1.Adult-onset Neuronal Intranuclear Inclusion Disease Presenting with Intermittent Visual Disturbances and Right Hemiparesis: Clinical Significance and Diagnostic Approach
Doyeon KOOK ; Yunjung CHOI ; Jiyun LEE ; Hyung Jun PARK ; Hanna CHO ; Hyunjin PARK ; HanKyeol KIM ; Takeshi MIZUGUCHI ; Naomichi MATSUMOTO ; Won-Joo KIM
Journal of the Korean Neurological Association 2025;43(2):100-104
Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disorder characterized by the presence of eosinophilic nuclear inclusions in neurons and somatic cells. It clinically manifests as cognitive decline, seizures, and autonomic dysfunction. A 44-year-old man presented with a transient visual field defect and hemiparesis. Based on characteristic imaging findings and pathological findings, NIID was suspected and diagnosed through genetic testing. This case emphasizes the importance of comprehensive clinical phenotype analysis and accurate genetic diagnosis.
2.Profiling of Anti-Signal-Recognition Particle Antibodies and Clinical Characteristics in South Korean Patients With Immune-Mediated Necrotizing Myopathy
Soo-Hyun KIM ; Yunjung CHOI ; Eun Kyoung OH ; Ichizo NISHINO ; Shigeaki SUZUKI ; Bum Chun SUH ; Ha Young SHIN ; Seung Woo KIM ; Byeol-A YOON ; Seong-il OH ; Yoo Hwan KIM ; Hyunjin KIM ; Young-Min LIM ; Seol-Hee BAEK ; Je-Young SHIN ; Hung Youl SEOK ; Seung-Ah LEE ; Young-Chul CHOI ; Hyung Jun PARK
Journal of Clinical Neurology 2025;21(1):31-39
Background:
and Purpose This study evaluated the diagnostic utility of an anti-signal-recognition particle 54 (anti-SRP54) antibody-based enzyme-linked immunosorbent assay (ELISA) as well as the clinical, serological, and pathological characteristics of patients with SRP immune-mediated necrotizing myopathy (IMNM).
Methods:
We evaluated 87 patients with idiopathic inflammatory myopathy and 107 healthy participants between January 2002 and December 2023. The sensitivity and specificity of the ELISA for anti-SRP54 antibodies were assessed, and the clinical profiles of patients with antiSRP54 antibodies were determined.
Results:
The ELISA for anti-SRP54 antibodies had a sensitivity and specificity of 88% and 99%, respectively, along with a test–retest reliability of 0.92 (p<0.001). The 32 patients diagnosed with anti-SRP IMNM using a line-blot immunoassay included 28 (88%) who tested positive for anti-SRP54 antibodies using the ELISA, comprising 12 (43%) males and 16 (57%) females whose median ages at symptom onset and diagnosis were 43.0 years and 43.5 years, respectively. Symptoms included proximal muscle weakness in all 28 (100%) patients, neck weakness in 9 (32%), myalgia in 15 (54%), dysphagia in 5 (18%), dyspnea in 4 (14%), dysarthria in 2 (7%), interstitial lung disease in 2 (7%), and myocarditis in 2 (7%). The median serum creatine kinase (CK) level was 7,261 U/L (interquartile range: 5,086–10,007 U/L), and the median anti-SRP54 antibody level was 2.0 U/mL (interquartile range: 1.0–5.6 U/mL). The serum CK level was significantly higher in patients with coexisting anti-Ro-52 antibodies.
Conclusions
This study has confirmed the reliability of the ELISA for anti-SRP54 antibodies and provided insights into the clinical, serological, and pathological characteristics of South Korean patients with anti-SRP IMNM.
3.Profiling of Anti-Signal-Recognition Particle Antibodies and Clinical Characteristics in South Korean Patients With Immune-Mediated Necrotizing Myopathy
Soo-Hyun KIM ; Yunjung CHOI ; Eun Kyoung OH ; Ichizo NISHINO ; Shigeaki SUZUKI ; Bum Chun SUH ; Ha Young SHIN ; Seung Woo KIM ; Byeol-A YOON ; Seong-il OH ; Yoo Hwan KIM ; Hyunjin KIM ; Young-Min LIM ; Seol-Hee BAEK ; Je-Young SHIN ; Hung Youl SEOK ; Seung-Ah LEE ; Young-Chul CHOI ; Hyung Jun PARK
Journal of Clinical Neurology 2025;21(1):31-39
Background:
and Purpose This study evaluated the diagnostic utility of an anti-signal-recognition particle 54 (anti-SRP54) antibody-based enzyme-linked immunosorbent assay (ELISA) as well as the clinical, serological, and pathological characteristics of patients with SRP immune-mediated necrotizing myopathy (IMNM).
