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1. The impact of reduced skeletal muscle mass at stroke onset on 3-month functional outcomes in acute ischemic stroke patients.

Author

Kyong Young Kim, Seunguk Jung, Eun Bin Cho, Tae-Won Yang, Seung Joo Kim, Hyunsung Kim, and Sunhye Jung

Subjects
Medicine, Science
Abstract

IntroductionSarcopenia, characterized by reduced skeletal muscle mass (RMM), is increasingly recognized as a significant factor influencing outcomes in various health conditions, including stroke. Although most studies focus on sarcopenia developing during stroke rehabilitation, the impact of sarcopenia present at the onset of acute ischemic stroke remains underexplored. This study aims to evaluate the effect of RMM at stroke onset on 3-month functional outcomes in acute ischemic stroke patients.Materials and methodsWe prospectively enrolled acute ischemic stroke patients admitted between May 2019 and December 2019. Muscle mass was accessed early during hospitalization using whole-body dual-energy X-ray absorptiometry (DXA), and patients were categorized into RMM and normal muscle mass (NMM) groups based on the Asian Working Group for Sarcopenia (AWGS) criteria. Functional outcomes at 3 months were assessed using the modified Rankin Scale (mRS), with unfavorable outcomes defined as mRS scores 2-5. Multivariable logistic regression and SHAP (Shapley Additive exPlanations) analyses were used to evaluate the independent impact of RMM on 3-months functional outcomes.ResultsA total of 99 patients were analyzed. The RMM group had a significantly higher prevalence of unfavorable outcomes at 3 months compared to the NMM group (p < 0.001). Patients with RMM were older and presented with more severe strokes. Multivariable analysis confirmed RMM as an independent predictor of unfavorable outcomes (adjusted OR: 8.07, 95% CI: 1.603-40.66, p = 0.011), even after adjusting for age and initial stroke severity. SHAP analysis ranked RMM as the second most influential predictor of unfavorable outcomes, following NIHSS on admission. These findings indicate that RMM not only worsens initial stroke severity but also independently hinders post-stroke recovery.ConclusionsReduced muscle mass at the onset of acute ischemic stroke is a significant, independent predictor of unfavorable outcomes at 3 months. In addition to its impact on recovery, RMM is linked to older age and more severe strokes, worsening prognosis. Maintaining muscle mass is also crucial for stroke prevention, as it supports cardiovascular health and resilience. Early identification and intervention for sarcopenia can improve recovery and reduce future stroke risk.

Published
2025
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2. Differentiated pattern of complement system activation between MOG-IgG-associated disease and AQP4-IgG-positive neuromyelitis optica spectrum disorder

Author

Eun Bin Cho, Ju-Hong Min, Patrick Waters, Miyoung Jeon, Eun-Seon Ju, Ho Jin Kim, Su-Hyun Kim, Ha Young Shin, Sa-Yoon Kang, Young-Min Lim, Sun-Young Oh, Hye Lim Lee, Eunhee Sohn, Sang-Soo Lee, Jeeyoung Oh, Sunyoung Kim, So-Young Huh, Joong-Yang Cho, Jin Myoung Seok, Byung-Jo Kim, and Byoung Joon Kim

Subjects
myelin oligodendrocyte glycoprotein, neuromyelitis optica spectrum disorder, complement, terminal complement complex (sC5b-9), classical complement cascade, alternative complement activity, Immunologic diseases. Allergy, RC581-607
Abstract

BackgroundMyelin oligodendrocyte glycoprotein antibody (MOG) immunoglobulin G (IgG)-associated disease (MOGAD) has clinical and pathophysiological features that are similar to but distinct from those of aquaporin-4 antibody (AQP4-IgG)-positive neuromyelitis optica spectrum disorders (AQP4-NMOSD). MOG-IgG and AQP4-IgG, mostly of the IgG1 subtype, can both activate the complement system. Therefore, we investigated whether the levels of serum complement components, regulators, and activation products differ between MOGAD and AQP4-NMOSD, and if complement analytes can be utilized to differentiate between these diseases.MethodsThe sera of patients with MOGAD (from during an attack and remission; N=19 and N=9, respectively) and AQP4-NMOSD (N=35 and N=17), and healthy controls (N=38) were analyzed for C1q-binding circulating immune complex (CIC-C1q), C1 inhibitor (C1-INH), factor H (FH), C3, iC3b, and soluble terminal complement complex (sC5b-9).ResultsIn attack samples, the levels of C1-INH, FH, and iC3b were higher in the MOGAD group than in the NMOSD group (all, p

Published
2024
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3. Changes in smoking, alcohol consumption, and the risk of Parkinson’s disease

Author

Se Young Jung, Sohyun Chun, Eun Bin Cho, Kyungdo Han, Juhwan Yoo, Yohwan Yeo, Jung Eun Yoo, Su Min Jeong, Ju-Hong Min, and Dong Wook Shin

Subjects
cigarette smoking, alcohol consumption, Parkinson’s disease, Asia, nicotine, Neurosciences. Biological psychiatry. Neuropsychiatry, RC321-571
Abstract

ObjectiveThere have been no studies on the association between changes in smoking and alcohol consumption or combined changes in smoking and alcohol consumption frequencies and PD risk. To assess the influence of changes in smoking and alcohol consumption on the risk of Parkinson’s disease (PD).MethodsNational Health Insurance Service (NHIS) database between January 2009 to December 2011 was analyzed. A total of 3,931,741 patients were included. Study participants were followed up for the incidence of PD until December 2017.ResultsCompared to the sustained non-smokers, sustained light smokers (adjusted hazard ratio [aHR] 0.80, 95% confidence interval [CI] 0.75–0.85), sustained moderate smokers (aHR 0.54, 95% CI 0.47–0.61), and sustained heavy smokers (aHR 0.49, 95% CI 0.44–0.55) had a lower risk of PD. Compared to those who sustained non-drinking, sustained light drinkers (aHR 0.85 95% CI 0.89–0.91), sustained moderate drinkers (aHR 0.68, 95% CI 0.60–0.78), and sustained heavy drinkers (aHR 0.77, 95% CI 0.68–0.87) showed decreased risk of PD. Among non-drinkers, those who started drinking to a light level were at decreased risk of PD (aHR 0.84, 95% CI 0.77–0.91). Among non-smoking and non-drinking participants, those who initiated smoking only (aHR 0.78, 95% CI 0.70–0.86), drinking only (aHR 0.77, 95% CI 0.68–0.87), and both smoking and drinking (aHR 0.69, 95% CI 0.58–0.82) showed decreased risk of PD.ConclusionSmoking is associated with decreased risk of PD with a dose–response relationship. Alcohol consumption at a light level may also be associated with decreased risk of PD. Further studies are warranted to find the possible mechanisms for the protective effects of smoking and drinking on PD, which may present insights into the etiology of PD.

Published
2023
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4. The risk of dementia in multiple sclerosis and neuromyelitis optica spectrum disorder

Author

Eun Bin Cho, Se Young Jung, Jin-Hyung Jung, Yohwan Yeo, Hee Jin Kim, Kyungdo Han, Dong Wook Shin, and Ju-Hong Min

Subjects
multiple sclerosis, neuromyelitis optica spectrum disorders, dementia, risk, population, Neurosciences. Biological psychiatry. Neuropsychiatry, RC321-571
Abstract

IntroductionCognitive impairment is a common feature of multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD). However, there is a lack of population-based study of dementia risk in these disorders. In the present study, the risk of dementia in MS and NMOSD patients in Republic of Korea was estimated.MethodsData analyzed in this study were obtained from the Korean National Health Insurance Service (KNHIS) database between January 2010 and December 2017. The study included 1,347 MS patients and 1,460 NMOSD patients ≥40 years of age who had not been diagnosed with dementia within 1 year prior to the index date. Matched controls were selected based on age, sex, and the presence of hypertension, diabetes mellitus, or dyslipidemia.ResultsIn MS and NMOSD patients, the risk of developing any dementia [adjusted hazard ratio (aHR) = 2.34; 95% confidence interval (CI) = 1.84–2.96 and aHR = 2.19; 95% CI = 1.61–3.00, respectively], Alzheimer’s disease [AD; aHR = 2.23; 95% confidence interval (CI) = 1.70–2.91 and aHR = 1.99; 95% CI = 1.38–2.88, respectively], and vascular dementia (aHR = 3.75; 95% CI = 1.91–7.35 and aHR = 3.21; 95% CI = 1.47–7.02, respectively) was higher compared with the matched controls. NMOSD patients had a lower risk of any dementia and AD compared with MS patients after adjusting for age, sex, income, hypertension, diabetes, and dyslipidemia (aHR = 0.67 and 0.62).ConclusionThe risk of dementia increased in MS and NMOSD patients and dementia risk was higher in MS than in NMOSD.

Published
2023
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5. Disrupted structural network of inferomedial temporal regions in relapsing–remitting multiple sclerosis compared with neuromyelitis optica spectrum disorder

Author

Eun Bin Cho, Daegyeom Kim, ByeongChang Jeong, Jong Hwa Shin, Yeon Hak Chung, Sung Tae Kim, Byoung Joon Kim, Cheol E. Han, and Ju-Hong Min

Subjects
Medicine, Science
Abstract

Abstract 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.

Published
2022
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6. Association of subcortical structural shapes with fatigue in neuromyelitis optica spectrum disorder

Author

Jin Myoung Seok, Wanzee Cho, Doo-Hwan Son, Jong Hwa Shin, Eun Bin Cho, Sung Tae Kim, Byoung Joon Kim, Joon-Kyung Seong, and Ju-Hong Min

Subjects
Medicine, Science
Abstract

Abstract 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

Published
2022
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7. Multiple embolic infarctions and intracranial hemorrhage in a patient with gestational trophoblastic disease

Author

Kyong Young Kim, Seunguk Jung, Changhyo Yoon, Heejeong Jeong, Eun Bin Cho, Tae-Won Yang, Seung Joo Kim, Sean Hwang, and Ki-Jong Park

Subjects
gestational trophoblastic disease, stroke, intracranial hemorrhage, Neurology. Diseases of the nervous system, RC346-429
Abstract

Background Choriocarcinoma is a subtype of gestational trophoblastic disease (GTD) that can spread to multiple organs, including the central nervous system. Most cases of GTD affecting the central nervous system can cause intra- or extra-axial hemorrhages. Herein, we describe a rare case of multiple embolic infarctions and intracranial hemorrhages in a patient with GDT. Case Report A 36-year-old woman with sudden headache and right homonymous hemianopsia was admitted to our hospital 19 hours from symptom onset. Brain magnetic resonance imaging revealed small acute infarctions in the territories of the left posterior cerebral artery and both middle cerebral arteries. Furthermore, intracerebral hemorrhage in the left occipital lobe, small amounts of intraventricular hemorrhage, and subarachnoid hemorrhage were observed. In the past, she gave birth to her child through cesarean section 6 months ago. D-dimer level was elevated with a value of 1.61 µg/mL (reference range 1,000 IU/mL. She was diagnosed with GTD and underwent chemotherapy. Conclusion The precise pathogenesis of the coexistence of multiple embolic infarctions and intracranial hemorrhage remains unclear. Cancer-related coagulopathy, micro-tumor emboli, or both could be involved in the pathogenesis of the rare presentation of this patient.

Published
2020
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8. Effects of Vitamin D and Dexamethasone on Lymphocyte Proportions and Their Associations With Serum Concentrations of 25-Hydroxyvitamin D3In Vitro in Patients With Multiple Sclerosis or Neuromyelitis Optica Spectrum Disorder

Author

Eun Bin Cho, Jong Hwa Shin, Soonwook Kwon, Juhyeon Kim, Jin Myoung Seok, Byoung Joon Kim, and Ju-Hong Min

Subjects
vitamin D, lymphocytes, memory B cells, CYP24A1, multiple sclerosis, neuromyelitis optica spectrum disorder, Immunologic diseases. Allergy, RC581-607
Abstract

BackgroundClear associations have been found between vitamin D deficiency and several autoimmune diseases including multiple sclerosis (MS). However, the benefits of vitamin D supplementation on disease management remain a matter of debate.Objective and MethodsPatients with MS (N=12) and neuromyelitis optica spectrum disorder (NMOSD; N=12) were enrolled along with 15 healthy controls. Changes in lymphocyte subset proportions during stimulation of their peripheral blood mononuclear cells (PBMCs) with the active form of vitamin D, 1,25-dihydroxyvitamin D3 (1,25(OH)2D3), and correlations with serum concentrations of the vitamin D precursor 25-hydroxyvitamin D3 (serum 25(OH)D3) were explored. The impact of 1,25(OH)2D3 stimulation on the expression of vitamin-D-responsive genes in immune cells was also investigated.ResultsIn both MS and NMOSD, stimulation of PBMCs with 1,25(OH)2D3 followed by steroid suppressed the proliferation of total lymphocytes and T cells. The ratio of CD19+CD27+ memory B cells (Bmem) to all B cells after stimulation with 1,25(OH)2D3 was negatively correlated with serum 25(OH)D3 in MS (Spearman’s ρ=–0.594, p=0.042), but positively correlated in NMOSD (Pearson’s r = 0.739, p=0.006). However, there was no relationship between the ratio of Bmem to CD19+CD24+CD38+ regulatory B cells and serum 25(OH)D3 in either MS or NMOSD. In addition, the level of 1,25(OH)2D3-induced CYP24A1 mRNA expression in PBMCs was significantly and negatively correlated with serum 25(OH)D3 (for ΔCT, r=0.744, p=0.014) in MS.ConclusionThese findings suggest a beneficial impact of stimulation of PBMCs with vitamin D followed by steroid on the T-cell population. The association between patient serum 25(OH)D3 and the proportion of Bmem under immune-cell stimulation differed between MS and NMOSD. Further investigations are warranted with larger patient populations.

Published
2021
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9. White Matter Network Disruption and Cognitive Dysfunction in Neuromyelitis Optica Spectrum Disorder

Author

Eun Bin Cho, Cheol E. Han, Sang Won Seo, Juhee Chin, Jeong-Hyeon Shin, Hye-Jin Cho, Jin Myoung Seok, Sung Tae Kim, Byoung Joon Kim, Duk L. Na, Kwang-Ho Lee, Joon-Kyung Seong, and Ju-Hong Min

Subjects
neuromyelitis optica spectrum disorder, cognitive dysfunction, white matter network, diffusion tensor imaging, graph theory, multiple sclerosis, Neurology. Diseases of the nervous system, RC346-429
Abstract

Background: In neuromyelitis optica spectrum disorder (NMOSD), brain involvement is common and cognitive dysfunction is frequently found. The study investigated alterations of white matter (WM) connectivity using graph theory and correlations with cognitive dysfunction in patients with NMOSD.Methods: We prospectively enrolled patients with NMOSD (N = 14) and age- and sex-matched healthy controls (N = 21). Structural connections between any pair of the 90 cortical and subcortical regions were established using diffusion tensor imaging and graph theory. Network-based statistics was employed to assess differences in WM connectivity between the NMOSD and healthy control groups. We further investigated the relationship between the topological network characteristics and cognitive test performances.Results: WM network analysis showed decreased total strength of brain networks and two disrupted sub-networks in patients with NMOSD. The first featured six hub nodes in the rectus, hippocampus, calcarine, cuneus, and precuneus with the left-sided predominance. The second had six hub nodes in the orbitomiddle frontal, post-central, superior parietal, superior, and middle temporal, and caudate with the right-sided predominance. Compared to healthy controls, NMOSD patients showed poor performance on tests for attention/working memory and processing speed, visuospatial processing, and executive function, which were associated with significant decreases in nodal clustering coefficient, local efficiency, and regional efficiency in the disrupted sub-networks (all p < 0.05).Conclusions: The data show the overall WM disruption and the relationship between poor cognitive function and sub-network alterations identified by the network analysis in NMOSD patients. We suggest that cognitive dysfunction is related to dysconnectivity of WM network including default mode network in NMOSD.

Published
2018
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10. Fatigue in patients with neuromyelitis optica spectrum disorder and its impact on quality of life.

Author

Jin Myoung Seok, Misong Choi, Eun Bin Cho, Hye Lim Lee, Byoung Joon Kim, Kwang Ho Lee, Pamela Song, Eun Yeon Joo, and Ju-Hong Min

Subjects
Medicine, Science
Abstract

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 =

Published
2017
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11. Utility of the rio score and modified rio score in korean patients with multiple sclerosis.

Author

Jae-Won Hyun, Su-Hyun Kim, In Hye Jeong, Suk-Won Ahn, So-Young Huh, Min Su Park, Young In Eom, In Soo Joo, Joong-Yang Cho, Eun Bin Cho, Ju-Hong Min, Byoung Joon Kim, Nam-Hee Kim, Jeeyoung Oh, Kee Duk Park, and Ho Jin Kim

Subjects
Medicine, Science
Abstract

Early identification of suboptimal responders to multiple sclerosis (MS) treatment is critical for optimizing therapeutic decisions. The Rio score (RS) and modified Rio score (MRS) were developed to discriminate the responses to interferon-beta (IFNB) treatment in MS patients. This study was performed to evaluate the utility of RS and MRS in daily clinical practice in Korea.This was a real-world setting, multicenter, retrospective study of MS patients treated with IFNB from 10 hospitals in Korea. We investigated whether the RS and MRS at the early stage of IFNB therapy could predict treatment responses over 3 years. Suboptimal treatment responses at 3 years were defined as the presence of clinical relapse and/or EDSS progression and/or patients who had been treated with INFB for at least for 1 year and therapy was switched due to perceived treatment failure during the 2 years of follow-up.Seventy patients (50 females and 20 males) were enrolled; 92% (12/13) of patients with high RS and 86% (12/14) of patients with high MRS (score 2 or 3) were suboptimal responders, whereas 93% (53/57) of patients with low RS and 93% (52/56) patients with low MRS (score 0 or 1) showed optimal responses. New active lesions on MRI with clinical relapse in high RS and MRS were the most common combination in suboptimal responders.We confirmed that RS and MRS at 6-15 months of IFNB therapy were useful for predicting poor responders over 3 years.

Published
2015
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12. Prognostic Role of Functional Neuroimaging after Multilobar Resection in Patients with Localization-Related Epilepsy.

