagic stroke (HR=2.63 and 2.93, respectively), whereas those with NMOSD had a higher risk for ischaemic stroke (HR=1.60) with marginal statistical significance.ConclusionsThe risk of stroke is increased in patients with MS and NMOSD and seemed comparable between the two conditions. This is the first study that estimates the risk of stroke in patients with MS and NMOSD within the same population.

BackgroundNeurodegeneration is associated with pathogenesis of both multiple sclerosis (MS) and neuromyelitis optica (NMOSD). Parkinson’s disease (PD) is a representative neurodegenerative disease, however, whether MS or NMOSD is associated with risk of PD is not known.MethodsMS and NMOSD cohorts were collected from the Korean National Health Insurance Service between 1 January 2010 and 31 December 2017, using International Classification of Diseases 10th revision diagnosis codes and information in the Rare Intractable Disease management programme. The PD incidence rate that occurred after a 1-year lag period was calculated and compared with that of a control cohort matched for age, sex, hypertension, diabetes and dyslipidaemia in a 1:5 ratio.ResultsThe incidence rates of PD in patients with MS and NMOSD were 3.38 and 1.27 per 1000 person-years, respectively, and were higher than that of their matched control groups. The adjusted HR of PD was 7.73 (95% CI, 3.87 to 15.47) in patients with MS and 2.61 (95% CI, 1.13 to 6.02) in patients with NMOSD compared with matched controls. In both patients with MS and NMOSD, there were no significant differences in relative risk when stratified by sex, age, diabetes, hypertension and dyslipidaemia.ConclusionThe PD risk was higher in patients with MS and NMOSD compared with healthy controls and was particularly high in patients with MS. Further investigations should be performed to determine the pathophysiology and occurrence of PD in patients with MS and NMOSD.

BackgroundThe risk–benefit relationship of immunosuppressive therapies (ISTs) for elderly patients with neuromyelitis optica spectrum disorder (NMOSD) is not well established. This study aimed to investigate the safety and efficacy of IST in elderly patients with NMOSD.MethodsThis retrospective study analysed IST efficacy and safety in 101 patients with aquaporin-4 antibody-positive NMOSD aged over 65 years, treated for at least 6 months at five Korean referral centres, focusing on relapse rates, infection events and discontinuation due to adverse outcomes.ResultsThe mean age at disease onset was 59.8 years, and female-to-male ratio was 4:1. Concomitant comorbidities at NMOSD diagnosis were found in 87 patients (86%). The median Expanded Disability Status Scale score at the initiation of IST was 3.5. The administered ISTs included azathioprine (n=61, 60%), mycophenolate mofetil (MMF) (n=48, 48%) and rituximab (n=41, 41%). Over a median of 5.8 years of IST, 58% of patients were relapse-free. The median annualised relapse rate decreased from 0.76 to 0 (p<0.001), and 81% experienced improved or stabilised disability. Patients treated with rituximab had a higher relapse-free rate than those treated with azathioprine or MMF (p=0.022). During IST, 21 patients experienced 25 severe infection events (SIEs) over the age of 65 years, and 3 died from pneumonia. 14 patients (14%) experienced 17 adverse events that led to switching or discontinuation of IST. When comparing the incidence rates of SIEs and adverse events, no differences were observed among patients receiving azathioprine, MMF and rituximab.ConclusionIn elderly patients with NMOSD, IST offers potential benefits in reducing relapse rates alongside a tolerable risk of adverse events.

Although fatigue is a major symptom in patients with neuromyelitis optica spectrum disorder (NMOSD), the underlying mechanism remains unclear. We explored the relationship between subcortical structures and fatigue severity to identify neural substrates of fatigue in NMOSD. Clinical characteristics with brain magnetic resonance imaging were evaluated in forty patients with NMOSD. Fatigue was assessed using the Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-fatigue) questionnaire (a higher score indicates less fatigue). We assessed the correlation between subcortical structures and fatigue severity using surface-based shape analysis. Most of the enrolled patients showed fatigue (72.5%; mean FACIT-fatigue score, 34.8 ± 10.8). The FACIT-fatigue score was negatively correlated with Expanded Disability Status Scale and Beck Depression Inventory scores ( r  = − 0.382, p  = 0.016; r  = − 0.578, p  < 0.001). We observed that the right thalamus was the only extracted region for various threshold experiments. Further, patients with lower FACIT-fatigue scores (more fatigue) had decreased local shape volume in the right thalamus. Fatigue is common in patients with NMOSD, and atrophy in the right thalamus is strongly correlated with fatigue severity. The local shape volume of the right thalamus might serve as a biomarker of fatigue in NMOSD.

Multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) are two representative chronic inflammatory demyelinating disorders of the central nervous system. We aimed to determine and compare the alterations of white matter (WM) connectivity between MS, NMOSD, and healthy controls (HC). This study included 68 patients with relapsing–remitting MS, 50 with NMOSD, and 26 HC. A network-based statistics method was used to assess disrupted patterns in WM networks. Topological characteristics of the three groups were compared and their associations with clinical parameters were examined. WM network analysis indicated that the MS and NMOSD groups had lower total strength, clustering coefficient, global efficiency, and local efficiency and had longer characteristic path length than HC, but there were no differences between the MS and NMOSD groups. At the nodal level, the MS group had more brain regions with altered network topologies than did the NMOSD group when compared with the HC group. Network alterations were correlated with Expanded Disability Status Scale score and disease duration in both MS and NMOSD groups. Two distinct subnetworks that characterized the disease groups were also identified. When compared with NMOSD, the most discriminative connectivity changes in MS were located between the thalamus, hippocampus, parahippocampal gyrus, amygdala, fusiform gyrus, and inferior and superior temporal gyri. In conclusion, MS patients had greater network dysfunction compared to NMOSD and altered short connections within the thalamus and inferomedial temporal regions were relatively spared in NMOSD compared with MS.

PurposeTo describe the causes of third, fourth, and sixth cranial nerve palsies in children and adolescents.MethodsIn this retrospective case series, a total of 66 patients aged 0–19 years with third, fourth, and sixth cranial nerve palsies seen in strabismus and neuro-ophthalmic practice from 2010 to 2017 were included. Causes of palsies were determined based on clinical assessment, high-resolution magnetic resonance imaging (MRI), and laboratory work-up.ResultsThirty-five patients had sixth cranial nerve palsy, 14 patients had third cranial nerve palsy (7 partial, 7 complete), 13 patients had fourth cranial nerve palsy, and 4 patients had combined cranial nerve palsies in this study. Neoplasia involving central nervous system was one of the most common causes of third, fourth, and sixth cranial nerve palsies both in children (age: 0–14 years) and adolescents (age: 15–19 years) (20% and 31%, respectively). Overall, neoplasia (23%) was the most common cause of acute third, fourth, and sixth cranial nerve palsies, followed by idiopathic cause (14%), inflammation (11%), and non-aneurysmal vascular contact (11%). Neoplasia was also the most common cause of sixth and third cranial nerve palsies (25% and 29%, respectively). The most common cause of fourth cranial nerve palsy was late decompensation in congenital fourth cranial nerve palsy (46%).ConclusionsA substantial proportion of pediatric and juvenile patients had serious pathologies for third, fourth, and sixth cranial nerve palsies. If nerve palsies are indicated, prompt diagnosis of etiologies using high-resolution MRI with contrast and laboratory work-up are important for this disease population.