Methods:
We evaluated 87 patients with idiopathic inflammatory myopathy and 107 healthy participants between January 2002 and December 2023. The sensitivity and specificity of the ELISA for anti-SRP54 antibodies were assessed, and the clinical profiles of patients with antiSRP54 antibodies were determined.
Results:
The ELISA for anti-SRP54 antibodies had a sensitivity and specificity of 88% and 99%, respectively, along with a test–retest reliability of 0.92 (p<0.001). The 32 patients diagnosed with anti-SRP IMNM using a line-blot immunoassay included 28 (88%) who tested positive for anti-SRP54 antibodies using the ELISA, comprising 12 (43%) males and 16 (57%) females whose median ages at symptom onset and diagnosis were 43.0 years and 43.5 years, respectively. Symptoms included proximal muscle weakness in all 28 (100%) patients, neck weakness in 9 (32%), myalgia in 15 (54%), dysphagia in 5 (18%), dyspnea in 4 (14%), dysarthria in 2 (7%), interstitial lung disease in 2 (7%), and myocarditis in 2 (7%). The median serum creatine kinase (CK) level was 7,261 U/L (interquartile range: 5,086–10,007 U/L), and the median anti-SRP54 antibody level was 2.0 U/mL (interquartile range: 1.0–5.6 U/mL). The serum CK level was significantly higher in patients with coexisting anti-Ro-52 antibodies.
Conclusions
This study has confirmed the reliability of the ELISA for anti-SRP54 antibodies and provided insights into the clinical, serological, and pathological characteristics of South Korean patients with anti-SRP IMNM.
4.Adult-onset Neuronal Intranuclear Inclusion Disease Presenting with Intermittent Visual Disturbances and Right Hemiparesis: Clinical Significance and Diagnostic Approach
Doyeon KOOK ; Yunjung CHOI ; Jiyun LEE ; Hyung Jun PARK ; Hanna CHO ; Hyunjin PARK ; HanKyeol KIM ; Takeshi MIZUGUCHI ; Naomichi MATSUMOTO ; Won-Joo KIM
Journal of the Korean Neurological Association 2025;43(2):100-104
Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disorder characterized by the presence of eosinophilic nuclear inclusions in neurons and somatic cells. It clinically manifests as cognitive decline, seizures, and autonomic dysfunction. A 44-year-old man presented with a transient visual field defect and hemiparesis. Based on characteristic imaging findings and pathological findings, NIID was suspected and diagnosed through genetic testing. This case emphasizes the importance of comprehensive clinical phenotype analysis and accurate genetic diagnosis.
5.Profiling of Anti-Signal-Recognition Particle Antibodies and Clinical Characteristics in South Korean Patients With Immune-Mediated Necrotizing Myopathy
Soo-Hyun KIM ; Yunjung CHOI ; Eun Kyoung OH ; Ichizo NISHINO ; Shigeaki SUZUKI ; Bum Chun SUH ; Ha Young SHIN ; Seung Woo KIM ; Byeol-A YOON ; Seong-il OH ; Yoo Hwan KIM ; Hyunjin KIM ; Young-Min LIM ; Seol-Hee BAEK ; Je-Young SHIN ; Hung Youl SEOK ; Seung-Ah LEE ; Young-Chul CHOI ; Hyung Jun PARK
Journal of Clinical Neurology 2025;21(1):31-39
Background:
and Purpose This study evaluated the diagnostic utility of an anti-signal-recognition particle 54 (anti-SRP54) antibody-based enzyme-linked immunosorbent assay (ELISA) as well as the clinical, serological, and pathological characteristics of patients with SRP immune-mediated necrotizing myopathy (IMNM).
Methods:
We evaluated 87 patients with idiopathic inflammatory myopathy and 107 healthy participants between January 2002 and December 2023. The sensitivity and specificity of the ELISA for anti-SRP54 antibodies were assessed, and the clinical profiles of patients with antiSRP54 antibodies were determined.
Results:
The ELISA for anti-SRP54 antibodies had a sensitivity and specificity of 88% and 99%, respectively, along with a test–retest reliability of 0.92 (p<0.001). The 32 patients diagnosed with anti-SRP IMNM using a line-blot immunoassay included 28 (88%) who tested positive for anti-SRP54 antibodies using the ELISA, comprising 12 (43%) males and 16 (57%) females whose median ages at symptom onset and diagnosis were 43.0 years and 43.5 years, respectively. Symptoms included proximal muscle weakness in all 28 (100%) patients, neck weakness in 9 (32%), myalgia in 15 (54%), dysphagia in 5 (18%), dyspnea in 4 (14%), dysarthria in 2 (7%), interstitial lung disease in 2 (7%), and myocarditis in 2 (7%). The median serum creatine kinase (CK) level was 7,261 U/L (interquartile range: 5,086–10,007 U/L), and the median anti-SRP54 antibody level was 2.0 U/mL (interquartile range: 1.0–5.6 U/mL). The serum CK level was significantly higher in patients with coexisting anti-Ro-52 antibodies.