Author

Eun Bin Cho, Eun Yeon Joo, Dae-Won Seo, Seung-Chyul Hong, and Seung Bong Hong

Subjects
Medicine, Science
Abstract

To investigate the usage of functional neuroimaging as a prognostic tool for seizure recurrence and long-term outcomes in patients with multilobar resection, we recruited 90 patients who received multilobar resections between 1995 and 2013 with at least 1-year follow-up (mean 8.0 years). All patients were monitored using intracranial electroencephalography (EEG) after pre-surgical evaluation. Clinical data (demographics, electrophysiology, and neuroimaging) were reviewed retrospectively. Surgical outcomes were evaluated at 1, 2, 5 years after surgery, and at the end of the study. After 1 year, 56 patients (62.2%) became Engel class I and at the last follow-up, 47 patients (52.2%) remained seizure-free. Furthermore, non-localized 18F-fluorodeoxyglucose positron emission tomography (PET), identifying hypometabolic areas not concordant with ictal onset zones, significantly correlated with seizure recurrence after 1 year. Non-lesional magnetic resonance imaging (MRI) and left-sided resection correlated with poor outcomes. In the last follow-up, non-localized PET and left-sided resection significantly correlated with seizure recurrence. Both localized PET and ictal-interictal SPECT subtraction co-registered to MR (SISCOM) predicted good surgical outcomes in the last follow-up (69.2%, Engel I). This study suggests that PET and SISCOM may predict postoperative outcomes for patients after multilobar epilepsy and shows comparable long-term surgical outcomes after multilobar resection.

Published
2015
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14. Hereditary Diffuse Leukoencephalopathy with Axonal Spheroids Mistaken for Stroke: A Case Report

Author

Sean Hwang, Jangwan Ha, Min Young Choi, Heejeong Jeong, Eun Bin Cho, Tae-Won Yang, Ki-Jong Park, Seunguk Jung, and Seung Joo Kim

Abstract

Hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS) is a rare autosomal dominant disorder that is caused by colony stimulating factor 1 receptor gene mutation degenerating the cerebral white matter. It is characterized by various symptoms such as progressive cognitive decline, personality changes, and motor disorders. Since these clinical features are not consistent, HDLS is often underdiagnosed or misdiagnosed. This case report presents a patient priorly diagnosed with cerebral infarction by clinical features, but eventually with HDLS by a genetic test.

Published
2022
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17. Generalised autonomic failure as a prognostic factor in systemic light-chain (AL) amyloidosis

Author

Soonwook Kwon, Woo Kyo Jeong, Ji-Hyung Park, Juhyun Kim, Eun Bin Cho, Jin Myoung Seok, Seok Jin Kim, Kihyun Kim, Eun-Seok Jeon, Ju-Hong Min, and Byoung Joon Kim

Subjects
Internal Medicine
Abstract

In the present study, it was investigated whether autonomic dysfunction could predict prognosis in light-chain (AL) amyloidosis patients.Seventy-two patients with biopsy-proven AL amyloidosis were included and underwent an autonomic function test (AFT) between January 2016 and June 2019. Autonomic failure was evaluated using the Composite Autonomic Severity Score (CASS). Survival curves and the three-year overall survival (OS) rate were estimated using the Kaplan-Meier curve, and the Cox proportional hazards regression method was used to evaluate the variables that influenced survival.Autonomic dysfunction was observed in 69 (96%) patients with AL amyloidosis, and the three-year OS rate was 67%. Generalised autonomic failure (GAF) was observed in 31 (43%) patients. In the Kaplan-Meier curve, the three-year OS rates in patients with sudomotor dysfunction or GAF were lower than that in control patients (35 vs. 84%, and 33 vs. 81%, respectively). In Cox proportional hazards regression model, female, bone marrow plasma cell percentage, left ventricular systolic dysfunction, and GAF were significant independent variables associated with survival.The results of this study indicate that GAF on the AFT is an independent adverse prognostic factor for survival in AL amyloidosis patients.

Published
2022
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18. Lumbosacral polyradiculoneuropathy associated with combination therapy of nivolumab and ipilimumab

Author

Eun Bin Cho, Seung Joo Kim, Tae-Won Yang, Seunguk Jung, Heejeong Jeong, and Ki-Jong Park

Subjects
Immunology
Abstract

Immune checkpoint inhibitors (ICIs) have emerged as one of the most promising therapeutic options for advanced cancers. While ICIs have improved survival in multiple cancers, their increased use is restricted by various immune-related adverse events. In this report we describe a patient with renal cell carcinoma who received a combination of ICIs, nivolumab plus ipilimumab, and who developed lumbosacral polyradiculoneuropathy. Corticosteroid use was an effective treatment for this patient.

Published
2022
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19. Clinical Assessment Scales in Autonomic Nervous System Disorders

Author

Eun Bin Cho and Ki-Jong Park

Subjects
03 medical and health sciences, Autonomic nervous system, 0302 clinical medicine, business.industry, Medicine, 030204 cardiovascular system & hematology, business, Neuroscience, 030217 neurology & neurosurgery
Abstract

The autonomic nervous system plays an important role in maintaining homeostasis mediated by the parasympathetic, sympathetic and enteric systems. Autonomic failure adversely affects body function and may increase morbidity and mortality. Therefore, the scoring systems, such as Ewing’s classification and Composite Autonomic Scoring Scale (CASS), were developed to detect and quantify autonomic deficits, primarily focusing on the cardiovascular reflex system. Autonomic disorders manifest with a myriad of symptoms resulting from the dysfunction of the gastrointestinal, genitourinary, secretomotor, pupillomotor systems as well as cardiovascular system. Several self-report questionnaires, such as Composite Autonomic Symptom Scale (COMPASS), Scale for Outcomes in Parkinson’s disease for Autonomic Symptoms (SCOPA-AUT), Survey of Autonomic Symptom (SAS), were also used to support to detect various signs and symptoms of autonomic dysfunction in clinical settings. In this review, we introduce clinically useful assessment scales in autonomic nervous system disorders.

Published
2021
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20. Acute myocardial infarction risk in multiple sclerosis and neuromyelitis optica spectrum disorder: A nationwide cohort study in South Korea

Author

Eun Bin Cho, Yohwan Yeo, Jin-Hyung Jung, Su-Min Jeong, Kyungdo Han, Jeong Hoon Yang, Dong Wook Shin, and Ju-Hong Min

Subjects
Adult, Cohort Studies, Multiple Sclerosis, Neurology, Hypertension, Neuromyelitis Optica, Myocardial Infarction, Humans, Neurology (clinical), Aged
Abstract

Background: The link between neuromyelitis optica spectrum disorder (NMOSD) and cardiovascular disease is currently unclear. Objective: To determine the acute myocardial infarction (MI) risk in patients with MS and NMOSD. Methods: This study analyzed the Korean National Health Insurance Service database between January 2010 and December 2017. The included patients comprised 1503/1675 adults with MS/NMOSD who had not experienced ischemic heart disease or ischemic stroke at the index date. Matched controls were selected based on age, sex, and the presence of hypertension, diabetes mellitus (DM), and dyslipidemia. Results: The risks of developing MI were 2.61 (hazard ratio (HR), 95% confidence interval (CI) 1.73–3.95) and 1.95 (95% CI = 1.18–3.22) times higher in MS and NMOSD compared with the control populations. Patients with NMOSD had a similar MI risk compared with patients with MS, after adjusting for age, sex, income, hypertension, DM, and dyslipidemia (HR = 0.59, 95% CI = 0.34–1.02, p = 0.059). Among each patient group, the MI risk did not differ significantly with age (20–39, 40–64 or ⩾65 years), sex, or the presence of hypertension, DM, or dyslipidemia. Conclusion: The MI risk increased in MS and NMOSD and seemed to be comparable between NMOSD and MS.

Published
2022

21. Long-term safety and efficacy of patisiran for hereditary transthyretin-mediated amyloidosis with polyneuropathy: 12-month results of an open-label extension study

Author

David Adams, Michael Polydefkis, Alejandra González-Duarte, Jonas Wixner, Arnt V Kristen, Hartmut H Schmidt, John L Berk, Inés Asunción Losada López, Angela Dispenzieri, Dianna Quan, Isabel M Conceição, Michel S Slama, Julian D Gillmore, Theodoros Kyriakides, Senda Ajroud-Driss, Márcia Waddington-Cruz, Michelle M Mezei, Violaine Planté-Bordeneuve, Shahram Attarian, Elizabeth Mauricio, Thomas H Brannagan, Mitsuharu Ueda, Emre Aldinc, Jing Jing Wang, Matthew T White, John Vest, Erhan Berber, Marianne T Sweetser, Teresa Coelho, Giuseppe Vita, Vincenzo Rizzo, Massimo Russo, Anna Mazzeo, Luca Gentile, Caitlin Brueckner, Victoria Lazzari, Janice Wiesman, Douglas DeLong, Jennifer Victory, James Dalton, John May, Catherine Gilmore, Saran Diallo, Emilien Delmont, Jean Pouget, Annie Verschueren, Aude-Marie Grapperon, Emmanuelle Campana-Salort, Ana Lopes, Filipa Lamas, Carlos Neves, Jose Castro, Pedro Pereira, Isabel Castro, Ana Franco, Miguel Oliveira Santos, Conceição de Azevedo Coutinho, Catarina Falcao de Campos, Antonio Hipólito Reis, Nuno Correia, Javier M Perez, Ana Martins da Silva, Cristina Alves, Marcio Cardoso, Katia Valdrez, Julia R Monte, Bernardete Pessoa, Nadia Guimaraes, Monica Freitas, Joana Ramalho, Natalia Ferreira, Daisuke Kuzume, Celine Tard, Nawal Waucquier, Isabelle Rougeaux, Sylvie Brice, Emmanuelle Kasprzyk, Elise Elrezzi, Sayah Meguig, Eric Hachulla, Clement Gauvain, Maria-Claire Migaud-Chervy, Dominique Deplanque, Elsa Jozefowicz, Loic Lebellec, Line Balaya-Gouraya, Nathalie Jehan Lacour, Halima Bournane, Nathalie Martin, Mongia Elabed, Niamey Sacko, Yasmine Boubrit, Amina Gaouar, Fetra Rakotondratafika, Marie Théaudin-Saliou, Cécile Cauquil-Michon, Celine Labeyrie, Adeline Not, Abdallah Al-Salameh, Anne-Lise Lecoq, Maeva Stephant, Andoni Echaniz-Laguna, Laurent Becquemont, Guillemette Beaudonnet, Vincent Algalarrondo, Ludivine Eliahou, Antoine Rousseau, Aissatou Signate, Emeline Berthelot, Jocelyn Inamo, Laetitia Vervoitte, Cecile Focseneanu, Thierry Gendre, Raphaele Arrouasse, Samar S. Ayache, Laura Ernande, Philippe Le Corvoisier, Hayet Salhi, Ariane Choumert, Vincent Ehinger, Julie Ruiz, Cyril Charlin, Thomas Megelin, Thomas H Brannagan III, Raisy Fayerman, Arreum Kim, Allan Paras, Leidy J Gonzalez, Steven Tsang, Fernanda Wajnsztajn, Jeffrey Shije, Christina Ulane, Inna Kleyman, Louis Weimer, Comana Cioroiu, Sakis Lambrianides, Rana Abu-Manneh, Eleni Zamba-Papanicolaou, Petros Agathangelou, Eleni Leonidou, Satoshi Tada, Akemi Fujita, Masahiro Nagai, Rina Ando, Yuko Hosokawa, Yuki Yamanishi, J. Scott Overcash, Elena Giardino, Leslie Boyer, Lien Dang, An Le, Tyler Nguyen, Lien Giang, Peter Sellers, Leyla Tran, Nghi Truong, Maita Vinas, Nicole Hrkman, Sarah Miller, David Nguyen, Ashley Smith, Helen Pu, Steve Li, Thao Vuong, Holly Dioso, Sinikka Green, Kia Lee, Hanh Chu, Michael Waters, Derya J Coskun, Karla A Zepeda, William O'Riordan, Laura Obici, Andrea Cortese, Alessandro Lozza, Giampaolo Merlini, Vittorio Rosti, Mario Sabatelli, Giulia Bisogni, Daniela Bernardo, Marco Luigetti, Andrea Di Paolantonio, Valeria Guglielmino, Angela Romano, Hans Nienhuis, Janita Bulthuis-Kuiper, Olga Gerk, Hannah Ulbricht, Lenka Taylor, Eva Meyle, Natalia Kleinschmidt, David Meyrath, Simone Noe-Schwenn, Ulrike Meng, Ralf Bauer, Fabian aus dem Siepen, Selina Hein, Tetsuya Takahashi, Tomohiko Oshita, Yoko Koujin, Shuichiro Neshige, Tomohisa Nezu, Akiko Segawa, Hiroki Ueno, Hiroyuki Morino, Josep M Campistol, Lida Maria Rodas Marin, Josep Miquel Blasco Pelicano, Lucía Galán Dávila, Marta Palacios, Vanesa Pytel Cordoba, Antonio Guerrero Sola, Alejandro Horga, Julián García Feijoo, Leopoldo Perez de Isla, Wilson Marques Júnior, Mariana Moscardini, Debora Cristina Litcanov, Ana Flavia Viera Lima, Leonardo Rodrigues, Barbara Marques Coutinho, Carolina Lavigne Moreira, Vanessa Daccach Marques, Francisco Munoz Beamud, Álvaro Gragera Martínez, Cristina Borrachero, Eugenia Cisneros Barroso, Adrián Rodríguez Rodríguez, Monica Sanz, Elena Rigo Oliver, Juan González Moreno, Jose M Gamez Martinez, Cristina Descals, Mercedes Uson, Francisco Jose Vega, Antoni Figuerola, Carles Montala, Moises Dias da Silva, Renata Gervais de Santa Rosa, Luiz Felipe Pinto, Marcus Vinicius Pinto, Amanda Cardoso Berensztejn, Fabio Barroso, Andrea Lautre, Lucas G Orellana, Maria Alejandra González-Duarte Briseño, Karla Cárdenas-Soto, Brenda Poled Jiménez López, Sandra Lorena Pérez-Castañeda, Carlos Gerardo Cantú Brito, David Rivera de la Parra, Jose Pablo Hernandez Reyes, Maria del Mar Saniger Alba, Elia Criollo Mora, Yesim Parman, Kus Jülide Rezzan, Erdi Sahin, Nail G Serbest, Hacer Durmus, Arman Cakar, Nuriye Ilknur Tugal Tutkun, Sacit Karamursel, Ali Elitok, Nermin G Sirin Inan, Emre Altinkurt, Jing Ye, Adriane C Allen, Vinay Chaudhry, Raquel Jarrett, Neil Bressler, Kathleen L Burks, Qingfeng Liu, Mohammad Khoshnoodi, Daniel P Judge, Geno Vista, Syed Mahmood Shah, Hirotoshi Hamaguchi, Junko Oda, Emi Fukase, Ikuko Taniguchi, Tetsuya Oda, Hironobu Endo, Masahiro Shimomura, Kimitaka Katanazaka, Shusuke Koto, Takahiro Nakano, Christof Scheid, Andreas Zueiter, Lars Pester, Doreen Walter, Betül Özdemir, Lukas F Frenzel, Udo Holtick, Jeeyoung Oh, Hee Jin Kim, Hyun Jin Shin, Kyomin Choi, Taro Yamashita, Teruaki Masuda, Yohei Misumi, Akihiko Ueda, Keiichi Nakahara, Akiko Yorita, Seiko Tsuruhisa, Takayuki Taniwaki, Masaya Harada, Taiga Moritaka, Naonori Sakurada, Elizabeth A Mauricio, Amber Baskin, Elliot Dimberg, Amie Fonder, Miriam Hobbs, Stephen J Russell, Peter Dyck, Wilson Gonsalves, Nelson Leung, Thomas E Witzig, Steven R Zeldenrust, Lisa Hwa, Prashant Kapoor, Shaji K Kumar, Yi Lin, John A Lust, Vincent S Rajkumar, David Dingli, Morie A Gertz, Ronald Go, Suzanne R Hayman, Samir Dalia, Esmeralda Carrillo, Peter Gorevic, Garnette Mason, Chi-Chao Chao, Ming-Jen Lee, Jen-Jen Su, Sung-Tsang Hsieh, Li-Kai Tsai, Shin-Joe Yeh, Chih-Chao Yang, Senda Ajroud-Driss Ajroud-Driss, Patricia Casey, Benjamin C Joslin, Miriam Freimer, Alison Sankey, Amanda Kenepp, Sarah Heintzman, Samantha LoRusso, Youichi Hokezu, Byoung-Joon Kim, JuHyeon Kim, Ga Yeon Lee, Eun Bin Cho, Eun-Seok Jeon, Ju-Hong Min, Jin Myoung Seok, Hye Lim Lee, Jae Hong Park, Yoshiki Sekijima, Chinatsu Miyazawa, Nagaaki Kato, Dai Kishida, Akiyo Hineno, Minori Kodaira, Tsuneaki Yoshinaga, Teruyoshi Miyahara, Akira Imai, Kazuhiko Matsumoto, Kon-Ping Lin, Yi-Chung Lee, Malin Falk, Bjorn Pilebro, Ole Suhr, Per Lindqvist, Karin Soderberg, Fatima Pedrosa-Domellöf, Intissar Anan, Erik Nordh, Ivaylo Tournev, Sashka Zhelyazkova-Glaveeva, Zheyna Cherneva, Staiko Sarafov, Teodora Chamova, Sylvia Cherninkova-Gopina, Frauke Friebel, Andree Zibert, Natasa Mihailovic, Friederike Schubert, Elena Vorona, Larissa Lahme, Anna Huesing-Kabar, Matthias Schilling, Iyad Kabar, Ana Martinez-Naharro, Liza Chacko, Oliver Cohen, Steven Law, Tamer Rezk, Helen J Lachmann, Brianna Blume, Stacy Dixon, Soon Chai Low, Soo Looi Chan, He Eng Li Lim, Khean Jin Goh, Deborah Kraus, Kristin Jack, N. Kevin Wade, Glenn Lopate, Brittany Zwijack, Julaine Florence, R. Brian Sommerville, Graeme Stewart, Julie Ryder, Linda Mekhael, Mark Taylor, Daniel Suan, Karen Wells, Paula Stone, Amenze Itoya, Mercy Owusu-Sekyere, Desmond Thai, Ilonah Chahine, Salve Pedrosa, Thi Hoa (Therese) Do, and Repositório da Universidade de Lisboa

