1.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.
2.Evaluating Rituximab Failure Rates in Neuromyelitis Optica Spectrum Disorder: A Nationwide Real-World Study From South Korea
Su-Hyun KIM ; Ju-Hong MIN ; Sung-Min KIM ; Eun-Jae LEE ; Young-Min LIM ; Ha Young SHIN ; Young Nam KWON ; Eunhee SOHN ; Sooyoung KIM ; Min Su PARK ; Tai-Seung NAM ; Byeol-A YOON ; Jong Kuk KIM ; Kyong Jin SHIN ; Yoo Hwan KIM ; Jin Myoung SEOK ; Jeong Bin BONG ; Sohyeon KIM ; Hung Youl SEOK ; Sun-Young OH ; Ohyun KWON ; Sunyoung KIM ; Sukyoon LEE ; Nam-Hee KIM ; Eun Bin CHO ; Sa-Yoon KANG ; Seong-il OH ; Jong Seok BAE ; Suk-Won AHN ; Ki Hoon KIM ; You-Ri KANG ; Woohee JU ; Seung Ho CHOO ; Yeon Hak CHUNG ; Jae-Won HYUN ; Ho Jin KIM
Journal of Clinical Neurology 2025;21(2):131-136
Background:
and Purpose Treatments for neuromyelitis optica spectrum disorder (NMOSD) such as eculizumab, ravulizumab, satralizumab, and inebilizumab have significantly advanced relapse prevention, but they remain expensive. Rituximab is an off-label yet popular alternative that offers a cost-effective solution, but its real-world efficacy needs better quantification for guiding the application of newer approved NMOSD treatments (ANTs). This study aimed to determine real-world rituximab failure rates to anticipate the demand for ANTs and aid in resource allocation.
Methods:
We conducted a nationwide retrospective study involving 605 aquaporin-4-antibody-positive NMOSD patients from 22 centers in South Korea that assessed the efficacy and safety of rituximab over a median follow-up of 47 months.
Results:
The 605 patients treated with rituximab included 525 (87%) who received continuous therapy throughout the follow-up period (median=47 months, interquartile range=15–87 months). During this period, 117 patients (19%) experienced at least 1 relapse. Notably, 68 of these patients (11% of the total cohort) experienced multiple relapses or at least 1 severe relapse.Additionally, 2% of the patients discontinued rituximab due to adverse events, which included severe infusion reactions, neutropenia, and infections.
Conclusions
This study has confirmed the efficacy of rituximab in treating NMOSD, as evidenced by an 87% continuation rate among patients over a 4-year follow-up period. Nevertheless, the occurrence of at least one relapse in 19% of the cohort, including 11% who experienced multiple or severe relapses, and a 2% discontinuation rate due to adverse events highlight the urgent need for alternative therapeutic options.
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.Evaluating Rituximab Failure Rates in Neuromyelitis Optica Spectrum Disorder: A Nationwide Real-World Study From South Korea
Su-Hyun KIM ; Ju-Hong MIN ; Sung-Min KIM ; Eun-Jae LEE ; Young-Min LIM ; Ha Young SHIN ; Young Nam KWON ; Eunhee SOHN ; Sooyoung KIM ; Min Su PARK ; Tai-Seung NAM ; Byeol-A YOON ; Jong Kuk KIM ; Kyong Jin SHIN ; Yoo Hwan KIM ; Jin Myoung SEOK ; Jeong Bin BONG ; Sohyeon KIM ; Hung Youl SEOK ; Sun-Young OH ; Ohyun KWON ; Sunyoung KIM ; Sukyoon LEE ; Nam-Hee KIM ; Eun Bin CHO ; Sa-Yoon KANG ; Seong-il OH ; Jong Seok BAE ; Suk-Won AHN ; Ki Hoon KIM ; You-Ri KANG ; Woohee JU ; Seung Ho CHOO ; Yeon Hak CHUNG ; Jae-Won HYUN ; Ho Jin KIM
Journal of Clinical Neurology 2025;21(2):131-136
Background:
and Purpose Treatments for neuromyelitis optica spectrum disorder (NMOSD) such as eculizumab, ravulizumab, satralizumab, and inebilizumab have significantly advanced relapse prevention, but they remain expensive. Rituximab is an off-label yet popular alternative that offers a cost-effective solution, but its real-world efficacy needs better quantification for guiding the application of newer approved NMOSD treatments (ANTs). This study aimed to determine real-world rituximab failure rates to anticipate the demand for ANTs and aid in resource allocation.