Conclusions
This study has confirmed the reliability of the ELISA for anti-SRP54 antibodies and provided insights into the clinical, serological, and pathological characteristics of South Korean patients with anti-SRP IMNM.
6.Adult-onset Neuronal Intranuclear Inclusion Disease Presenting with Intermittent Visual Disturbances and Right Hemiparesis: Clinical Significance and Diagnostic Approach
Doyeon KOOK ; Yunjung CHOI ; Jiyun LEE ; Hyung Jun PARK ; Hanna CHO ; Hyunjin PARK ; HanKyeol KIM ; Takeshi MIZUGUCHI ; Naomichi MATSUMOTO ; Won-Joo KIM
Journal of the Korean Neurological Association 2025;43(2):100-104
Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disorder characterized by the presence of eosinophilic nuclear inclusions in neurons and somatic cells. It clinically manifests as cognitive decline, seizures, and autonomic dysfunction. A 44-year-old man presented with a transient visual field defect and hemiparesis. Based on characteristic imaging findings and pathological findings, NIID was suspected and diagnosed through genetic testing. This case emphasizes the importance of comprehensive clinical phenotype analysis and accurate genetic diagnosis.
7.Immunomodulatory effects of ovalbumin extracts on splenocyte and macrophages in mice
Yunjung LEE ; Mijoo CHOI ; Eunju PARK
Journal of Nutrition and Health 2024;57(6):605-618
Purpose:
Ovalbumin, a major protein derived from egg white, has significant functional properties, including antioxidant, antibacterial, anti-inflammatory, and immunomodulatory effects. Therefore, ovalbumin has potential use in the nutraceutical, pharmaceutical, and cosmeceutical industries. This study evaluated the immunomodulatory effects of ovalbumin water extract (OAW) and ethanol extract (OAE) on splenocytes and peritoneal macrophages in Balb/c mice, focusing on their potential to regulate the Th1/Th2 balance and enhance immune functions.
Methods:
The effects of OAW and OAE on T and B-cell proliferation, cytokines, immunoglobulins, phagocytic activity, and nitric oxide (NO) production were examined using mitogen (ConA or LPS)-treated mouse splenocytes and macrophages from Balb/c mice.OAW and OAE were applied at concentrations ranging from 50 to 500 μg/mL. T and B-cell proliferation, cytokine production, including Th1 (interleukin [IL]-2, interferon [IFN]-γ, tumor necrosis factor [TNF]-α) and Th2 (IL-4, IL-6, and IL-10) profiles, and immunoglobulin (IgA, IgG, and IgE) production were analyzed using enzyme-linked immunosorbent assay.NO production and phagocytic activity were evaluated using a Griess assay and substratebased assays, respectively.
Results:
OAW and OAE modulated T and B-cell proliferation and inhibited the production of Th1 (IL-2, IFN-γ, and TNF-α) and Th2 (IL-4, IL-6, and IL-10) cytokines, restoring the Th1/ Th2 balance. Moreover, at 500 μg/mL, the OAW extracts exhibited comparable effects to the zymosan-stimulated controls. The production of immunoglobulin (IgA, IgG, and IgE) and NO levels were reduced, while the macrophage phagocytic activity was enhanced in a dosedependent manner.
Conclusion
Ovalbumin extracts can modulate the systemic immune system by influencing immunoglobulin production and regulating the Th1/Th2 balance.
8.Immunomodulatory effects of ovalbumin extracts on splenocyte and macrophages in mice
Yunjung LEE ; Mijoo CHOI ; Eunju PARK
Journal of Nutrition and Health 2024;57(6):605-618
Purpose:
Ovalbumin, a major protein derived from egg white, has significant functional properties, including antioxidant, antibacterial, anti-inflammatory, and immunomodulatory effects. Therefore, ovalbumin has potential use in the nutraceutical, pharmaceutical, and cosmeceutical industries. This study evaluated the immunomodulatory effects of ovalbumin water extract (OAW) and ethanol extract (OAE) on splenocytes and peritoneal macrophages in Balb/c mice, focusing on their potential to regulate the Th1/Th2 balance and enhance immune functions.