Subjects
Adult, Male, medicine.medical_specialty, Drug-Related Side Effects and Adverse Reactions, 030204 cardiovascular system & hematology, Placebo, Severity of Illness Index, Polyneuropathies, 03 medical and health sciences, 0302 clinical medicine, Quality of life, Internal medicine, Outcome Assessment, Health Care, Severity of illness, medicine, Humans, Prealbumin, RNA, Small Interfering, Infusions, Intravenous, Adverse effect, Aged, Amyloid Neuropathies, Familial, business.industry, Middle Aged, medicine.disease, Interim analysis, amyloidosis, transthyretin, polyneuropathy, patisiran, OLE study, Clinical trial, Female, Neurology (clinical), business, Polyneuropathy, 030217 neurology & neurosurgery, Progressive disease
Abstract

© 2020 Elsevier Ltd. All rights reserved., Background: Hereditary transthyretin-mediated amyloidosis is a rare, inherited, progressive disease caused by mutations in the transthyretin (TTR) gene. We assessed the safety and efficacy of long-term treatment with patisiran, an RNA interference therapeutic that inhibits TTR production, in patients with hereditary transthyretin-mediated amyloidosis with polyneuropathy. Methods: This multicentre, open-label extension (OLE) trial enrolled patients at 43 hospitals or clinical centres in 19 countries as of Sept 24, 2018. Patients were eligible if they had completed the phase 3 APOLLO or phase 2 OLE parent studies and tolerated the study drug. Eligible patients from APOLLO (patisiran and placebo groups) and the phase 2 OLE (patisiran group) studies enrolled in this global OLE trial and received patisiran 0·3 mg/kg by intravenous infusion every 3 weeks with plans to continue to do so for up to 5 years. Efficacy assessments included measures of polyneuropathy (modified Neuropathy Impairment Score +7 [mNIS+7]), quality of life, autonomic symptoms, nutritional status, disability, ambulation status, motor function, and cardiac stress, with analysis by study groups (APOLLO-placebo, APOLLO-patisiran, phase 2 OLE patisiran) based on allocation in the parent trial. The global OLE is ongoing with no new enrolment, and current findings are based on the interim analysis of the patients who had completed 12-month efficacy assessments as of the data cutoff. Safety analyses included all patients who received one or more dose of patisiran up to the data cutoff. This study is registered with ClinicalTrials.gov, NCT02510261. Findings: Between July 13, 2015, and Aug 21, 2017, of 212 eligible patients, 211 were enrolled: 137 patients from the APOLLO-patisiran group, 49 from the APOLLO-placebo group, and 25 from the phase 2 OLE patisiran group. At the data cutoff on Sept 24, 2018, 126 (92%) of 137 patients from the APOLLO-patisiran group, 38 (78%) of 49 from the APOLLO-placebo group, and 25 (100%) of 25 from the phase 2 OLE patisiran group had completed 12-month assessments. At 12 months, improvements in mNIS+7 with patisiran were sustained from parent study baseline with treatment in the global OLE (APOLLO-patisiran mean change -4·0, 95 % CI -7·7 to -0·3; phase 2 OLE patisiran -4·7, -11·9 to 2·4). Mean mNIS+7 score improved from global OLE enrolment in the APOLLO-placebo group (mean change from global OLE enrolment -1·4, 95% CI -6·2 to 3·5). Overall, 204 (97%) of 211 patients reported adverse events, 82 (39%) reported serious adverse events, and there were 23 (11%) deaths. Serious adverse events were more frequent in the APOLLO-placebo group (28 [57%] of 49) than in the APOLLO-patisiran (48 [35%] of 137) or phase 2 OLE patisiran (six [24%] of 25) groups. The most common treatment-related adverse event was mild or moderate infusion-related reactions. The frequency of deaths in the global OLE was higher in the APOLLO-placebo group (13 [27%] of 49), who had a higher disease burden than the APOLLO-patisiran (ten [7%] of 137) and phase 2 OLE patisiran (0 of 25) groups. Interpretation: In this interim 12-month analysis of the ongoing global OLE study, patisiran appeared to maintain efficacy with an acceptable safety profile in patients with hereditary transthyretin-mediated amyloidosis with polyneuropathy. Continued long-term follow-up will be important for the overall assessment of safety and efficacy with patisiran.

Published
2021
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22. Post-intervention Status in Patients With Refractory Myasthenia Gravis Treated With Eculizumab During REGAIN and Its Open-Label Extension

Author

Mantegazza, R., Wolfe, G. I., Muppidi, S., Wiendl, H., Fujita, K. P., O'Brien, F. L., Booth, H. D. E., Howard, J. F., Claudio Gabriel Mazia, Miguel, Wilken, Fabio, Barroso, Juliet, Saba, Marcelo, Rugiero, Mariela, Bettini, Marcelo, Chaves, Gonzalo, Vidal, Alejandra Dalila Garcia, Jan De Bleecker, Guy Van den Abeele, Kathy de Koning, Katrien De Mey, Rudy, Mercelis, Délphine, Mahieu, Linda, Wagemaekers, Philip Van Damme, Annelies, Depreitere, Caroline, Schotte, Charlotte, Smetcoren, Olivier, Stevens, Sien Van Daele, Nicolas, Vandenbussche, Annelies, Vanhee, Sarah, Verjans, Jan, Vynckier, Ann, D'Hont, Petra, Tilkin, Alzira Alves de Siqueira Carvalho, Igor Dias Brockhausen, David, Feder, Daniel, Ambrosio, Gabor Lovasamela César, Ana Paula Melo, Renata Martins Ribeiro, Rosana, Rocha, Bruno Bezerra Rosa, Thabata, Veiga, Luiz Augusto da Silva, Murilo Santos Engel, Jordana Gonçalves Geraldo, Maria da Penha Ananias Morita, Erica Nogueira Coelho, Gabriel, Paiva, Marina, Pozo, Natalia, Prando, Debora Dada Martineli Torres, Cristiani Fernanda Butinhao, Gustavo, Duran, Tomás Augusto Suriane Fialho, Tamires Cristina Gomes da Silva, Luiz Otavio Maia Gonçalves, Lucas Eduardo Pazetto, Luciana Renata Cubas Volpe, Luciana Souza Duca, Maurício AndréGheller Friedrich, Alexandre, Guerreiro, Henrique, Mohr, Maurer Pereira Martins, Daiane da Cruz Pacheco, Luciana, Ferreira, Ana Paula Macagnan, Graziela, Pinto, Aline de Cassia Santos, Acary Souza Bulle Oliveira, Ana Carolina Amaral de Andrade, Marcelo, Annes, Liene Duarte Silva, Valeria Cavalcante Lino, Wladimir, Pinto, Natália, Assis, Fernanda, Carrara, Carolina, Miranda, Iandra, Souza, Patrícia, Fernandes, Zaeem, Siddiqi, Cecile, Phan, Jeffrey, Narayan, Derrick, Blackmore, Ashley, Mallon, Rikki, Roderus, Elizabeth, Watt, Stanislav, Vohanka, Josef, Bednarik, Magda, Chmelikova, Marek, Cierny, Stanislava, Toncrova, Jana, Junkerova, Barbora, Kurkova, Katarina, Reguliova, Olga, Zapletalova, Jiri, Pitha, Iveta, Novakova, Michaela, Tyblova, Ivana, Jurajdova, Marcela, Wolfova, Henning, Andersen, Thomas, Harbo, Lotte, Vinge, Susanne, Krogh, Anita, Mogensen, John, Vissing, Joan, Højgaard, Nanna, Witting, Anne Mette Ostergaard Autzen, Jane, Pedersen, Juha-Pekka, Erälinna, Mikko, Laaksonen, Olli, Oksaranta, Tuula, Harrison, Jaana, Eriksson, Csilla, Rozsa, Melinda, Horvath, Gabor, Lovas, Judit, Matolcsi, Gedeonne, Jakab, Gyorgyi, Szabo, Brigitta, Szabadosne, Laszlo, Vecsei, Livia, Dezsi, Edina, Varga, Monika, Konyane, Antonini, Giovanni, Antonella Di Pasquale, Garibaldi, Matteo, Morino, Stefania, Troili, Fernanda, Fionda, Laura, Amelia, Evoli, Paolo Emilio Alboini, Valentina, D'Amato, Raffaele, Iorio, Inghilleri, Maurizio, Frasca, Vittorio, Elena, Giacomelli, Gori, MARIA CRISTINA, Diego, Lopergolo, Onesti, Emanuela, Maria, Gabriele, Francesco, Saccà, Alessandro, Filla, Teresa, Costabile, Enrico, Marano, Angiola, Fasanaro, Angela, Marsili, Giorgia, Puorro, Carlo, Antozzi, Silvia, Bonanno, Giorgia, Camera, Alberta, Locatelli, Lorenzo, Maggi, Maria, Pasanisi, Angela, Campanella, Akiyuki, Uzawa, Tetsuya, Kanai, Naoki, Kawaguchi, Masahiro, Mori, Yoko, Kaneko, Akiko, Kanzaki, Eri, Kobayashi, Hiroyuki, Murai, Katsuhisa, Masaki, Dai, Matsuse, Takuya, Matsushita, Taira, Uehara, Misa, Shimpo, Maki, Jingu, Keiko, Kikutake, Yumiko, Nakamura, Yoshiko, Sano, Kimiaki, Utsugisawa, Yuriko, Nagane, Ikuko, Kamegamori, Tomoko, Tsuda, Yuko, Fujii, Kazumi, Futono, Yukiko, Ozawa, Aya, Mizugami, Yuka, Saito, Makoto, Samukawa, Hidekazu, Suzuki, Miyuki, Morikawa, Sachiko, Kamakura, Eriko, Miyawaki, Meinoshin, Okumura, Soichiro, Funaka, Tomohiro, Kawamura, Masayuki, Nakamori, Masanori, Takahashi, Namie, Taichi, Tomoya, Hasuike, Eriko, Higuchi, Hisako, Kobayashi, Kaori, Osakada, Hirokazu, Shiraishi, Teiichiro, Miyazaki, Masakatsu, Motomura, Akihiro, Mukaino, Shunsuke, Yoshimura, Shizuka, Asada, Seiko, Yoshida, Shoko, Amamoto, Tomomi, Kobashikawa, Megumi, Koga, Maeda, Yasuko, Kazumi, Takada, Mihoko, Takada, Masako, Tsurumaru, Yumi, Yamashita, Yasushi, Suzuki, Tetsuya, Akiyama, Koichi, Narikawa, Ohito, Tano, Kenichi, Tsukita, Rikako, Kurihara, Fumie, Meguro, Yusuke, Fukuda, Miwako, Sato, Tomihiro, Imai, Emiko, Tsuda, Shun, Shimohama, Takashi, Hayashi, Shin, Hisahara, Jun, Kawamata, Takashi, Murahara, Masaki, Saitoh, Shuichiro, Suzuki, Daisuke, Yamamoto, Yoko, Ishiyama, Naoko, Ishiyama, Mayuko, Noshiro, Rumi, Takeyama, Kaori, Uwasa, Ikuko, Yasuda, Anneke van der Kooi, Marianne de Visser, Tamar, Gibson, Byung-Jo, Kim, Chang Nyoung Lee, Yong Seo Koo, Hung Youl Seok, Hoo Nam Kang, Hyejin, Ra, Byoung Joon Kim, Eun Bin Cho, Misong, Choi, Hyelim, Lee, Ju-Hong, Min, Jinmyoung, Seok, Jieun, Lee, Da Yoon Koh, Juyoung, Kwon, Sangae, Park, Eun Haw Choi, Yoon-Ho, Hong, So-Hyun, Ahn, Dae Lim Koo, Jae-Sung, Lim, Chae Won Shin, Ji Ye Hwang, Miri, Kim, Seung Min Kim, Ha-Neul, Jeong, Jinwoo, Jung, Yool-Hee, Kim, Hyung Seok Lee, Ha Young Shin, Eun Bi Hwang, Miju, Shin, Carlos, Casasnovas, Maria Antonia Alberti Aguilo, Christian, Homedes-Pedret, Natalia Julia Palacios, Laura Diez Porras, Valentina Velez Santamaria, Ana, Lazaro, Josep Gamez Carbonell, Pilar, Sune, Maria Salvado Figueras, Gisela, Gili, Gonzalo, Mazuela, Isabel, Illa, Elena Cortes Vicente, Jordi, Diaz-Manera, Luis Antonio Querol Gutiérrez, Ricardo Rojas Garcia, Nuria, Vidal, Elisabet, Arribas-Ibar, Exuperio Diez Tejedor, Pilar Gomez Salcedo, Mireya, Fernandez-Fournier, Pedro Lopez Ruiz, Francisco Javier Rodriguez de Rivera, Maria, Sastre, Fredrik, Piehl, Albert, Hietala, Lena, Bjarbo, Ihsan, Sengun, Arzu, Meherremova, Pinar, Ozcelik, Bengu, Balkan, Celal, Tuga, Muzeyyen, Ugur, Sevim, Erdem-Ozdamar, Can Ebru Bekircan-Kurt, Nazire Pinar Acar, Ezgi, Yilmaz, Yagmur, Caliskan, Gulsah, Orsel, Husnu, Efendi, Seda, Aydinlik, Hakan, Cavus, Ayse, Kutlu, Gulsah, Becerikli, Cansu, Semiz, Ozlem, Tun, Murat, Terzi, Baki, Dogan, Musa Kazim Onar, Sedat, Sen, Tugce Kirbas Cavdar, Adife, Veske, Fiona, Norwood, Aikaterini, Dimitriou, Jakit, Gollogly, Mohamed, Mahdi-Rogers, Arshira, Seddigh, Giannis, Sokratous, Gal, Maier, Faisal, Sohail, Saiju, Jacob, Girija, Sadalage, Pravin, Torane, Claire, Brown, Amna, Shah, Sivakumar, Sathasivam, Heike, Arndt, Debbie, Davies, Dave, Watling, Anthony, Amato, Thomas, Cochrane, Mohammed, Salajegheh, Kristen, Roe, Katherine, Amato, Shirli, Toska, Nicholas, Silvestri, Kara, Patrick, Karen, Zakalik, Jonathan, Katz, Robert, Miller, Marguerite, Engel, Dallas, Forshew, Elena, Bravver, Benjamin, Brooks, Mohammed, Sanjak, Sarah, Plevka, Maryanne, Burdette, Scott, Cunningham, Megan, Kramer, Joanne, Nemeth, Clara, Schommer, Tierney, Scott, Vern, Juel, Jeffrey, Guptill, Lisa, Hobson-Webb, Janice, Massey, Kate, Beck, Donna, Carnes, John, Loor, Amanda, Anderson, Robert, Pascuzzi, Cynthia, Bodkin, John, Kincaid, Riley, Snook, Sandra, Guingrich, Angela, Micheels, Vinay, Chaudhry, Andrea, Corse, Betsy, Mosmiller, Andrea, Kelley, Doreen, Ho, Jayashri, Srinivasan, Michal, Vytopil, Jordan, Jara, Nicholas, Ventura, Cynthia, Carter, Craig, Donahue, Carol, Herbert, Stephanie, Scala, Elaine, Weiner, Sharmeen, Alam, Jonathan, Mckinnon, Laura, Haar, Naya, Mckinnon, Karan, Alcon, Kaitlyn, Mckenna, Nadia, Sattar, Kevin, Daniels, Dennis, Jeffery, Miriam, Freimer, Joseph Chad Hoyle, John, Kissel, Julie, Agriesti, Sharon, Chelnick, Louisa, Mezache, Colleen, Pineda, Filiz, Muharrem, Chafic, Karam, Julie, Khoury, Tessa, Marburger, Harpreet, Kaur, Diana, Dimitrova, James, Gilchrist, Brajesh, Agrawal, Mona, Elsayed, Stephanie, Kohlrus, Angela, Ardoin, Taylor, Darnell, Laura, Golden, Barbara, Lokaitis, Jenna, Seelbach, Neelam, Goyal, Sarada, Sakamuri, Yuen, T So, Shirley, Paulose, Sabrina, Pol, Lesly, Welsh, Ratna, Bhavaraju-Sanka, Alejandro Tobon Gonzalez, Lorraine, Dishman, Floyd, Jones, Anna, Gonzalez, Patricia, Padilla, Amy, Saklad, Marcela, Silva, Sharon, Nations, Jaya, Trivedi, Steve, Hopkins, Mohamed, Kazamel, Mohammad, Alsharabati, Liang, Lu, Kenkichi, Nozaki, Sandi, Mumfrey-Thomas, Amy, Woodall, Tahseen, Mozaffar, Tiyonnoh, Cash, Namita, Goyal, Gulmohor, Roy, Veena, Mathew, Fatima, Maqsood, Brian, Minton, H James Jones, Jeffrey, Rosenfeld, Rebekah, Garcia, Laura, Echevarria, Sonia, Garcia, Michael, Pulley, Shachie, Aranke, Alan Ross Berger, Jaimin, Shah, Yasmeen, Shabbir, Lisa, Smith, Mary, Varghese, Laurie, Gutmann, Ludwig, Gutmann, Nivedita, Jerath, Christopher, Nance, Andrea, Swenson, Heena, Olalde, Nicole, Kressin, Jeri, Sieren, Richard, Barohn, Mazen, Dimachkie, Melanie, Glenn, April, Mcvey, Mamatha, Pasnoor, Jeffery, Statland, Yunxia, Wang, Tina, Liu, Kelley, Emmons, Nicole, Jenci, Jerry, Locheke, Alex, Fondaw, Kathryn, Johns, Gabrielle, Rico, Maureen, Walsh, Laura, Herbelin, Charlene, Hafer-Macko, Justin, Kwan, Lindsay, Zilliox, Karen, Callison, Valerie, Young, Beth, Disanzo, Kerry, Naunton, Michael, Benatar, Martin, Bilsker, Khema, Sharma, Anne, Cooley, Eliana, Reyes, Sara-Claude, Michon, Danielle, Sheldon, Julie, Steele, Rebecca, Traub, Manisha, Chopra, Tuan, Vu, Lara, Katzin, Terry, Mcclain, Brittany, Harvey, Adam, Hart, Kristin, Huynh, Said, Beydoun, Amaiak, Chilingaryan, Victor, Doan, Brian, Droker, Hui, Gong, Sanaz, Karimi, Frank, Lin, Krishna, Polaka, Akshay, Shah, Anh, Tran, Salma, Akhter, Ali, Malekniazi, Rup, Tandan, Michael, Hehir, Waqar, Waheed, Shannon, Lucy, Michael, Weiss, Jane, Distad, Susan, Strom, Sharon, Downing, Bryan, Kim, Tulio, Bertorini, Thomas, Arnold, Kendrick, Henderson, Rekha, Pillai, Liu, Ye, Lauren, Wheeler, Jasmine, Hewlett, Mollie, Vanderhook, Richard, Nowak, Daniel, Dicapua, Benison, Keung, Aditya, Kumar, Huned, Patwa, Kimberly, Robeson, Irene, Yang, Joan, Nye, and Hong, Vu