Methods:
We conducted a nationwide retrospective study involving 605 aquaporin-4-antibody-positive NMOSD patients from 22 centers in South Korea that assessed the efficacy and safety of rituximab over a median follow-up of 47 months.
Results:
The 605 patients treated with rituximab included 525 (87%) who received continuous therapy throughout the follow-up period (median=47 months, interquartile range=15–87 months). During this period, 117 patients (19%) experienced at least 1 relapse. Notably, 68 of these patients (11% of the total cohort) experienced multiple relapses or at least 1 severe relapse.Additionally, 2% of the patients discontinued rituximab due to adverse events, which included severe infusion reactions, neutropenia, and infections.
Conclusions
This study has confirmed the efficacy of rituximab in treating NMOSD, as evidenced by an 87% continuation rate among patients over a 4-year follow-up period. Nevertheless, the occurrence of at least one relapse in 19% of the cohort, including 11% who experienced multiple or severe relapses, and a 2% discontinuation rate due to adverse events highlight the urgent need for alternative therapeutic options.
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.Evaluating Rituximab Failure Rates in Neuromyelitis Optica Spectrum Disorder: A Nationwide Real-World Study From South Korea
Su-Hyun KIM ; Ju-Hong MIN ; Sung-Min KIM ; Eun-Jae LEE ; Young-Min LIM ; Ha Young SHIN ; Young Nam KWON ; Eunhee SOHN ; Sooyoung KIM ; Min Su PARK ; Tai-Seung NAM ; Byeol-A YOON ; Jong Kuk KIM ; Kyong Jin SHIN ; Yoo Hwan KIM ; Jin Myoung SEOK ; Jeong Bin BONG ; Sohyeon KIM ; Hung Youl SEOK ; Sun-Young OH ; Ohyun KWON ; Sunyoung KIM ; Sukyoon LEE ; Nam-Hee KIM ; Eun Bin CHO ; Sa-Yoon KANG ; Seong-il OH ; Jong Seok BAE ; Suk-Won AHN ; Ki Hoon KIM ; You-Ri KANG ; Woohee JU ; Seung Ho CHOO ; Yeon Hak CHUNG ; Jae-Won HYUN ; Ho Jin KIM
Journal of Clinical Neurology 2025;21(2):131-136
Background:
and Purpose Treatments for neuromyelitis optica spectrum disorder (NMOSD) such as eculizumab, ravulizumab, satralizumab, and inebilizumab have significantly advanced relapse prevention, but they remain expensive. Rituximab is an off-label yet popular alternative that offers a cost-effective solution, but its real-world efficacy needs better quantification for guiding the application of newer approved NMOSD treatments (ANTs). This study aimed to determine real-world rituximab failure rates to anticipate the demand for ANTs and aid in resource allocation.
Methods:
We conducted a nationwide retrospective study involving 605 aquaporin-4-antibody-positive NMOSD patients from 22 centers in South Korea that assessed the efficacy and safety of rituximab over a median follow-up of 47 months.
Results:
The 605 patients treated with rituximab included 525 (87%) who received continuous therapy throughout the follow-up period (median=47 months, interquartile range=15–87 months). During this period, 117 patients (19%) experienced at least 1 relapse. Notably, 68 of these patients (11% of the total cohort) experienced multiple relapses or at least 1 severe relapse.Additionally, 2% of the patients discontinued rituximab due to adverse events, which included severe infusion reactions, neutropenia, and infections.