Methods:
The effects of OAW and OAE on T and B-cell proliferation, cytokines, immunoglobulins, phagocytic activity, and nitric oxide (NO) production were examined using mitogen (ConA or LPS)-treated mouse splenocytes and macrophages from Balb/c mice.OAW and OAE were applied at concentrations ranging from 50 to 500 μg/mL. T and B-cell proliferation, cytokine production, including Th1 (interleukin [IL]-2, interferon [IFN]-γ, tumor necrosis factor [TNF]-α) and Th2 (IL-4, IL-6, and IL-10) profiles, and immunoglobulin (IgA, IgG, and IgE) production were analyzed using enzyme-linked immunosorbent assay.NO production and phagocytic activity were evaluated using a Griess assay and substratebased assays, respectively.
Results:
OAW and OAE modulated T and B-cell proliferation and inhibited the production of Th1 (IL-2, IFN-γ, and TNF-α) and Th2 (IL-4, IL-6, and IL-10) cytokines, restoring the Th1/ Th2 balance. Moreover, at 500 μg/mL, the OAW extracts exhibited comparable effects to the zymosan-stimulated controls. The production of immunoglobulin (IgA, IgG, and IgE) and NO levels were reduced, while the macrophage phagocytic activity was enhanced in a dosedependent manner.
Conclusion
Ovalbumin extracts can modulate the systemic immune system by influencing immunoglobulin production and regulating the Th1/Th2 balance.
9.Immunomodulatory effects of ovalbumin extracts on splenocyte and macrophages in mice
Yunjung LEE ; Mijoo CHOI ; Eunju PARK
Journal of Nutrition and Health 2024;57(6):605-618
Purpose:
Ovalbumin, a major protein derived from egg white, has significant functional properties, including antioxidant, antibacterial, anti-inflammatory, and immunomodulatory effects. Therefore, ovalbumin has potential use in the nutraceutical, pharmaceutical, and cosmeceutical industries. This study evaluated the immunomodulatory effects of ovalbumin water extract (OAW) and ethanol extract (OAE) on splenocytes and peritoneal macrophages in Balb/c mice, focusing on their potential to regulate the Th1/Th2 balance and enhance immune functions.
Methods:
The effects of OAW and OAE on T and B-cell proliferation, cytokines, immunoglobulins, phagocytic activity, and nitric oxide (NO) production were examined using mitogen (ConA or LPS)-treated mouse splenocytes and macrophages from Balb/c mice.OAW and OAE were applied at concentrations ranging from 50 to 500 μg/mL. T and B-cell proliferation, cytokine production, including Th1 (interleukin [IL]-2, interferon [IFN]-γ, tumor necrosis factor [TNF]-α) and Th2 (IL-4, IL-6, and IL-10) profiles, and immunoglobulin (IgA, IgG, and IgE) production were analyzed using enzyme-linked immunosorbent assay.NO production and phagocytic activity were evaluated using a Griess assay and substratebased assays, respectively.
Results:
OAW and OAE modulated T and B-cell proliferation and inhibited the production of Th1 (IL-2, IFN-γ, and TNF-α) and Th2 (IL-4, IL-6, and IL-10) cytokines, restoring the Th1/ Th2 balance. Moreover, at 500 μg/mL, the OAW extracts exhibited comparable effects to the zymosan-stimulated controls. The production of immunoglobulin (IgA, IgG, and IgE) and NO levels were reduced, while the macrophage phagocytic activity was enhanced in a dosedependent manner.
Conclusion
Ovalbumin extracts can modulate the systemic immune system by influencing immunoglobulin production and regulating the Th1/Th2 balance.
10.Rapid Direct Identification of Microbial Pathogens and Antimicrobial Resistance Genes in Positive Blood Cultures Using a Fully Automated Multiplex PCR Assay
Keun Ju KIM ; Seung Gyu YUN ; Yunjung CHO ; Chang Kyu LEE ; Myung-Hyun NAM
Journal of Korean Medical Science 2024;39(17):e157-
This study assessed the performance of the BioFire Blood Culture Identification 2 (BCID2) panel in identifying microorganisms and antimicrobial resistance (AMR) profiles in positive blood cultures (BCs) and its influence on turnaround time (TAT) compared with conventional culture methods. We obtained 117 positive BCs, of these, 102 (87.2%) were correctly identified using BCID2. The discordance was due to off-panel pathogens detected by culture (n = 13), and additional pathogens identified by BCID2 (n = 2). On-panel pathogen concordance between the conventional culture and BCID2 methods was 98.1% (102/104).The conventional method detected 19 carbapenemase-producing organisms, 14 extendedspectrum beta-lactamase-producing Enterobacterales, 18 methicillin-resistant Staphylococcus spp., and four vancomycin-resistant Enterococcus faecium. BCID2 correctly predicted 53 (96.4%) of 55 phenotypic resistance patterns by detecting AMR genes. The TAT for BCID2 was significantly lower than that for the conventional method. BCID2 rapidly identifies pathogens and AMR genes in positive BCs.

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