Subjects
Adult, Male, 0301 basic medicine, medicine.medical_specialty, Clinical Neurology, Antibodies, Monoclonal, Humanized, Placebo, Article, Antibodies, Post-intervention, law.invention, 03 medical and health sciences, 0302 clinical medicine, Randomized controlled trial, Refractory, law, Internal medicine, Monoclonal, Myasthenia Gravis, Medicine and Health Sciences, Humans, Medicine, Humanized, Science & Technology, business.industry, Middle Aged, Eculizumab, Complement Inactivating Agents, Female, Treatment Outcome, EFFICACY, medicine.disease, Myasthenia gravis, Clinical trial, 030104 developmental biology, SAFETY, Neurosciences & Neurology, Neurology (clinical), business, Life Sciences & Biomedicine, COMPLEMENT INHIBITOR ECULIZUMAB, 030217 neurology & neurosurgery, medicine.drug
Abstract

ObjectiveTo evaluate whether eculizumab helps patients with anti–acetylcholine receptor–positive (AChR+) refractory generalized myasthenia gravis (gMG) achieve the Myasthenia Gravis Foundation of America (MGFA) post-intervention status of minimal manifestations (MM), we assessed patients' status throughout REGAIN (Safety and Efficacy of Eculizumab in AChR+ Refractory Generalized Myasthenia Gravis) and its open-label extension.MethodsPatients who completed the REGAIN randomized controlled trial and continued into the open-label extension were included in this tertiary endpoint analysis. Patients were assessed for the MGFA post-intervention status of improved, unchanged, worse, MM, and pharmacologic remission at defined time points during REGAIN and through week 130 of the open-label study.ResultsA total of 117 patients completed REGAIN and continued into the open-label study (eculizumab/eculizumab: 56; placebo/eculizumab: 61). At week 26 of REGAIN, more eculizumab-treated patients than placebo-treated patients achieved a status of improved (60.7% vs 41.7%) or MM (25.0% vs 13.3%; common OR: 2.3; 95% CI: 1.1–4.5). After 130 weeks of eculizumab treatment, 88.0% of patients achieved improved status and 57.3% of patients achieved MM status. The safety profile of eculizumab was consistent with its known profile and no new safety signals were detected.ConclusionEculizumab led to rapid and sustained achievement of MM in patients with AChR+ refractory gMG. These findings support the use of eculizumab in this previously difficult-to-treat patient population.ClinicalTrials.gov IdentifierREGAIN, NCT01997229; REGAIN open-label extension, NCT02301624.Classification of EvidenceThis study provides Class II evidence that, after 26 weeks of eculizumab treatment, 25.0% of adults with AChR+ refractory gMG achieved MM, compared with 13.3% who received placebo.

Published
2020
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23. Neuronal Intranuclear Inclusion Disease Presenting with Recurrent Headache, Confusion and Progressive Cognitive Decline

Author

Seung Joo Kim, Tae-Won Yang, Seunguk Jung, Eun Bin Cho, Heejeong Jeong, Ki-Jong Park, Chang Hyo Yoon, and Hee Young Kang

Subjects
NEURONAL INTRANUCLEAR INCLUSION DISEASE, Pathology, medicine.medical_specialty, business.industry, medicine, Dementia, Intranuclear Inclusion Body, medicine.symptom, medicine.disease, business, Progressive cognitive decline, Confusion
Published
2020
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24. Risk of stroke in multiple sclerosis and neuromyelitis optic spectrum disorder: a Nationwide cohort study in South Korea

Author

Eun Bin Cho, Yohwan Yeo, Jin Hyung Jung, Su-Min Jeong, Kyung-do Han, Dong Wook Shin, and Ju-Hong Min

Subjects
Psychiatry and Mental health, Surgery, Neurology (clinical)
Abstract

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.

Published
2022

25. Risk of Parkinson's disease in multiple sclerosis and neuromyelitis optica spectrum disorder: a nationwide cohort study in South Korea

Author

Soonwook Kwon, Se Young Jung, Kyung-do Han, Jin Hyung Jung, Yohwan Yeo, Eun Bin Cho, Jong Hyeon Ahn, Dong Wook Shin, and Ju-Hong Min

Subjects
Psychiatry and Mental health, Surgery, Neurology (clinical)
Abstract

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.

Published
2022

27. Benefits of eculizumab in AQP4+ neuromyelitis optica spectrum disorder: Subgroup analyses of the randomized controlled phase 3 PREVENT trial

Author

Jacqueline Palace, Dean M. Wingerchuk, Kazuo Fujihara, Achim Berthele, Celia Oreja-Guevara, Ho Jin Kim, Ichiro Nakashima, Michael Levy, Murat Terzi, Natalia Totolyan, Shanthi Viswanathan, Kai-Chen Wang, Amy Pace, Marcus Yountz, Larisa Miller, Róisín Armstrong, Sean Pittock, Daniel Julio Muñoz, Jorge David Amor, Carolina Bocchiardo, Julieta Iourno Danielle, Alfredo Laffue, Carolina Daniela Diaz Obregon, Maria Fernanda Paez, Roberto Martin Perez, Viviana Ana Maria Rocchi, Loreley Deborah Teijeiro, Jesica Gómez, Andres Maria Villa, Florencia Aguirre, Victoria Carla Fernández, Ramon F. Goicoechea, Luciana Melamud, Ana Stillman, Mariana de Virgiliis, Fatima Pagani Cassara, Marta Cordoba, Maria Teresa Gutierrez, Mariana Ingolotti, Natalia Larripa, Anahi Lupinacci, Josefina Arroyo, Alejandra Romano, Mariana Foa Torres, Carlos Héctor Ballario, Ana Elisa Chiesa, Hernán Gustavo Gómez, Hernán Gabriel Lattini, Carolina Natalia Mainella, Gisel Edith Bolner, María Soledad Eschoyez, Simon Andrew Broadley, Saman Heshmat, Arman Sabet, Andrew Swayne, Susan Freeman, Sofia Jimenez Sanchez, Neil Shuey, Linda Dalic, Ann French, Guru Kuma, Joshua Laing, Lai Yin Law, Jennifer MacIntyre, Andrew Neal, Christopher Plummer, Prashanth Ramachandran, Leslie Sedal, Ian Wilson, Antony Winkel, Wenwen Zhang, Tina Chen, Rani Watts, Michael Barnett, Joshua Barton, Heidi Beadnall, Justin Garber, Todd Andrew Hardy, Benjamin Trewin, Marinda Taha, Deleni Walters, Federico Arturo Silva Sieger, Nhora Patricia Ruiz Alfonso, Anna Maria Pinzon Camacho, Alexander Pabón Moreno, Jorge Armando Castellanos Prad, Adriana Paola Duarte Rueda, Tatiana Castillo, Karol Melissa Castillo Gonzalez, Martha Yolanda Moreno Pico, Judith Castill, Mario Habek, Ivan Adamec, Barbara Barun, Luka Crnosija, Tereza Gabelic, Petra Nytrova, Eva Krasulova, Jana Pavlickova, Michaela Tyblova, Jana Zubkova, Thor Petersen, Gro Helen Dale, Peter Vestergaard Rasmussen, Morten Stilund, Kristina Bacher Svendsen, Vivi Brandt, Nicolas Collongues, Marie-Celine Fleury, Laurent Kremer, Sandrine Bendele, Valérie Neff, Ricarda Diem, Michael Platten, Anne Berberich, Jonabelle Jansen, Hannah Jaschoneck, Brigitte Wildemann, Ursula Aures, Tanja Brandenburger, Tanja Haut, Maria-Lourdes Treceno Fernández, Lilian Aly, Kirsten Brinkhoff, Dorothea Buck, Daniel Golkowski, Mirjam Hermisson, Muna-Miriam Hoshi, Miriam Kaminski, Markus Christian Kowarik, Helena Kronsbein, Klaus Lehmann-Horn, Viola Maria Pongratz, Andreas Schweiker, Lisa-Ann Leddy, Silvia Mueller, Kim Obergfell, Marion Wanka, Uwe Klaus Zettl, Jan Klinke, Micha Loebermann, Stefanie Meister, Florian Rimmele, Alexander Winkelmann, Ina Schroeder, Alexander Yuk-Lun Lau, Lisa Wing-Chi Au, Florence Sin-Ying Fan, Vincent Hing-Lung Ip, Karen Ka-Yan Ma, Sze-Ho Ma, Vincent Chung-Tong Mok, Cheryl Chung-Kwan Au, Pauline Wing-Lam Kwan, Francesco Patti, Andrea Salvatore Caramma, Clara Grazia Chisari, Salvatore Lo Fermo, Silvia Messina, Maria Projetto, Cinzia Caserta, Alessandro Filla, Teresa Costabile, Chiara Pane, Francesco Sacca, Angela Marsili, Giorgia Puorro, Roberto Bergamaschi, Eliana Berra, Giulia Mallucci, Cinzia Fattore, Claudio Gasperini, Simonetta Galgani, Shalom Haggiag, Serena Ruggieri, Claudio Vento, Esmeralda Maria Quartuccio, Carlo Pozzilli, Valeria Teresa Barletta, Giovanna Borriello, Laura De Giglio, Fabiana Marinelli, Miriam Tasillo, Alessandra Amadori, Mariano Fischetti, Flavia Gurreri, Masahiro Mori, Hiroki Masuda, Ryohei Ohtani, Yukari Sekiguchi, Tomohiko Uchida, Akiyuki Uzawa, Hiromi Ito, Emi Kabasawa, Yoko Kaneko, Takuya Matsushita, Dai Matsuse, Hiroyuki Murai, Shintaro Hayashi, Katsuhisa Masak, Hidenori Ogata, Koji Shinoda, Taira Uehara, Mitsuru Watanabe, Hiroo Yamaguchi, Ryo Yamasaki, Tomomi Yonekawa, Maki Jingu, Makiko Nagano, Yumiko Nakamura, Yoshiko Sano, Manabu Araki, Youwei Lin, Madoka Mori, Yohei Mukai, Terunori Sano, Wakiro Sato, Naoya Gogun, Yuriko Maeda, Asami Nishimoto, Sachiko Tsukamoto, Ritsuko Yanagi, Takahiko Saida, Shinichi Nakamura, Tetsuya Nasu, Kyoko Saida, Yuko Shikata, Yoshimi Kodani, Megumi Saeki, Yukako Sawada, Hiroo Yoshikawa, Takashi Kimura, Masamitsu Nishi, Shun Sakamoto, Shinichiro Ukon, Shohei Watanabe, Saori Ebisuya, Nami Kimura, Manami Matsuura, Yukie Morisaki, Yoshiko Muroi, Kuniko Onishi, Ikuko Oshima, Yuki Washino, Tomomi Yamashita, Tatsuro Misu, Kimihiko Kaneko, Masaaki Kato, Hiroshi Kuroda, Kazuhiro Kurosawa, Shuhei Nishiyama, Hirohiko Ono, Yoshiki Takai, Keiko Abe, Hitomi Hoshi, Mari Jinushi, Azusa Oyama, Motonari Sakuma, Yuko Sawada, Satoru Ishibashi, Takanori Yokota, Yoichiro Nishida, Kokoro Ozaki, Nobuo Sanjo, Nozomu Sato, Fuki Denno, Haruko Hiraki, Yumi Matsubara, Takashi Kanda, Masaaki Abe, Masaya Honda, Motoharu Kawai, Michiaki Koga, Toshihiko Maeda, Junichi Ogasawara, Masatoshi Omoto, Yasuteru Sano, Ryota Sato, Fumitaka Shimizu, Hideki Arima, Sachie Fukui, Yoshiko Ishikawa, Tomoko Koyama, Shigemi Shimose, Hirokazu Shinozaki, Masanori Watanabe, Sachi Yasuda, Chieko Yoshiwaka, Suffian Adenan, Mohd Azman M Aris, Ahmad Shahir bin Mawardi, Muhammad Al Hafiz Adnan, Nanthini Munusamy, Siti Nur Omaira Razali, Punitha Somasundram, Jae Won Hyun, In Hye Jeong, Su-Hyun Kim, Hyun-June Shin, Ji Sung Yoo, HyunMin Jang, AeRan Joung, Byung-Jo Kim, Seol-Hee Baek, Jung Bin Kim, Yoo Hwan Kim, Yong Seo Koo, Chan Nyoung Lee, Hung Youl Seok, Jinhee Hwang, Sung Min Kim, So Hyun Ahn, Kyomin Choi, Seok-Jin Choi, Jun-Soon Kim, Young Nam Kwon, Je-Young Shin, Hyeonju Kwon, Byoung Joon Kim, Eun Bin Cho, Hye-Jin Cho, Misong Choi, DongSun Kim, Ju Hyeon Kim, SeungJu Ki, Hye Lim Lee, Kwang-Ho Lee, Ju-Hong Min, Ji-Hyung Park, Jinmyoung Seok, Eunhwa Choi, Sang Ae Park, Seung Min Kim, Ha-Neul Jeong, Bong Jeongbin, Jin Woo Jung, Seung Woo Kim, Yool-hee Kim, Hyung Seok Lee, Ha Young Shin, Yeon Jung, Min Jung Kim, Nou Ri Lee, MiJu Shin, Farit A Khabirov, Lyudmila Averyanova, Natalya Babicheva, Eugenii Granatov, Sergey Kazarov, Timur Khaybullin, Alexander Rogozhin, Dmitry V Pokhabov, Vladislav Abramov, Anastasia Amelina, Yulia Nesteroca, Tatyana Bozhenkina, Aleksey N Boyko, Elena G Demyan, Inessa Khoroshilova, Mikhail Melnikov, Ekaterina V Popova, Svetlana N Sharanova, Sergey G Shchur, Denis V Sazonov, Larisa Babenko, Elena Bayandina, Asya Yarmoschuk, Victor A Baliazin, Elena Baliazina, Elena Budaeva, Irina Chernikova, Zoya Goncharova, Vladimir Krasnov, Marina Myatleva, Olga V. Rodionova, Iuliana Samulyzhko, Alla A. Timofeeva, Sabas Boyero Duran, Maria Mar Mendibe Bilbao, Irene Diaz Cuervo, Jose Maria Losada Domingo, Amaia Gonzalez Eizaguirre, Jose Eulalio Barcena Llona, Roberto Valverde Moyano, Carmen Bahamonde, Fernando Sanchez Lopez, Raquel Pinar Morales, Eduardo Agüera Morales, Carmen Bahamonde Roman, Juan Jose Ochoa Sepulveda, Maria del Carmen Blanco Valero, Nazaret Pelaez Viña, Cristina Conde Gavilan, Ana Maria Jover Sanchez, Sara Vila Bedmar, Nuria Gonzalez Garcia, Aida Orviz Garcia, Ines Gonzalez-Suarez, Elena Miñano Guillamon, Miguel Kawiorski, Elena Guerra Schulz, Alba Garcia Alonso, Francisco Jesus Lopez Perez, Marta Palacios Sarmiento, Guillermo Izquierdo Ayuso, Guillermo Navarro Mascarell, Cristina Paramo Camino, Asuncion Varas Garcia, Yaiza Montserrat Mendoza, Veronica Ines Vargas Muñoz, Patricia Torres Tonda, Ching-Piao Tsai, Jiu-Haw Yin, Mei-Jung Chen, Shan-Ni Li, Fei-Ti Wang, Suwat Srisuwannanukorn, Thanatat Boonmongkol, Duangporn Borisutbuathip, Duangkamol Singwicha, Krittika Siritanan, Chidchanoke Thearapati, Kwanmuang Sornda, Metha Apiwattanakul, Saharat Aungsumart, Narupat Suanprasert, Kaona Suksuchano, Nittaya Parkinsee, Kongkiat Kulkantrakorn, Praween Lolekha, Artit Potigumjon, Puchit Sukphulloprat, Dararat Suksasunee, Chankawee Komaratat, Sunattana Luangtong, Arkhom Arayawichanont, Phanpaphon Konpan, Nathapol Riablershirun, Thaddao Wiroteurairuang, Panadda Jantaweesirirat, Aslı Kurne, Irem Erkent, Ebru Bekircan Kurt, Ezgi Saylam, Yagmur Caliskan, Gulsah Orsel, Yahya Celik, Canan Celebi, Aslan Tekatas, Tugce Banbal, Gulsen Akman Demir, Burcu Altunrende, Zeliha Matur, Baris Topcular, Tules Elmas, Aysenur Gulo, Selin Ozdemir, Cansu Ozkoklesen, Mahinur Ozturk, Mertkan Yanik, Elif Yildirim, Melih Tutuncu, Ayse Altintas, Abdulsamet Cam, Ayse Deniz Elmali, Sabahattin Saip, Aksel Siva, Uygur Tanrıverdi, Ugur Uygunoglu, Sinem Caliskan, Pinar Gulo, Esra Kozig, Sakine Sakiz, Ihsan Sukru Sengun, Egemen Idiman, Rahmi Tumay Ala, Duygu Arslan, Utku Bulut, Yasemin Karakaptan, Derya Kaya, Zaur Mehdiyev, Bengu Balkan, Berfu Kuku, Mujgan Ozhun, Celal Tuga, Muzeyyen Ugur, Husnu Efendi, Sena Destan Bunul, Hakan Cavus, Yunus Emre Gorke, Ayse Kutlu, Seda Ozturk, Cansu Egilmez Sarikaya, Gulsah Becerikli, Cansu Semiz, Ozlem Tun, Sehriban Ayer, Musa Kazim Onar, Mehlika Berra Ozberk, Sedat Sen, Tugce Kirbas Cavdar, Adife Veske, Cavit Boz, Didem Altiparmak, Cigdem Ozen Aydin, Sibel Gazioglu, Duygu Bekircan, Anu Jacob, Heike Arndt, Liene Elsone, Shahd Hassan Mahmoud Hamid, Daniel Hugh Whittam, Martin Wilson, Imelda O'Brien, Maria Isabel da Silva Leite, Pedro Maria Rodriguez Cruz, Damian Robert Jenkins, George Tackley, Ana Cavey, Rosie Everett, Joy Hodder, Abigail Koelewyn, Ellen Mowry, Walter Royal, Robert Shin, Christopher Bever, Daniel Harrison, Horea Rus, Wei Zheng, Karen Callison, Kerry Naunton, Benjamin M Frishberg, Andrew M Blumenfeld, Andrew Inocelda, Kalyani Korabathina, Michael Lobatz, Melissa M Mortin, Irene J Oh, Jay H Rosenberg, Mark Sadoff, Gregory A Sahagian, Anchi Wang, Yasmin Camberos, Guadalupe Sanchez, Estela Soto, Jacqueline A Nicholas, Aaron Boster, Geoffrey Eubank, Katy Groezinger, Meghan Lauf, Annette F Okai, Rashedul Hasan, Chaouki Khoury, Victoria Stokes, Stacey Clardy, Melissa Cortez, John Greenlee, John Rose, Mateo Paz Soldan, Amanda Emett, Lawanda Esquibel, Lilly Fagatele, Ka-Ho Wong, James C Stevens, Thomas M Banas, Marlene C Bultemeyer, Andrea Haller, Natalie Manalo, Keri Aeschliman, Debi Kocks, Michael Racke, Aaron Lee Boster, Michelle Bowman, Jaime Imitola, Yasushi Kisanuki, Misty Green, Stephanie Scarberry, Sharon G Lynch, Heather S Anderson, Gary S Gronseth, Nancy E Hammond, Yasir N Jassam, Manoj K Mittal, Muhammed M Nashatizadeh, Nicholas Levine, Lisa Schmidt, Jill Sibley, Vonda Whitley, James Winkley, Timothy Coleman, Gregory Cooper, Stephanie Sheffield, Keri Turner, Dana Galloway, Robert S Tillett, Geeta A Ganesh, Brian M Plato, Tad D Seifert, Diana Godwin, Deborah Lockridge, Kottil W Rammohan, William A Sheremata, Silvia Delgado, Jose Gonzalez, Alexis Lizarraga, Janice Maldonado, Melissa Ortega, Leticia Tornes, Yanet Babcock, Osmara Cailam, Yesica Campos, Irlisse Couvertier, Bettina Daneri, Jeremy Deni, Jeffrey Hernandez, Tatiana Jaramillo, Tenita Morris, Daniel Nobel, Anjelis Oliveira, Reshma Richardson, Gloria Rodriguez, Ana Romero, Carlos Sandova, Ruta Sawant, Lissett Tueros, Eric S Zetka, Chao Zheng, Daniel H Jacobs, Constance Easterling, Jennifer Fairbank, Revathi Iyengar, Mark Klafter, Justin Lindquist, Ahmed H Sadek, Elizabeth Carmona Toro, Navin Verma, Brigith Patino Castro, Nadia Sukhraj-Singh, Joseph Berger, Eric Williamson, Salim Chahin, Dina Jacobs, Clyde Markowitz, Jessica Dobbins, Lauren Mace, Maria Martin, Ashley Pinckney, Amber Roberts, Islam Zaydan, Galen W Mitchell, Rock A Heyman, Ryan L Orie, Valerie R Suski, Kerry Oddis, Darlene Punjack, Eoin Flanagan, Avi Gadoth, Andrew McKeon, W Oliver Tobin, Anastasia Zekeridou, Katie Dunlay, Jessica Sagen, Jonathan L Carter, Bachir Estephan, Brent P Goodman, Charlene R Hoffman Snyder, Andrea Francone, Irene Galasky, Martha Thomas, Pavle Repovic, James Bowen, Angeli Mayadev, Peiqing Qian, Yuriko Courtney, Lauren Lennox, Robert Thomas Naismith, Anne Haney Cross, Emily Evans, Erin E Longbrake, Megan E Orchard, Gregory F Wu, Linda Heinrich, Susan Sommers, Faria Amjad, Erika Mitchell, Carlos Mora, Bethany Schreiber, Carlo Tornatore, Alexis Carlson, Sacha McCarthy, and Alexandria Oliver