Conclusions
This study has confirmed the efficacy of rituximab in treating NMOSD, as evidenced by an 87% continuation rate among patients over a 4-year follow-up period. Nevertheless, the occurrence of at least one relapse in 19% of the cohort, including 11% who experienced multiple or severe relapses, and a 2% discontinuation rate due to adverse events highlight the urgent need for alternative therapeutic options.
7.Obesity in Children and Adolescents: 2022 Update of Clinical Practice Guidelines for Obesity by the Korean Society for the Study of Obesity
Eungu KANG ; Yong Hee HONG ; Jaehyun KIM ; Sochung CHUNG ; Kyoung-Kon KIM ; Ji-Hee HAAM ; Bom Taeck KIM ; Eun Mi KIM ; Jung Hwan PARK ; Sang Youl RHEE ; Jee-Hyun KANG ; Young-Jun RHIE
Journal of Obesity & Metabolic Syndrome 2024;33(1):11-19
The prevalence of obesity in children and adolescents has been gradually increasing in recent years and has become a major health problem. Childhood obesity can readily progress to adult obesity. It is associated with obesity-related comorbidities, such as type 2 diabetes mellitus, hypertension, obstructive sleep apnea, non-alcoholic fatty liver disease, and the risk factor for cardiovascular disease. It is important to make an accurate assessment of overweight and obesity in children and adolescents with consideration of growth and development. Childhood obesity can then be prevented and treated using an appropriate treatment goal and safe and effective treatment strategies. This article summarizes the clinical practice guidelines for obesity in children and adolescents that are included in the 8th edition of the Clinical Practice Guidelines for Obesity of the Korean Society for the Study of Obesity.
8.Description of FDG and Prostate-Specific Membrane Antigen PET/CT Findings in Korean Patients With Advanced Metastatic Castration-Resistant Prostate Cancer
Sae Jung NA ; Seunggyun HA ; In-Ho KIM ; Ji Youl LEE ; Joo Hyun O
Korean Journal of Radiology 2024;25(11):1022-1028
Objective:
We aimed to describe the [ 18 F]fluorodeoxyglucose (FDG) and prostate-specific membrane antigen (PSMA) PET/CT findings in Korean men with advanced metastatic castration-resistant prostate cancer (mCRPC).
Materials and Methods:
The results of paired FDG and PSMA PET/CT examinations performed in 42 consecutive men with prostate cancer for treatment planning after failure of anti-androgen therapy and chemotherapy were studied. Tumor lesions with FDG or PSMA uptake intensity higher than that of the liver on visual review were considered positive and noted per patient and tumor site (prostate bed, lymph node, bone, and visceral organ). The presence of unequivocally discordant FDG and PSMA uptake patterns in tumor lesions was assessed. Patients were grouped according to the total tumor volume as seen on each PET/CT scan, and the clinical findings between the patient groups were compared using the Mann-Whitney U test.
Results:
On patient-based analysis, the image findings were PSMA+/FDG- in 2 patients, PSMA-/FDG+ in one, and PSMA+/FDG+ in 39 patients. On site-based analysis, the discordance (PSMA+/FDG- or PSMA-/FDG+) rate was 9.5% (4/42) for prostate/bed, 11.9% (5/42) for lymph nodes, 9.5% (4/42) for bones, and 11.9% (5/42) for visceral organs. FDG uptake was higher than PSMA uptake in at least one tumor site in 54.8% (23/42) of patients. Patients with greater total tumor volume on FDG PET/CT than that on PSMA PET/CT (“FDG-dominant pattern”) accounted for 28.6% (12/42), and they had significantly shorter time from diagnosis (median 25 months vs. 62 months, P = 0.049), and higher aspartate aminotransferase (median 28.5 vs. 22.5, P = 0.027) and lactate dehydrogenase (median 341.5 vs. 224.5, P = 0.010) levels.