Subjects
Adult, medicine.medical_specialty, Population, Placebo, Antibodies, Monoclonal, Humanized, 03 medical and health sciences, Complement inhibitor, 0302 clinical medicine, Internal medicine, medicine, Humans, 030212 general & internal medicine, education, Aquaporin 4, education.field_of_study, Neuromyelitis optica, business.industry, Neuromyelitis Optica, General Medicine, Eculizumab, medicine.disease, Comorbidity, ddc, Neurology, Concomitant, Rituximab, Neurology (clinical), business, 030217 neurology & neurosurgery, medicine.drug
Abstract

Background Antibodies to the aquaporin-4 (AQP4) water channel in neuromyelitis optica spectrum disorder (NMOSD) are reported to trigger the complement cascade, which is implicated in astrocyte damage and subsequent neuronal injury. The PREVENT study demonstrated that the terminal complement inhibitor eculizumab reduces adjudicated relapse risk in patients with anti-AQP4 immunoglobulin G-positive (AQP4+) NMOSD. The objective of this analysis was to evaluate the efficacy of eculizumab in reducing relapse risk and its safety in AQP4+ NMOSD across clinically relevant subgroups in PREVENT. Methods In the randomized, double-blind, time-to-event, phase 3 PREVENT trial, 143 adults received eculizumab (maintenance dose, 1200 mg/2 weeks) or placebo (2:1), with stable-dose concomitant immunosuppressive therapy (IST) permitted (except rituximab and mitoxantrone). Post hoc analyses of relapses and adverse events were performed for prespecified and post hoc subgroups based on concomitant IST and prior rituximab use, demographic and disease characteristics, and autoimmune comorbidity. Results The significant reduction in relapse risk observed for eculizumab versus placebo in the overall PREVENT population was consistently maintained across subgroups based on concomitant IST and previous rituximab use, age, sex, region, race, time since clinical onset of NMOSD, historical annualized relapse rate, baseline Expanded Disability Status Scale score, and history of another autoimmune disorder. The serious infection rate was lower with eculizumab than placebo regardless of rituximab use in the previous year, concomitant IST use, or history of another autoimmune disorder. Conclusion Across a wide range of clinically relevant AQP4+ NMOSD patient subgroups in PREVENT, eculizumab therapy was consistently effective versus placebo in reducing relapse risk, with no apparent increase in serious infection rate. Trial registration NCT01892345 (ClinicalTrials.gov).

Published
2020

28. Herpes simplex encephalitis manifested as hemifacial anhidrosis

Author

Young-Soo Kim, Seung Joo Kim, Heejeong Jeong, Dohyung Kim, Tae-Won Yang, Seunguk Jung, Oh-Young Kwon, Eun Bin Cho, Chang Hyo Yoon, and Ki-Jong Park

Subjects
Hypohidrosis, medicine.medical_specialty, Neurology, Endocrine and Autonomic Systems, business.industry, MEDLINE, medicine.disease, Dermatology, medicine, Humans, Neurology (clinical), Encephalitis, Herpes Simplex, Anhidrosis, medicine.symptom, business, Encephalitis
Published
2020

29. Comparison of MOG and AQP4 antibody seroprevalence in Korean adults with inflammatory demyelinating CNS diseases

Author

Jae-Won Hyun, So-Young Huh, Yoon-Ho Hong, Eun Bin Cho, Sa-Yoon Kang, Jong-Hwa Shin, Kyong Jin Shin, Byoung Joon Kim, Sang-Soo Lee, Ye-Seul Kim, Tai-Seung Nam, Suk Geun Han, Hyun Jin Kim, Min-Young Lee, Hyun-June Shin, Byung Jo Kim, Joong-Yang Cho, Hye Lim Lee, Young-Min Lim, Seolhee Baek, Hye Jung Lee, Jong Seok Bae, Seung Woo Kim, Woojun Kim, Sun Young Kim, So Yeon Kim, Jin Myoung Seok, Su-Hyun Kim, Eun Hee Sohn, Woo Kyo Jeong, Ju-Hong Min, Seung-Han Lee, Jeeyoung Oh, Ho Jin Kim, Seong-il Oh, Sun-Young Oh, Jong Kuk Kim, Ha Young Shin, and Ohyun Kwon

Subjects
Adult, Central nervous system, Myelin oligodendrocyte glycoprotein, 03 medical and health sciences, 0302 clinical medicine, immune system diseases, Central Nervous System Diseases, Seroepidemiologic Studies, Republic of Korea, medicine, Seroprevalence, Humans, 030212 general & internal medicine, chemistry.chemical_classification, Aquaporin 4, biology, business.industry, Multiple sclerosis, medicine.disease, Oligodendrocyte, nervous system diseases, medicine.anatomical_structure, nervous system, Neurology, chemistry, Immunology, biology.protein, Myelin-Oligodendrocyte Glycoprotein, sense organs, Neurology (clinical), Antibody, Glycoprotein, business, 030217 neurology & neurosurgery
Abstract

We aimed to compare seroprevalence of anti-myelin oligodendrocyte glycoprotein (MOG) and anti-aquaporin-4 (AQP4) antibodies in Korean adults with inflammatory demyelinating diseases (IDDs) of the central nervous system (CNS), based on a multicenter nationwide database. Sera were analyzed using a live cell–based assay for MOG and AQP4 antibodies. Of 586 Korean adults with IDDs of the CNS, 36 (6.1%) and 185 (31.6%) tested positive for MOG and AQP4 antibodies, respectively. No participant showed double positivity. Seroprevalence of MOG antibodies was about five times lower than that of AQP4 antibodies in a large cohort of Korean adults with IDDs of the CNS.

Published
2020

30. Autonomic Dysfunction in Dizziness Clinic

Author

Eun Bin Cho and Ki-Jong Park

Subjects
03 medical and health sciences, 0302 clinical medicine, business.industry, Medicine, General Medicine, 030204 cardiovascular system & hematology, business, 030217 neurology & neurosurgery
Published
2018
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31. The IL-10-producing regulatory B cells (B10 cells) and regulatory T cell subsets in neuromyelitis optica spectrum disorder

Author

Byoung Joon Kim, Jin Myoung Seok, Hye Jin Cho, Eun-Suk Kang, Ju-Hong Min, and Eun Bin Cho

Subjects
Adult, Male, 0301 basic medicine, Regulatory T cell, T cell, Regulatory B cells, Antigens, CD19, chemical and pharmacologic phenomena, Dermatology, T-Lymphocytes, Regulatory, CD19, 03 medical and health sciences, 0302 clinical medicine, Immune system, medicine, Humans, Lymphocyte Count, B cell, Aquaporin 4, B-Lymphocytes, Regulatory, Neuromyelitis optica, biology, business.industry, Apyrase, Interleukin-17, Neuromyelitis Optica, Remission Induction, General Medicine, Middle Aged, Flow Cytometry, medicine.disease, Interleukin-10, Psychiatry and Mental health, Interleukin 10, 030104 developmental biology, medicine.anatomical_structure, Immunology, biology.protein, Female, Neurology (clinical), Antigens, CD1d, business, Immunosuppressive Agents, 030217 neurology & neurosurgery
Abstract

B cells contribute to the pathogenesis of neuromyelitis optica (NMO) by producing Aquaporin 4-specific autoantibodies (AQP4-ab); on the other hand, there are certain B cells that suppress immune responses by producing regulatory cytokines, such as IL-10. In this study, we investigated the presence of IL-10-producing Breg cells among lymphocyte subsets. Twenty-two seropositive NMO spectrum disorder (NMOSD) patients (29 samples) and 13 healthy controls (HCs) (14 samples) were enrolled. All NMOSD patients have received one or more immunosuppressive drugs. The phenotype and frequency of B cell and T cell subsets in the peripheral blood were measured by flow cytometry. We defined Breg cells as IL-10-producing B (B10) cells, which are CD19+CD39+CD1d+IL-10+. The potential relations were evaluated between specific lymphocyte subsets and AQP4-ab intensity measured by the cell-based indirect immunofluorescence assay. The frequency of B10 cells was higher in patients with NMOSD regardless of the disease status than that in HCs (attack samples; p = 0.009 and remission samples; p

Published
2018
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32. Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis (REGAIN): a phase 3, randomised, double-blind, placebo-controlled, multicentre study

Author

Kazumi Takada, Vladislav Abramov, Seiko Yoshida, Pinar Ozcelik, Carolina Miranda, Jennifer Kane, Kaitlyn McKenna, Natasha Campbell, Sharon P. Nations, Shitiz Kumar Sriwastava, Yuko Fujii, Mayumi Murata, Linda Wagemaekers, Angela Andoin, Mollie Vanderhook, Yoshinori Okubo, Martin Bilsker, Taira Uehara, Vera Bril, Julia Wanschitz, Stanislava Toncrova, Mariela Bettini, Kazumi Futono, Shachie Aranke, Yool-hee Kim, Hiroyuki Murai, Anne Nyrhinen, Vinay Chaudhry, Raffaele Iorio, Takashi Kanda, Brittany Harvey, Francisco Javier Rodriguez de Rivera, Henning Andersen, Marianne de Visser, Miwako Sato, Yasuhiro Maeda, Fabienne Deruelle, Marina Pozo, Adam Hart, Masaki Saitoh, Wladimir Bocca Vieira de Rezende Pinto, Said R. Beydoun, Lindsay Zilliox, Akihiro Mukaino, Cinzia Caserta, Mahi Jasinarachchi, Andrea M. Corse, Nikoletta Papadopoulou, JuYoung Kwon, Fernanda Carrara, Juliet Saba, Masayuki Makamori, Vittorio Frasca, Luciana Souza Duca, Hoo Nam Kang, C. Trebst, Celile Phan, Muzeyyen Ugur, Eduardo Ng, Jonathan McKinnon, Hila Bali Kuperman, David Feder, Judit Matolcsi, Jiri Pitha, Martin Stangel, Kate Beck, Gabriel Paiva, Diego Lopergolo, Katrien De Mey, Hidenori Matsuo, Lucas Eduardo Pazetto, Eugene Lai, Amanda Anderson, Ann D'Hondt, Tetsuya Akiyama, Beverly Fyfe, Bella Gross, Elisabet Arribas-Ibar, Kathy de Koning, Gulmohor Roy, Dmitry Pokhabov, Maria Johanna Keijzers, Nicholas Ventura, Tessa Marburger, John Loor, Ji Eun Lee, Alessandro Filla, Celal Tuga, Stephanie Scala, Rudy Mercelis, Marc H. De Baets, Hisako Kobayashi, Stanislav Vohanka, Ana Paula Macagnan, Ana Carolina Amaral de Andrade, Heike Arndt, Giovanni Antonini, Yumi Yamashita, Gwendal Le Masson, Sonia Garcia, Sarah Verjans, James F. Howard, Zaeem A. Siddiqi, Yuen T. So, Megumi Koga, Exuperio Diez Tejedor, Teresa Costabile, Mihoko Takada Takada, Steve Hopkins, Jonathan S. Katz, Charlene Hafer-Macko, Erica Nogueira Coelho, Hung Youl Seok, Carol Herbert, Yuriko Nagane, Didem Altiparmak, Sachiko Kamakura, Mohammad Sanjak, Caroline Moreau, Jordi Díaz-Manera, Sivakumar Sathasivam, Michael Vytopil, Amelia Evoli, Masakatsu Motomura, Ester Reggio, Guy Van den Abeele, Hélène Zéphir, Asya Yarmoschuk, Jasmine Hewlett, Amy Wilson, Sachie Fukui, Cavit Boz, Iandra Souza, Morgane Gaboreau, Ivana Jurajdova, Sonia Decressac, Yong Seo Koo, Valentina Pegoraro, Seung Min Kim, Benison Keung, Rosana Rocha, Nanna Witting, John Vissing, Elaine Weiner, Ali Malekniazi, Larisa Babenko, Amanda C. Guidon, Gal Maier, Charlotte Smetcoren, Robert M. Pascuzzi, Domenico Marco Bonifati, Yumiko Nakamura, Tamires Cristina Gomes da Silva, Takashi Murahara, Sarah Plevka, Tomoko Tsuda, John C. Kincaid, Arnaud Lacour, Ibrez Bandukwala, Alan R. Berger, Chang Nyoung Lee, Jae-Sung Lim, Vern C. Juel, Tulio E. Bertorini, Valeria Cavalcante Lino, Namie Taichi, Ju-Hong Min, Josep Gamez, Nelly Greenbereg, William S. David, Srikanth Muppidi, Husnu Efendi, Pedro Lopez Ruiz, Baki Dogan, Cansu Semiz, Natalia Julia Palacios, Sharon Downing, Paola Cudia, Daniel Jacobs, Can Ebru Bekircan-Kurt, Takayasu Fukudome, Kristen Roe, Lena Bjarbo, Nicole Kassebaum, Makoto Samukawa, Shizuka Asada, Christina Dheel, Fatima Maqsood, Eun Bi Hwang, Kevin Daniels, Sevim Erdem-Ozdamar, Olivier Stevens, Claudio Mazia, Karan Alcon, Sibel Gazioglu, Keiko Kikutake, Luis Lay, Petra Tilkin, Corrado Angelini, Derrick Blackmore, Kimiaki Utsugisawa, Despoina Charalambous, Tuula Harrison, Kristin Huynh, Huned S. Patwa, Laura Echevarria, Henrique Mohr, Christian Homedes-Pedret, Richard J. Barohn, Byung Jo Kim, Daniel DiCapua, Terry McClain, Debora Dada Martineli Torres, Maria Salvado Figueras, Ana Paula Melo, Riley Snook, Miki Ogawa, Marcelo Annes, Yuka Saito, Isabel Illa, Evanthia Bernitsas, Nicole Smalley, Molly Lindsay, Robert G. Miller, Olga Azrilin, Silvia Bonanno, Evgeniya Kosykh, Marcela Wolfova, Olivier Outteryck, Shirli Toska, Anna Kostera-Pruszczyk, HyeJin Ra, Rup Tandan, Sotirios Papagiannopoulos, Natasha Willlems, Anne Mette Ostergaard Autzen, Meinoshin Okumura, Patrick Vermersch, Sarada Sakamuri, Maria Antonia Alberti Aguilo, Shigemi Shimose, Cynthia Carter, Ira Blount, Lisa Thompson, Maurer Pereira Martins, Richard Nowak, Hyung Seok Lee, Anna Kaminska, Joan Bratton, Nazire Pinar Acar, Junichi Ogasawara, Mohamed Mahdi-Rogers, Teiichiro Mitazaki, Marek Čierny, Craig Donahue, Jaya Trivedi, Neelam Goyal, Gonzalo Vidal, Brandy Quarles, Akiko Kanzaki, Yasuko Ikeda, Tomomi Kobashikawa, Morris Brown, Daisuke Yamamoto, Michel Deneve, Denis Korobko, Beth DiSanzo, Benedikt Schoser, Heidi Boterhoven, Eri Kobayashi, Maoko Shirane, Cristiani Fernanda Butinhao, Eriko Higuchi, Takashi Hayashi, Masanori Takahashi, Anne-Cécile Wielanek-Bachelet, Benjamin Rix Brooks, Emanuela Onesti, Tahseen Mozaffar, Liang Lu, Sevasti Bostantzopoulou, Christophe Vial, Shawn J. Bird, Sandi Mumfrey-Thomas, Julie Khoury, Kara Patrick, Kenichi Tsukita, Yoshiko Sano, Hiroshi Nakazora, David P. Richman, Gavin Brown, Yoon-Ho Hong, Tomohiro Kawamura, Igor Dias Brockhausen, Ye Liu, Acary Souza Bulle Oliveira, Soichiro Funaka, Tomoya Hasuike, Frank Lin, Luis Antonio Querol Gutierrez, Namita Goyal, Elena Pinzan, Michelle Mellion, Silvia Messina, Christopher Lindberg, Csilla Rozsa, J. Chad Hoyle, Yoko Kaneko, Gustavo Duran, Francesco Patti, Arshira Seddigh, Ele Kim Perez, Jayashri Srinivasan, Michael Benatar, Philip Van Damme, Salma Akhter, Daniel Ambrosio, Maria Salvado, Floyd Jones, Mark Sivak, Anneke J. van der Kooi, Karen Callison, Catherine Nigro, Rebekah Garcia, Thomas Arnold, Hideki Arima, Brigid Crabtree, Mary Varghese, Aditya Kumar, Miri Kim, Fanny O'Brien, Naya McKinnon, Lauren Wheeler, Hong Vu, Shunsuke Yoshimura, Masatoshi Omoto, Jeffrey T. Guptill, Maria Gabriele, Francoise Bouhour, Veena Mathew, Ritsu Nakayama, Rosa Hasan, Francesco Saccà, Mohammed Salajegheh, Diana Dimitrova, Alzira Alves de Siqueira Carvalho, Maurizio Inghilleri, George Sachs, Rekha Pillai, Enrico Marano, Monika Konyane, Anh Tran, Seda Aydinlik, Kendrick Henderson, Fumie Meguro, Alexandre Guerreiro, Amaiak Chilingaryan, Tiyonnoh Cash, Jun Kawamata, Julie Steele, Helene Gervais-Bernard, Thomas Harbo, Alejandra Dalila Garcia, Musa Kazim Onar, Sabrina Sacconi, Carlos Casasnovas Pons, Nadezhda Malkova, Denis Sazonov, Mireya Fernandez-Fournier, Karin Fricke, Laurie Gutmann, Amy Saklad, Clara Schommer, Sandra Taber, Fiona Norwood, Tugce Kirbas Cavdar, Monique Miesen, Fernanda Troili, Masanori Watanabe, Ratna Bhavaraju-Sanka, Ted M. Burns, Sari Atula, Faisal Sohail, Barbora Kurkova, Brigitta Szabadosne, Luciana Renata Cubas Volpe, Jane Pedersen, Jing Jing Wang, Masashi Inoue, Antonella Di Pasquale, Megan Kramer, Magda Chmelikova, Mehran Soltani, Tuan Vu, Laura Fionda, Eliz Agopian, Susan Shin, Anthony A. Amato, Lotte Vinge, Hakan Cavus, Gil I. Wolfe, Joan Nye, Delphine Mahieu, Miguel Wilken, Markus Färkkilä, Catherine Faber, Erin Manning, Emiko Tsuda, Rami Massie, Paolo Emilio Alboini, Yasmeen Shabbir, Angela Campanella, Aikaterini Dimitriou, Marcelo Rugiero, Cynthia Bodkin, Gyorgyi Szabo, Sharon Halton, Akshay Shah, Yasuko Maeda, Hans D. Katzberg, Yagmur Caliskan, Jaimin Shah, Katsuhisa Masaki, Valentina Damato, Blanka Andersson, Aline de Cassia Santos, Masahiro Mori, Renato Mantegazza, Misa Shimpo, Joanne Nemeth, Livia Dezsi, Anna De Rosa, Doreen Ho, Julie Moutarde, Efstathia Mitropoulou, Amy Woodall, Angela Micheels, László Vécsei, Byoung Joon Kim, Lisa Smith, Tomihiro Imai, Harpreet Kaur, Lorenzo Maggi, Jane Distad, Anita Mogensen, Ericka Simpson, Anne Cooley, Eliana Reyes, Ha Young Shin, Da Yoon Koh, Stefan Gingele, Susan Strom, Ezgi Yilmaz, Manisha Chopra, Anna Melnikova, Edouard Millois, Ludwig Gutmann, Miriam Freimer, Hirokazu Shinozaki, Heena Olalde, Kerry Naunton, Shunya Nakane, Ihsan Sengun, Dimos-Dimitrios Mitsikostas, Edina Varga, Juha-Pekka Erälinna, Wolfgang Löscher, Jan De Bleecker, Elena Bravver, Ana Lazaro, Eun Bin Cho, Thomas Cochrane, Jonathan Goldstein, Lisa D. Hobson-Webb, Michaela Tyblova, Angela Marsil, J. 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Rosenfeld, Cigdem Ozen Aydin, Shara Holzberg, Hélène Merle, Olga Zapletalova, Kurt-Wolfram Suehs, Robert P. Lisak, Dale J. Lange, Albert Hietala, Sedat Sen, Elena Giacomelli, Akiyuki Uzawa, Tomás Augusto Suriane Fialho, Matteo Garibaldi, Nadia Sattar, Wai-Kuen Leong, Lindsay Kaplan, Tetsuya Kanai, Jaana Eriksson, Akiko Nagaishi, Khema Sharma, Tamar Gibson, Mohamed Kazamel, Yulia Nesterova, Sascha Alvermann, Murat Terzi, Taylor Darnell, Donna Carnes, Victor Balyazin, John T. Kissel, Waqar Waheed, Jana Junkerova, Kimberly Robeson, Nicholas Vlaikidis, Nicholas Silvestri, Fredrik Piehl, Maurício André Gheller Friedrich, Shun Shimohama, Nuria Vidal, Eleni Kasioti, H. James Jones, Michael K. Hehir, Luiz Augusto da Silva, Dave Watling, Leslie Roberts, Casey Faigle, Caroline Hourquin, Olli Oksaranta, Tomomi Imamura, Shin Hisahara, Dennis Jeffery, Marie-Hélène Soriani, M. Kawai, Chieko Yoshikawa, Roseann Keo, Angela Genge, Michelangelo Maestri Tassoni, Milvia Pleitez, Michael H. 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Kwan, Hidekazu Suzuki, Dai Matsuse, Kelly Jia, Ozlem Tun, Lara Katzin, Yasushi Suzuki, Shannon Lucy, Carlo Antozzi, ANS - Neuroinfection & -inflammation, Neurology, Howard, James F, Utsugisawa, Kimiaki, Benatar, Michael, Murai, Hiroyuki, Barohn, Richard J, Illa, Isabel, Jacob, Saiju, Vissing, John, Burns, Ted M, Kissel, John T, Muppidi, Srikanth, Nowak, Richard J, O'Brien, Fanny, Wang, Jing-Jing, Mantegazza, Renato, Mazia, Claudio Gabriel, Wilken, Miguel, Ortea, Carolina, Saba, Juliet, Rugiero, Marcelo, Bettini, Mariela, Vidal, Gonzalo, Garcia, Alejandra Dalila, Lamont, Phillipa, Leong, Wai-Kuen, Boterhoven, Heidi, Fyfe, Beverly, Roberts, Leslie, Jasinarachchi, Mahi, Willlems, Natasha, Wanschitz, Julia, Löscher, Wolfgang, De Bleecker, Jan, Van den Abeele, Guy, de Koning, Kathy, De Mey, Katrien, Mercelis, Rudy, Wagemaekers, Linda, Mahieu, Delphine, Van Damme, Philip, Smetcoren, Charlotte, Stevens, Olivier, Verjans, Sarah, D'Hondt, Ann, Tilkin, Petra, Alves de Siqueira Carvalho, Alzira, 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Michel, Deruelle, Fabienne, Schoser, Benedikt, Wenninger, Stephan, Stangel, Martin, Alvermann, Sascha, Gingele, Stefan, Skripuletz, Thoma, Suehs, Kurt-Wolfram, Trebst, Corinna, Fricke, Karin, Papagiannopoulos, Sotirio, Bostantzopoulou, Sevasti, Vlaikidis, Nichola, Zampaki, Martha, Papadopoulou, Nikoletta, Mitsikostas, Dimos-Dimitrio, Kasioti, Eleni, Mitropoulou, Efstathia, Charalambous, Despoina, Rozsa, Csilla, Horvath, Melinda, Lovas, Gabor, Matolcsi, Judit, Szabo, Gyorgyi, Szabadosne, Brigitta, Vecsei, Laszlo, Dezsi, Livia, Varga, Edina, Konyane, Monika, Gross, Bella, Azrilin, Olga, Greenbereg, Nelly, Bali Kuperman, Hila, Antonini, Giovanni, Garibaldi, Matteo, Morino, Stefania, Troili, Fernanda, Di Pasquale, Antonella, Filla, Alessandro, Costabile, Teresa, Marano, Enrico, Sacca, Francesco, Marsili, Angela, Puorro, Giorgia, Maestri Tassoni, Michelangelo, De Rosa, Anna, Bonanno, Silvia, Antozzi, Carlo, Maggi, Lorenzo, Campanella, Angela, Angelini, Corrado, Cudia, Paola, Pegoraro, 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Edward, Quarles, Brandy, Smalley, Nicole, Amato, Anthony, Cochrane, Thoma, Salajegheh, Mohammed, Roe, Kristen, Amato, Katherine, Toska, Shirli, Wolfe, Gil, Silvestri, Nichola, Patrick, Kara, Zakalik, Karen, Katz, Jonathan, Miller, Robert, Engel, Marguerite, Bravver, Elena, Brooks, Benjamin, Plevka, Sarah, Burdette, Maryanne, Sanjak, Mohammad, Kramer, Megan, Nemeth, Joanne, Schommer, Clara, Juel, Vern, Guptill, Jeffrey, Hobson-Webb, Lisa, Beck, Kate, Carnes, Donna, Loor, John, Anderson, Amanda, Lange, Dale, Agopian, Eliz, Goldstein, Jonathan, Manning, Erin, Kaplan, Lindsay, Holzberg, Shara, Kassebaum, Nicole, Pascuzzi, Robert, Bodkin, Cynthia, Kincaid, John, Snook, Riley, Guinrich, Sandra, Micheels, Angela, Chaudhry, Vinay, Corse, Andrea, Mosmiller, Betsy, Ho, Doreen, Srinivasan, Jayashri, Vytopil, Michael, Ventura, Nichola, Scala, Stephanie, Carter, Cynthia, Donahue, Craig, Herbert, Carol, Weiner, Elaine, Mckinnon, Jonathan, Haar, Laura, Mckinnon, Naya, Alcon, Karan, Daniels, Kevin, Sattar, Nadia, Jeffery, Denni, Mckenna, Kaitlyn, Guidon, Amanda, David, William, Dheel, Christina, Levine-Weinberg, Mark, Nigro, Catherine, Simpson, Ericka, Appel, Stanley H, Lai, Eugene, Lay, Lui, Pleitez, Milvia, Halton, Sharon, Faigle, Casey, Thompson, Lisa, Sivak, Mark, Shin, Susan, Bratton, Joan, Jacobs, Daniel, Brown, Gavin, Bandukwala, Ibrez, Brown, Morri, Kane, Jennifer, Blount, Ira, Freimer, Miriam, Hoyle, J. Chad, Agriesti, Julie, Khoury, Julie, Marburger, Tessa, Kaur, Harpreet, Dimitrova, Diana, Mellion, Michelle, Sachs, George, Crabtree, Brigid, Keo, Roseann, Perez, Ele Kim, Taber, Sandra, Gilchrist, Jame, Andoin, Angela, Darnell, Taylor, Goyal, Neelam, Sakamuri, Sarada, So, Yuen T, Welsh, Lesly Welsh, Bhavaraju-Sanka, Ratna, Tobon Gonzalez, Alejandro, Jones, Floyd, Saklad, Amy, Nations, Sharon, Trivedi, Jaya, Hopkins, Steve, Kazamel, Mohamed, Alsharabati, Mohammad, Lu, Liang, Mumfrey-Thomas, Sandi, Woodall, Amy, Richman, David, Butters, Janelle, Lindsay, Molly, Mozaffar, Tahseen, Cash, Tiyonnoh, Goyal, Namita, Roy, Gulmohor, Mathew, Veena, Maqsood, Fatima, Minton, Brian, Jones, H. Jame, Rosenfeld, Jeffrey, Garcia, Rebekah, Garcia, Sonia, Echevarria, Laura, Pulley, Michael, Aranke, Shachie, Berger, Alan Ro, Shah, Jaimin, Shabbir, Yasmeen, Smith, Lisa, Varghese, Mary, Gutmann, Laurie, Gutmann, Ludwig, Swenson, Andrea, Olalde, Heena, Hafer-Macko, Charlene, Kwan, Justin, Zilliox, Lindsay, Callison, Karen, Disanzo, Beth, Naunton, Kerry, Bilsker, Martin, Sharma, Khema, Reyes, Eliana, Cooley, Anne, Michon, Sara-Claude, Steele, Julie, Karam, Chafic Karam, Chopra, Manisha, Bird, Shawn, Kaufman, Jacob, Gallatti, Nichole, Vu, Tuan, Katzin, Lara, Mcclain, Terry, Harvey, Brittany, Hart, Adam, Huynh, Kristin, Beydoun, Said, Chilingaryan, Amaiak, Droker, Brian, Lin, Frank, Shah, Akshay, Tran, Anh, Akhter, Salma, Malekniazi, Ali, Tandan, Rup, Hehir, Michael, Waheed, Waqar, Lucy, Shannon, Weiss, Michael, Distad, Jane, Downing, Sharon, Strom, Susan, Lisak, Robert, Bernitsas, Evanthia, Khan, Omar, Kumar Sriwastava, Shitiz, Tselis, Alexandro, Jia, Kelly, Bertorini, Tulio, Arnold, Thoma, Henderson, Kendrick, Pillai, Rekha, Liu, Ye, Wheeler, Lauren, Hewlett, Jasmine, Vanderhook, Mollie, Dicapua, Daniel, Keung, Benison, Kumar, Aditya, Patwa, Huned, Robeson, Kimberly, Nye, Joan, Vu, Hong, Howard, J, Utsugisawa, K, Benatar, M, Murai, H, Barohn, R, Illa, I, Jacob, S, Vissing, J, Burns, T, Kissel, J, Muppidi, S, Nowak, R, O'Brien, F, Wang, J, Mantegazza, R, and Bonanno, S

Subjects
Male, 0301 basic medicine, medicine.medical_treatment, Drug Resistance, Adult, Aged, Antibodies, Monoclonal, Humanized, Autoantibodies, Double-Blind Method, Female, Humans, Middle Aged, Myasthenia Gravis, Receptors, Cholinergic, Outcome Assessment (Health Care), Severity of Illness Index, Neurology (clinical), law.invention, Complement inhibitor, 0302 clinical medicine, Randomized controlled trial, law, Monoclonal, Receptors, Clinical endpoint, Humanized, Cholinergic, education.field_of_study, Eculizumab, Autoantibodie, Myasthenia Gravi, Settore MED/26 - NEUROLOGIA, Human, medicine.drug, Meningitides, medicine.medical_specialty, Population, Placebo, Antibodies, 03 medical and health sciences, Internal medicine, medicine, education, business.industry, Surgery, Thymectomy, 030104 developmental biology, business, 030217 neurology & neurosurgery
Abstract

Background Complement is likely to have a role in refractory generalised myasthenia gravis, but no approved therapies specifically target this system. Results from a phase 2 study suggested that eculizumab, a terminal complement inhibitor, produced clinically meaningful improvements in patients with anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis. We further assessed the efficacy and safety of eculizumab in this patient population in a phase 3 trial. Methods We did a phase 3, randomised, double-blind, placebo-controlled, multicentre study (REGAIN) in 76 hospitals and specialised clinics in 17 countries across North America, Latin America, Europe, and Asia. Eligible patients were aged at least 18 years, with a Myasthenia Gravis-Activities of Daily Living (MG-ADL) score of 6 or more, Myasthenia Gravis Foundation of America (MGFA) class II-IV disease, vaccination against Neisseria meningitides, and previous treatment with at least two immunosuppressive therapies or one immunosuppressive therapy and chronic intravenous immunoglobulin or plasma exchange for 12 months without symptom control. Patients with a history of thymoma or thymic neoplasms, thymectomy within 12 months before screening, or use of intravenous immunoglobulin or plasma exchange within 4 weeks before randomisation, or rituximab within 6 months before screening, were excluded. We randomly assigned participants (1:1) to either intravenous eculizumab or intravenous matched placebo for 26 weeks. Dosing for eculizumab was 900 mg on day 1 and at weeks 1, 2, and 3; 1200 mg at week 4; and 1200 mg given every second week thereafter as maintenance dosing. Randomisation was done centrally with an interactive voice or web-response system with patients stratified to one of four groups based on MGFA disease classification. Where possible, patients were maintained on existing myasthenia gravis therapies and rescue medication was allowed at the study physician's discretion. Patients, investigators, staff, and outcome assessors were masked to treatment assignment. The primary efficacy endpoint was the change from baseline to week 26 in MG-ADL total score measured by worst-rank ANCOVA. The efficacy population set was defined as all patients randomly assigned to treatment groups who received at least one dose of study drug, had a valid baseline MG-ADL assessment, and at least one post-baseline MG-ADL assessment. The safety analyses included all randomly assigned patients who received eculizumab or placebo. This trial is registered with ClinicalTrials.gov, number NCT01997229. Findings Between April 30, 2014, and Feb 19, 2016, we randomly assigned and treated 125 patients, 62 with eculizumab and 63 with placebo. The primary analysis showed no significant difference between eculizumab and placebo (least-squares mean rank 56·6 [SEM 4·5] vs 68·3 [4·5]; rank-based treatment difference -11·7, 95% CI -24·3 to 0·96; p=0·0698). No deaths or cases of meningococcal infection occurred during the study. The most common adverse events in both groups were headache and upper respiratory tract infection (ten [16%] for both events in the eculizumab group and 12 [19%] for both in the placebo group). Myasthenia gravis exacerbations were reported by six (10%) patients in the eculizumab group and 15 (24%) in the placebo group. Six (10%) patients in the eculizumab group and 12 (19%) in the placebo group required rescue therapy. Interpretation The change in the MG-ADL score was not statistically significant between eculizumab and placebo, as measured by the worst-rank analysis. Eculizumab was well tolerated. The use of a worst-rank analytical approach proved to be an important limitation of this study since the secondary and sensitivity analyses results were inconsistent with the primary endpoint result; further research into the role of complement is needed. Funding Alexion Pharmaceuticals.

Published
2017
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33. Physician- and self-assessed myasthenia gravis activities of daily living score

Author

Eun Bin Cho, Jin Myoung Seok, Hye Jin Cho, Byoung Joon Kim, Ju-Hong Min, Hye Lim Lee, and Yong-Dae Kim

Subjects
medicine.medical_specialty, Activities of daily living, Physiology, Intraclass correlation, business.industry, Mg composite, medicine.disease, Spearman's rank correlation coefficient, Myasthenia gravis, 030218 nuclear medicine & medical imaging, Correlation, 03 medical and health sciences, Cellular and Molecular Neuroscience, 0302 clinical medicine, Cronbach's alpha, Physiology (medical), Physical therapy, medicine, Daily living, Neurology (clinical), business, 030217 neurology & neurosurgery
Abstract

INTRODUCTION The Myasthenia Gravis-Activities of Daily Living (MG-ADL) profile scale is a simple-to-use instrument. We aimed to validate this scale in the Korean language and compare physician- and self-assessed MG-ADL scores (pMG-ADL-K and sMG-ADL-K). METHODS pMG-ADL-K and sMG-ADL-K and MG Composite (MGC) scores were obtained from patients. The correlation between pMG-ADL-K and MGC and the relationship between the pMG-ADL-K and sMG-ADL-K were assessed using the Cronbach α and the Spearman coefficient. By intraclass correlation coefficient (ICC), the reliability of each sub-item of pMG-ADL-K and sMG-ADL-K was evaluated. RESULTS We included data from 40 patients. The pMG-ADL-K score showed a strong correlation with the MGC score (rho = 0.80, P

Published
2017
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34. A multicenter prospective observational study on the safety and efficacy of tacrolimus in patients with myasthenia gravis

Author

Je Young Shin, Jung Im Seok, Ju Hong Min, Young-Eun Park, Hung Youl Seok, Byung Jo Kim, Eun Bin Cho, Yang Ki Minn, In Soo Joo, Tai-Seung Nam, Jeeyoung Oh, Bum Chun Suh, Byoung Joon Kim, Jung-Joon Sung, Yoon-Ho Hong, Suk Won Ahn, Jin Myoung Seok, Sa Youn Kang, Sun-Young Oh, Jin-Sung Park, and Ha Young Shin

Subjects
Male, 0301 basic medicine, medicine.medical_specialty, Time Factors, Prednisolone, chemical and pharmacologic phenomena, Mg composite, Tacrolimus, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Myasthenia Gravis, medicine, Humans, In patient, Prospective Studies, Drug reaction, business.industry, Middle Aged, medicine.disease, Myasthenia gravis, surgical procedures, operative, 030104 developmental biology, Neurology, Anesthesia, Drug Therapy, Combination, Female, Observational study, Neurology (clinical), business, Immunosuppressive Agents, 030217 neurology & neurosurgery, medicine.drug
Abstract

Introduction Several clinical studies using tacrolimus revealed reasonable therapeutic mechanisms and efficacy in patients with myasthenia gravis (MG). However, long-period studies in a large number of patients with MG are limited; therefore, the aim of this study was to investigate the therapeutic efficacies and safety of tacrolimus in patients with MG during a 12-month follow-up period. Methods Tacrolimus was administered to 150 patients with MG who were recruited based on the inclusion criteria. Fifteen medical centers in Korea participated in this study. The efficacy of tacrolimus was assessed using MG composite scales (MGCS) and the prednisolone-sparing effect. And the adverse drug reactions (ADRs) of tacrolimus were monitored in each patient from the beginning of tacrolimus treatment to the end of the follow-up period. Results After starting tacrolimus, the 32 patients were affected by ADRs, and consequentially 134 patients of the enrolled patients were followed up for 12 months. They showed that the mean prednisolone dosage significantly decreased (6.1 ± 7.6 mg/day), compared to that in the baseline (11.3 ± 9.5 mg/day), and MGCS significantly improved after 12 months of tacrolimus treatment, compared to that at the baseline. Conclusions Our study showed that tacrolimus would be an effective immunosuppressant as an initial therapeutic agent in patients with MG; in addition, it showed tolerable safety profiles during the 12-month follow-up evaluation.

Published
2017
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35. Low high-density lipoprotein cholesterol and high triglycerides lipid profile in neuromyelitis optica spectrum disorder: Associations with disease activity and disability

Author

Misong Choi, Byoung Joon Kim, Ju-Hong Min, Eun Bin Cho, Jin Myoung Seok, Hye-Jin Cho, and Hee Young Shin

Subjects
Adult, Male, medicine.medical_specialty, Inflammation, Severity of Illness Index, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, High-density lipoprotein, Internal medicine, medicine, Humans, 030212 general & internal medicine, Triglycerides, Aquaporin 4, Neuromyelitis optica, Triglyceride, medicine.diagnostic_test, business.industry, Multiple sclerosis, Cholesterol, HDL, Neuromyelitis Optica, Lipid metabolism, General Medicine, Middle Aged, medicine.disease, Magnetic Resonance Imaging, Endocrinology, Neurology, chemistry, lipids (amino acids, peptides, and proteins), Female, Neurology (clinical), medicine.symptom, business, Lipid profile, 030217 neurology & neurosurgery, Dyslipidemia
Abstract

Altered lipid metabolism is a feature of systemic autoimmune diseases. Dyslipidemia is associated with the disease activity and progression in patients with multiple sclerosis. However, in neuromyelitis optica spectrum disorder (NMOSD), changes in the lipid profile and the associations between specific lipid levels and disease activity/disability are unknown.Serum samples (N = 148) were collected from 53 patients with aquaporin-4 (AQP4)-positive NMOSD when they were not treated with lipid lowering agents. Fasting lipid (total cholesterol, triglyceride [TG], high-density lipoprotein cholesterol [HDL-C], low-density lipoprotein cholesterol) levels were compared between 39 patients with NMOSD, not taking steroids, and 142 age-, sex-, and body mass index-matched healthy controls. In addition, we analyzed the differences in the lipid profile between attack and remission samples and the associations between lipid profiles and clinical outcome in all 148 samples from 53 patients. The generalized estimating equation was used.Patients with NMOSD showed lower HDL-C and higher TG levels compared to healthy controls (p = 0.017 and p 0.001, respectively). HDL-C level was significantly lower during attack than remission (β = -7.851; p = 0.035), and TG level had positive correlation with EDSS scores (β = 0.014; p = 0.002) regardless of disease activity status. However, enhanced lesions on magnetic resonance imaging were not associated with lipid profiles.Dyslipidemia with low HDL-C and high TG correlated disease activity and disability in AQP4-positive NMOSD. It remains to be elucidated whether altered lipid metabolism contributes to deleterious immune response, possibly through inflammation, or is secondary to neurological disability in NMOSD.

Published
2020

36. Low T3 syndrome in neuromyelitis optica spectrum disorder: Associations with disease activity and disability

Author

Ju-Hong Min, Kwang Ho Lee, Jin Myoung Seok, Byoung Joon Kim, Eun Bin Cho, Hye Jin Cho, Hee Young Shin, and Hye Lim Lee

Subjects
Adult, Male, 0301 basic medicine, Thyroid Hormones, endocrine system, medicine.medical_specialty, Adolescent, CD3 Complex, endocrine system diseases, Thyroid Gland, medicine.disease_cause, Gastroenterology, Serology, Autoimmunity, Disability Evaluation, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Prevalence, Humans, Medicine, Spectrum disorder, Aged, Autoantibodies, Retrospective Studies, Subclinical infection, Aged, 80 and over, Aquaporin 4, Neuromyelitis optica, biology, business.industry, Neuromyelitis Optica, Low T3 Syndrome, Middle Aged, medicine.disease, Euthyroid Sick Syndromes, Anti-thyroid autoantibodies, 030104 developmental biology, Neurology, Immunology, biology.protein, Female, Neurology (clinical), Antibody, business, Biomarkers, 030217 neurology & neurosurgery
Abstract

Neuromyelitis optica (NMO) sometimes coexists with serological marker-positive, non-organ-specific autoimmune disorders. We evaluated the prevalence of thyroid dysfunction and anti-thyroid antibodies in patients with NMO spectrum disorder (NMOSD) and investigated the associations between thyroid dysfunction/autoimmunity and clinical features of NMOSD.Forty-nine NMOSD patients with anti-aquaporin-4 antibody and 392 age- and sex-matched healthy controls were included. We measured the levels of thyroid hormones and anti-thyroid antibodies.The prevalence of clinical hypothyroidism, subclinical hyperthyroidism, and low T3 syndrome were higher in patients with NMOSD (4.1%, 12.2%, and 20.4%, respectively) compared with healthy controls (0.3%, 2.8%, and 0.5%, respectively; p=0.034, p=0.001, and p0.001, respectively). However, anti-thyroperoxidase antibody (anti-TPO)-positivity did not significantly differ between NMOSD patients (20.4%) and controls (11.5%). Low T3 syndrome was more prevalent among patients during an attack (N=10/19, 52.6%) than those in remission (N=1/30, 3.3%). In addition, patients with low T 3 syndrome had significantly higher EDSS scores at the last visits as well as at sampling compared to those without low T3 syndrome. T3 levels were inversely correlated with EDSS score at the last visit after adjustment for age, sex, disease duration, clinical status (attack vs. remission), oral prednisolone use, iv methylprednisolone use, other immunosuppressive agents use, and the location of lesion (ρ=-0.416, p=0.010).Our study suggests that thyroid dysfunction is frequent in patients with NMOSD; particularly, serum T3 levels may be a useful indicator of disease activity and disability in NMOSD.

Published
2016
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37. Clinical characteristics of disabling attacks at onset in patients with neuromyelitis optica spectrum disorder

Author

Byoung Joon Kim, Hye Lim Lee, Eun Bin Cho, Kwang Ho Lee, Hye Jin Cho, Jin Myoung Seok, and Ju-Hong Min

Subjects
Adult, Male, 0301 basic medicine, medicine.medical_specialty, Pediatrics, Adolescent, Myelitis, Kaplan-Meier Estimate, Antibodies, Disability Evaluation, 03 medical and health sciences, 0302 clinical medicine, Physical medicine and rehabilitation, Severe visual impairment, medicine, Humans, Disabled Persons, Optic neuritis, In patient, Spectrum disorder, Retrospective Studies, Aquaporin 4, Neuromyelitis optica, Neuromyelitis Optica, Retrospective cohort study, Odds ratio, Middle Aged, medicine.disease, 030104 developmental biology, Neurology, Female, Neurology (clinical), Psychology, Immunosuppressive Agents, 030217 neurology & neurosurgery, Follow-Up Studies
Abstract

Background Individual attacks of neuromyelitis optica (NMO) are generally severe enough to cause disability even after the onset attack. We aimed to elucidate the clinical characteristics of disabling attacks at the onset of NMO. Methods We investigated the clinical characteristics at onset and at first relapse in patients with NMO or NMO spectrum disorder with seropositive for the anti-aquaporin-4 antibody. A disabling attack at onset (DAO) was defined as an onset attack in which, at best recovery (allowing up to one year), patients were unable to walk without assistance or were left functionally blind in at least one affected eye. Results Fifty-seven patients were enrolled (53 females; onset age, 41.9±14.8years). Ten patients (17.5%) had a DAO; four had become unable to walk without assistance following myelitis, and six had severe visual impairment following optic neuritis despite rescue treatments. Attack severity at nadir was the only clinical factor predicting a DAO (odds ratio, 2.120; 95% CI, 1.162–3.869; P =0.014). The use of immunosuppressants delayed the interval to the first relapse ( P =0.003). Conclusion Our study showed characteristics of NMO onset attacks that caused severe disability. However, no clinically modifiable factors predicted disabling attacks, except attack severity.

Published
2016
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38. The First Korean Family With Hereditary Gelsolin Amyloidosis Caused by p.D214Y Mutation in the GSN Gene

Author

Eun Bin Cho, Kyoung Jin Park, Byoung Joon Kim, Jong-Ho Park, Jong-Won Kim, and J. H. Park

Subjects
Male, 0301 basic medicine, Heterozygote, Pathology, medicine.medical_specialty, Genotype, DNA Mutational Analysis, Clinical Biochemistry, Case Report, Disease, Biology, Polymorphism, Single Nucleotide, 03 medical and health sciences, 0302 clinical medicine, Asian People, Republic of Korea, medicine, Humans, Hereditary gelsolin amyloidosis, Gelsolin, Base Sequence, Clinical pathology, Hereditary gelsolin amyloidosis (HGA), Biochemistry (medical), Dystrophy, Whole-exome sequencing (WES), General Medicine, Middle Aged, medicine.disease, Facial nerve, Neuropathy, Pedigree, 3. Good health, 030104 developmental biology, Peripheral neuropathy, Diagnostic Genetics, Amyloidosis, Familial, 030217 neurology & neurosurgery, Cutis laxa
Abstract

Hereditary gelsolin amyloidosis (HGA) is an autosomal dominant hereditary disease characterized by corneal lattice dystrophy, peripheral neuropathy, and cutis laxa. So far, no Korean patients with HGA have been reported. A 58-yr-old man presented with involuntary facial twitching, progressive bilateral facial weakness, and tongue atrophy. His mother, maternal uncle, two sisters, and son suffered from the same symptoms. Electrophysiological studies revealed signs of chronic denervation in the cervical and lumbar regions, mild sympathetic autonomic dysfunction, and bilateral facial nerve dysfunction. Diagnostic whole-exome sequencing (WES) revealed a p.D214Y heterozygous mutation in the gelsolin gene in affected members. We present the first report of a Korean family with HGA diagnosed by WES. WES facilitated a clinical diagnosis of HGA in patients with undiagnosed neuropathies.

Published
2016
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39. 'Sirim' (Cold) Pain as a Common Symptom in Korean Patients with Clinically Suspected Small-Fiber Neuropathy

Author

Ju Hong Min, Jin Myoung Seok, Eun Bin Cho, Ki-Jong Park, Bum Chun Suh, and Byoung Joon Kim

Subjects
medicine.medical_specialty, small fiber neuropathy, sensory, 03 medical and health sciences, 0302 clinical medicine, Quality of life, Rating scale, Internal medicine, medicine, pain, 030212 general & internal medicine, Small Fiber Neuropathy, Stabbing Pain, business.industry, autonomic, Odds ratio, cold, Sudomotor, Neurology, quality of life, Neuropathic pain, Cold pain, Original Article, Neurology (clinical), business, 030217 neurology & neurosurgery
Abstract

Background and purpose Diagnosing small-fiber neuropathy (SFN) is challenging because there is no gold-standard test and few diagnostic tests. This study investigated the clinical symptom profile and its associations with the results of quantitative sensory testing (QST) and the quantitative sudomotor axon reflex test (QSART) as well as the quality of life (QOL) in patients with clinically suspected SFN. Methods This study involved 63 patients with clinically suspected length-dependent SFN. Assessments were performed using QST, QSART, SFN Symptoms Inventory Questionnaire, Neuropathic Pain Symptom Inventory, 'Sirim' frequency and 'Sirim' (cold) pain severity, and 36-item Short-Form Health Survey. Multiple logistic and linear regression analyses were performed to predict risk factors for QST or QSART abnormalities and QOL, respectively. Results 'Sirim' and 'Sirim' pain was the most-common (84%) and the most-severe complaint (mean score of 6.3 on a numerical rating scale ranging from 0 to 10) in patients with clinically suspected SFN. The findings of QST [cold detection threshold (CDT)] and QSART were abnormal in 71% (n=45/57) and 62% (n=39/56) of the patients, respectively. An abnormal CDT was correlated with more-severe stabbing pain (odds ratio=2.23, 95% CI=1.02-4.87, p=0.045). Restless-leg symptoms (β=-7.077) and pressure-evoked pain (β=-5.034) were independent predictors of the physical aspects of QOL. Conclusions 'Sirim' pain, similar to cold pain, should be considered a major neuropathic pain in SFN. Among pain characteristics, stabbing pain of a spontaneous paroxysmal nature may be more pronounced in the setting of dysfunctional Aδ fibers with functional autonomic C fibers.

Published
2018

40. Usefulness of phrenic latency and forced vital capacity in patients with ALS with latent respiratory dysfunction

Author

Byoung Joon Kim, Eun Bin Cho, Soonwook Kwon, Jin Myoung Seok, Hye-Jin Cho, Min-Ji Kim, Byung-Euk Joo, and Ju-Hong Min

Subjects
Male, Vital capacity, Diaphragm, Vital Capacity, Neural Conduction, Action Potentials, Pulmonary function testing, FEV1/FVC ratio, Forced Expiratory Volume, Physiology (medical), Reaction Time, medicine, Humans, Respiratory function, Prospective Studies, Amyotrophic lateral sclerosis, Lung, Aged, Phrenic nerve, medicine.diagnostic_test, business.industry, Amyotrophic Lateral Sclerosis, Middle Aged, respiratory system, medicine.disease, Sensory Systems, Respiratory Function Tests, respiratory tract diseases, Compound muscle action potential, Phrenic Nerve, ROC Curve, Neurology, Case-Control Studies, Anesthesia, Nerve conduction study, Female, Neurology (clinical), business, circulatory and respiratory physiology
Abstract

The pulmonary function test (PFT) is a non-invasive and easily available technique to assess respiratory function in patients with amyotrophic lateral sclerosis (ALS); however, patients with dyspnea sometimes show normal PFT findings. Herein, we investigated whether phrenic nerve conduction study (NCS) and PFT are useful to evaluate respiratory function of patients with ALS with normal value ranges in the PFT.We prospectively enrolled 34 patients with definite or probable ALS, who showed FVC (%) ⩾80 of predicted and 78 healthy subjects. PFT and phrenic NCS were performed with the measurement of forced vital capacity (FVC, %), forced expiratory volumes in 1s (FEV1, %), FEV1/FCV ratio (%), and phrenic compound muscle action potential amplitude, and latency.Compared to healthy controls, ALS patients showed delayed phrenic nerve latency and the decrease of FVC (%) (p=0.006 and p0.0001, respectively). ROC curve analysis demonstrated that phrenic latency (AUC=0.7655) and FVC (%) (AUC=0.8239) discriminated ALS patients from healthy subjects.We demonstrated that ALS patients had early respiratory dysfunction, despite normal PFT findings.Phrenic latency and FVC (%) can be helpful to discriminate ALS patients with latent respiratory dysfunction from healthy subjects.

Published
2015
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41. Decreased hemoglobin levels, cerebral small-vessel disease, and cortical atrophy: among cognitively normal elderly women and men

Author

Tae Ok Son, Eun Bin Cho, Mira Kang, Hojeong Kim, Duk L. Na, Na-Yeon Jung, Yeo Jin Kim, Chang Hyung Hong, Hyemin Jang, Jin-Ju Yang, Hanna Cho, Sang Eon Park, Hee Young Shin, Na Kyung Lee, Eun Young Jang, Byoung Seok Ye, Jeong Min Lee, Hee Jin Kim, Jung Won Hwang, Sang Won Seo, and Jongmin Lee

Subjects
Male, 0301 basic medicine, medicine.medical_specialty, Pathology, Anemia, Hemoglobins, 03 medical and health sciences, Cognition, 0302 clinical medicine, Neuroimaging, Internal medicine, Republic of Korea, medicine, Humans, Dementia, Cognitive decline, Aged, Cerebral Cortex, Brain Diseases, medicine.diagnostic_test, Confounding, Magnetic resonance imaging, Middle Aged, medicine.disease, Magnetic Resonance Imaging, Healthy Volunteers, Confidence interval, Hyperintensity, Psychiatry and Mental health, Clinical Psychology, 030104 developmental biology, Multivariate Analysis, Cardiology, Regression Analysis, Female, Atrophy, Geriatrics and Gerontology, Psychology, Gerontology, 030217 neurology & neurosurgery
Abstract

Background:Decreased hemoglobin levels increase the risk of developing dementia among the elderly. However, the underlying mechanisms that link decreased hemoglobin levels to incident dementia still remain unclear, possibly due to the fact that few studies have reported on the relationship between low hemoglobin levels and neuroimaging markers. We, therefore, investigated the relationships between decreased hemoglobin levels, cerebral small-vessel disease (CSVD), and cortical atrophy in cognitively healthy women and men.Methods:Cognitively normal women (n = 1,022) and men (n = 1,018) who underwent medical check-ups and magnetic resonance imaging (MRI) were enrolled at a health promotion center. We measured hemoglobin levels, white matter hyperintensities (WMH) scales, lacunes, and microbleeds. Cortical thickness was automatically measured using surface based methods. Multivariate regression analyses were performed after controlling for possible confounders.Results:Decreased hemoglobin levels were not associated with the presence of WMH, lacunes, or microbleeds in women and men. Among women, decreased hemoglobin levels were associated with decreased cortical thickness in the frontal (Estimates, 95% confidence interval, −0.007, (−0.013, −0.001)), temporal (−0.010, (−0.018, −0.002)), parietal (−0.009, (−0.015, −0.003)), and occipital regions (−0.011, (−0.019, −0.003)). Among men, however, no associations were observed between hemoglobin levels and cortical thickness.Conclusion:Our findings suggested that decreased hemoglobin levels affected cortical atrophy, but not increased CSVD, among women, although the association is modest. Given the paucity of modifiable risk factors for age-related cognitive decline, our results have important public health implications.

Published
2015
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42. Clinical Characteristics and Treatment Response of Peripheral Neuropathy in the Presence of Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Experience at a Single Tertiary Center

Author

Yeon Lim Seo, Sehyo Yune, Hye Jin Cho, Jin Myoung Seok, Byung Jae Lee, Dong Chull Choi, Ju Hong Min, Eun Bin Cho, and Byoung Joon Kim

Subjects
Pathology, medicine.medical_specialty, peripheral neuropathy, Combination therapy, Cyclophosphamide, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Eosinophilic, medicine, Eosinophilia, Asthma, 030203 arthritis & rheumatology, business.industry, medicine.disease, eosinophilic granulomatosis with polyangiitis, Peripheral neuropathy, Neurology, Original Article, cyclophosphamide, Neurology (clinical), prognosis, medicine.symptom, Granulomatosis with polyangiitis, Vasculitis, business, 030217 neurology & neurosurgery, medicine.drug
Abstract

BACKGROUND AND PURPOSE Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic small-vessel vasculitis accompanied by asthma, eosinophilia, and eosinophilic inflammation of various tissues including the peripheral nerves. This study investigated the clinical course and long-term outcomes of peripheral neuropathy in patients with EGPA. METHODS Seventy-one patients with physician-diagnosed EGPA were identified at Samsung Medical Center between January 1995 and April 2014. Sixty-one of these patients were followed-up for more than 1 year and received corticosteroid therapy with or without intravenous cyclophosphamide pulse therapy for 6 to 18 months. Medical records of the 61 patients including demographic data, clinical features, laboratory and pathological findings, treatments, and outcomes were reviewed. RESULTS Peripheral neuropathy as a manifestation of EGPA was present in 46 (75%) of the 61 patients. The mean follow-up duration of the patients with neuropathy was 6.4 years (range 1.2-18.8 years). The scores on the neurological functional disability scale before and after the combination treatment with corticosteroid and cyclophosphamide were 2.43±0.86 and 0.54±0.95 (mean±SD; p

Published
2017

43. Clinical characteristics of late-onset neuromyelitis optica spectrum disorder: A multicenter retrospective study in Korea

Author

So Young Huh, Eun Bin Cho, Byoung Joon Kim, Jin Myoung Seok, Sun Young Kim, Hye Jin Cho, Kwang Ho Lee, Byung Jo Kim, In Soo Joo, Jong Kuk Kim, Ha Young Shin, Ju Hong Min, Suk Won Ahn, Min Su Park, Joong Yang Cho, and Ohyun Kwon

Subjects
0301 basic medicine, Adult, Male, Pediatrics, medicine.medical_specialty, Late onset, Severity of Illness Index, 03 medical and health sciences, 0302 clinical medicine, Republic of Korea, Medicine, Humans, Spectrum disorder, Age of Onset, Autoantibodies, Retrospective Studies, Aquaporin 4, Neuromyelitis optica, medicine.diagnostic_test, business.industry, Multiple sclerosis, Neuromyelitis Optica, Magnetic resonance imaging, Retrospective cohort study, Middle Aged, medicine.disease, Magnetic Resonance Imaging, 030104 developmental biology, Neurology, Optic nerve, Female, Neurology (clinical), Age of onset, business, 030217 neurology & neurosurgery, Follow-Up Studies
Abstract

Background: There are currently few studies regarding late-onset neuromyelitis optica spectrum disorder (LO-NMOSD). Objective: We aimed to describe the characteristic features of patients with LO-NMOSD in Korea. Methods: Anti-aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder (NMOSD) from nine tertiary hospitals were reviewed retrospectively. The patients were divided into two groups based on age of onset: LO-NMOSD (⩾50 years of age at onset) versus early-onset neuromyelitis optica spectrum disorder (EO-NMOSD) (Results: Among a total of 147 patients (125 female; age of onset, 39.4 ± 15.2 years), 45 patients (30.6%) had an age of onset of more than 50 years. Compared to patients with EO-NMOSD, patients with LO-NMOSD had more frequent isolated spinal cord involvement at onset (64.4% vs 37.2%, p = 0.002), less frequent involvement of the optic nerve (40.0% vs 67.7%, p = 0.002), and less frequent brain MRI lesions (31.1% vs 50.0%, p = 0.034). Furthermore, there was a significant positive correlation between age of onset and Expanded Disability Status Scale (EDSS) score at last follow-up ( r = 0.246, p = 0.003). Conclusion: Age of onset could be an important predictor of lesion location and clinical course of patients with NMOSD.

Published
2017

44. Fatigue in patients with neuromyelitis optica spectrum disorder and its impact on quality of life

Author

Ju-Hong Min, Pamela Song, Hye Lim Lee, Eun Yeon Joo, Byoung Joon Kim, Kwang Ho Lee, Jin Myoung Seok, Misong Choi, and Eun Bin Cho

Subjects
Male, Central Nervous System, Physiology, lcsh:Medicine, Disease, Pathology and Laboratory Medicine, Severity of Illness Index, Nervous System, Material Fatigue, Disability Evaluation, 0302 clinical medicine, Quality of life, Materials Physics, Infectious Diseases of the Nervous System, Medicine and Health Sciences, Spectrum disorder, 030212 general & internal medicine, Prospective Studies, Registries, Prospective cohort study, lcsh:Science, Depression (differential diagnoses), Fatigue, Pain Measurement, Multidisciplinary, Depression, Physics, Neuromyelitis Optica, Classical Mechanics, Middle Aged, Myelitis, Infectious Diseases, Neurology, Physical Sciences, Female, Anatomy, Research Article, medicine.medical_specialty, Materials Science, Pain, 03 medical and health sciences, Signs and Symptoms, Diagnostic Medicine, Internal medicine, Severity of illness, Mental Health and Psychiatry, medicine, Humans, Autoantibodies, Aquaporin 4, Damage Mechanics, Expanded Disability Status Scale, Neuromyelitis optica, business.industry, Mood Disorders, lcsh:R, Biology and Life Sciences, medicine.disease, Linear Models, Quality of Life, Lesions, lcsh:Q, Self Report, business, Sleep, Physiological Processes, Sleep Disorders, 030217 neurology & neurosurgery, Biomarkers
Abstract

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 =

Published
2017

45. Effect of Kidney Dysfunction on Cortical Thinning in Patients with Probable Alzheimer's Disease Dementia

Author

Duk L. Na, Kiho Im, Hanna Cho, Uicheul Yoon, Young Noh, Cindy W. Yoon, Eun Bin Cho, Sang Won Seo, Hee Jin Kim, Geon Ha Kim, Jongmin Lee, and Hojeong Kim

Subjects
Male, medicine.medical_specialty, Cross-sectional study, Renal function, Cortical thinning, Disease, Kidney Function Tests, urologic and male genital diseases, Alzheimer Disease, Internal medicine, medicine, Humans, Dementia, Aged, Aged, 80 and over, Cerebral Cortex, urogenital system, business.industry, General Neuroscience, Kidney dysfunction, Organ Size, General Medicine, Middle Aged, medicine.disease, Hyperintensity, Psychiatry and Mental health, Clinical Psychology, Cross-Sectional Studies, Quartile, Cardiology, Female, Kidney Diseases, Geriatrics and Gerontology, business, Glomerular Filtration Rate
Abstract

There are some studies identifying the association between kidney dysfunction and cognitive impairment through various mechanisms including small vessel disease. However, results concerning the relationship between kidney dysfunction and cortical atrophy have been inconsistent. Thus, we aimed to evaluate the relationship among kidney dysfunction, small vessel disease, and cortical thinning in probable Alzheimer's disease (AD) dementia patients. Patients consisted of 162 subjects with probable AD dementia who underwent high-resolution T1-weighted volumetric magnetic resonance imaging (MRI) scans using the same scanner. The estimated glomerular filtration rate (GFR) was calculated and divided into the quartiles of patients for comparison. Volume of white matter hyperintensities (WMH) was automatically measured. Two neurologists counted the number of lacunes. Cortical thickness was measured using a surface-based method. GFR was not associated with WMH and the number of lacunes. However, the lowest quartile group of GFR (GFR 1) had cortical thinning in each lobe, compared to the highest quartile group of GFR (GFR 4). The topography of cortical thinning in the GFR 1 group was distributed predominantly in temporoparietal regions, compared to GFR 4. After further adjustment of small vessel disease MRI markers, the association between GFR and the cortical thinning remained. Our findings suggested that kidney dysfunction, represented by GFR, was related to temporoparietal thinning independent of small vessel disease in probable AD dementia patients.

Published
2013
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46. Reference ranges for autonomic function tests in healthy Korean adults

Author

Eun Hee Sohn, Kee Hong Park, Heejeong Jeong, Eun Bin Cho, Kyoung Hwa Kang, Rock Bum Kim, Sa-Yoon Kang, Kyeong-jin Song, Byoung Joon Kim, Ki-Jong Park, Hyung Lee, Hyun Ah Kim, Sun-Young Oh, and Jin-Hong Shin

Subjects
Autonomic function, Parasympathetic nervous system, Autonomic nervous system, medicine.anatomical_structure, business.industry, Immunology, medicine, Reference range, business, Neuroscience
Published
2019
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47. Albuminuria, Cerebrovascular Disease and Cortical Atrophy: among Cognitively Normal Elderly Individuals

Author

Jin San Lee, Hee Jin Kim, Yeo Jin Kim, Hye Ryoun Jang, Jin-Ju Yang, Seungho Ryu, Eun Young Jang, Ju Hong Min, Sang Won Seo, Sang Eon Park, Eun Bin Cho, Mira Kang, Juyoun Lee, Changsoo Kim, Phillip Chun, Young Kyoung Jang, Jongmin Lee, Hee Young Shin, Na Yeon Jung, and Duk L. Na

Subjects
Adult, Male, medicine.medical_specialty, Renal function, Comorbidity, 030204 cardiovascular system & hematology, Article, 03 medical and health sciences, 0302 clinical medicine, Atrophy, Cognition, Risk Factors, Internal medicine, Diabetes mellitus, Medicine, Albuminuria, Humans, Renal Insufficiency, Chronic, Stroke, Aged, Aged, 80 and over, Cerebral Cortex, Multidisciplinary, business.industry, Middle Aged, medicine.disease, Magnetic Resonance Imaging, Hyperintensity, Cerebrovascular Disorders, Endocrinology, Decreased glomerular filtration rate, Population Surveillance, Cardiology, Female, medicine.symptom, business, Body mass index, 030217 neurology & neurosurgery, Glomerular Filtration Rate
Abstract

We tested the hypothesis that decreased glomerular filtration rate and albuminuria have different roles in brain structure alterations. We enrolled 1,215 cognitively normal individuals, all of whom underwent high-resolution T1-weighted volumetric magnetic resonance imaging scans. The cerebral small vessel disease burdens were assessed with white matter hyperintensities (WMH), lacunes, and microbleeds. Subjects were considered to have an abnormally elevated urine albumin creatinine ratio if the value was ≥17 mg/g for men and ≥25 mg/g for women. Albuminuria, but not estimated glomerular filtration rate (eGFR), was associated with increased WMH burdens (p = 0.002). The data was analyzed after adjusting for age, sex, education, history of hypertension, diabetes mellitus, hyperlipidemia, ischemic heart disease, stroke, total cholesterol level, body mass index, status of smoking and alcohol drinking, and intracranial volume. Albuminuria was also associated with cortical thinning, predominantly in the frontal and occipital regions (both p

Published
2016

48. Utility of the Rio Score and Modified Rio Score in Korean Patients withMultiple Sclerosis

Author

Joong Yang Cho, Ju Hong Min, Min Su Park, Ho Jin Kim, In Soo Joo, Jae Won Hyun, So Young Huh, Kee Duk Park, Eun Bin Cho, Nam Hee Kim, Jeeyoung Oh, In Hye Jeong, Su Hyun Kim, Young In Eom, Suk Won Ahn, and Byoung Joon Kim

Subjects
Adult, Male, medicine.medical_specialty, Multiple Sclerosis, Adolescent, Poor responder, lcsh:Medicine, Severity of Illness Index, Treatment failure, Young Adult, Internal medicine, Republic of Korea, Severity of illness, Humans, Immunologic Factors, Medicine, Stage (cooking), Young adult, lcsh:Science, Child, Retrospective Studies, Multidisciplinary, medicine.diagnostic_test, business.industry, Multiple sclerosis, lcsh:R, Magnetic resonance imaging, Retrospective cohort study, Interferon-beta, Middle Aged, medicine.disease, Surgery, Treatment Outcome, Disease Progression, lcsh:Q, Female, business, Research Article
Abstract

Objectives Early identification of suboptimal responders to multiple sclerosis (MS) treatment is critical for optimizing therapeutic decisions. The Rio score (RS) and modified Rio score (MRS) were developed to discriminate the responses to interferon-beta (IFNB) treatment in MS patients. This study was performed to evaluate the utility of RS and MRS in daily clinical practice in Korea. Methods This was a real-world setting, multicenter, retrospective study of MS patients treated with IFNB from 10 hospitals in Korea. We investigated whether the RS and MRS at the early stage of IFNB therapy could predict treatment responses over 3 years. Suboptimal treatment responses at 3 years were defined as the presence of clinical relapse and/or EDSS progression and/or patients who had been treated with INFB for at least for 1 year and therapy was switched due to perceived treatment failure during the 2 years of follow-up. Results Seventy patients (50 females and 20 males) were enrolled; 92% (12/13) of patients with high RS and 86% (12/14) of patients with high MRS (score 2 or 3) were suboptimal responders, whereas 93% (53/57) of patients with low RS and 93% (52/56) patients with low MRS (score 0 or 1) showed optimal responses. New active lesions on MRI with clinical relapse in high RS and MRS were the most common combination in suboptimal responders. Conclusions We confirmed that RS and MRS at 6–15 months of IFNB therapy were useful for predicting poor responders over 3 years.

Published
2015

49. Stiff-person syndrome after thymectomy in myasthenia gravis mimicking a post-thymectomy myasthenic crisis

Author

Hye Lim Lee, Byoung Joon Kim, Sang-Soo Lee, Eun Bin Cho, Jin Myoung Seok, Ju-Hong Min, and Hye Jin Cho

Subjects
Thymectomy, Pediatrics, medicine.medical_specialty, Neurology, business.industry, medicine.medical_treatment, Myasthenic crisis, medicine, Neurology (clinical), medicine.disease, business, Stiff person syndrome, Myasthenia gravis
Published
2017
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50. Skull Base Dermoid Cyst in the Right Infratemporal Fossa Diagnosed Using the Dixon Technique: a Case Report and Review of Literature

Author

Sung Eun Park, Hyo Jung An, Hye Jin Baek, Kyeong Hwa Ryu, Soo Buem Cho, Eun Bin Cho, Seung Jin Kim, Kyungsoo Bae, Jin Il Moon, Kyung Nyeo Jeon, and Bo Hwa Choi

Subjects
medicine.diagnostic_test, business.industry, Infratemporal fossa, Magnetic resonance imaging, Anatomy, medicine.disease, 030218 nuclear medicine & medical imaging, 03 medical and health sciences, Skull, 0302 clinical medicine, medicine.anatomical_structure, Dermoid cyst, 030220 oncology & carcinogenesis, Medicine, business, Base (exponentiation)
Published
2017
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