Conclusion
Most patients with advanced mCRPC had tumors with positive findings on both FDG and PSMA PET/CT. However, the uptake patterns varied; 54.8% of the patients had tumor(s) with FDG uptake greater than PSMA uptake, and FDGdominant pattern was noted in 28.6% of the patients.
9.Description of FDG and Prostate-Specific Membrane Antigen PET/CT Findings in Korean Patients With Advanced Metastatic Castration-Resistant Prostate Cancer
Sae Jung NA ; Seunggyun HA ; In-Ho KIM ; Ji Youl LEE ; Joo Hyun O
Korean Journal of Radiology 2024;25(11):1022-1028
Objective:
We aimed to describe the [ 18 F]fluorodeoxyglucose (FDG) and prostate-specific membrane antigen (PSMA) PET/CT findings in Korean men with advanced metastatic castration-resistant prostate cancer (mCRPC).
Materials and Methods:
The results of paired FDG and PSMA PET/CT examinations performed in 42 consecutive men with prostate cancer for treatment planning after failure of anti-androgen therapy and chemotherapy were studied. Tumor lesions with FDG or PSMA uptake intensity higher than that of the liver on visual review were considered positive and noted per patient and tumor site (prostate bed, lymph node, bone, and visceral organ). The presence of unequivocally discordant FDG and PSMA uptake patterns in tumor lesions was assessed. Patients were grouped according to the total tumor volume as seen on each PET/CT scan, and the clinical findings between the patient groups were compared using the Mann-Whitney U test.
Results:
On patient-based analysis, the image findings were PSMA+/FDG- in 2 patients, PSMA-/FDG+ in one, and PSMA+/FDG+ in 39 patients. On site-based analysis, the discordance (PSMA+/FDG- or PSMA-/FDG+) rate was 9.5% (4/42) for prostate/bed, 11.9% (5/42) for lymph nodes, 9.5% (4/42) for bones, and 11.9% (5/42) for visceral organs. FDG uptake was higher than PSMA uptake in at least one tumor site in 54.8% (23/42) of patients. Patients with greater total tumor volume on FDG PET/CT than that on PSMA PET/CT (“FDG-dominant pattern”) accounted for 28.6% (12/42), and they had significantly shorter time from diagnosis (median 25 months vs. 62 months, P = 0.049), and higher aspartate aminotransferase (median 28.5 vs. 22.5, P = 0.027) and lactate dehydrogenase (median 341.5 vs. 224.5, P = 0.010) levels.
Conclusion
Most patients with advanced mCRPC had tumors with positive findings on both FDG and PSMA PET/CT. However, the uptake patterns varied; 54.8% of the patients had tumor(s) with FDG uptake greater than PSMA uptake, and FDGdominant pattern was noted in 28.6% of the patients.
10.Description of FDG and Prostate-Specific Membrane Antigen PET/CT Findings in Korean Patients With Advanced Metastatic Castration-Resistant Prostate Cancer
Sae Jung NA ; Seunggyun HA ; In-Ho KIM ; Ji Youl LEE ; Joo Hyun O
Korean Journal of Radiology 2024;25(11):1022-1028
Objective:
We aimed to describe the [ 18 F]fluorodeoxyglucose (FDG) and prostate-specific membrane antigen (PSMA) PET/CT findings in Korean men with advanced metastatic castration-resistant prostate cancer (mCRPC).
Materials and Methods:
The results of paired FDG and PSMA PET/CT examinations performed in 42 consecutive men with prostate cancer for treatment planning after failure of anti-androgen therapy and chemotherapy were studied. Tumor lesions with FDG or PSMA uptake intensity higher than that of the liver on visual review were considered positive and noted per patient and tumor site (prostate bed, lymph node, bone, and visceral organ). The presence of unequivocally discordant FDG and PSMA uptake patterns in tumor lesions was assessed. Patients were grouped according to the total tumor volume as seen on each PET/CT scan, and the clinical findings between the patient groups were compared using the Mann-Whitney U test.
Results:
On patient-based analysis, the image findings were PSMA+/FDG- in 2 patients, PSMA-/FDG+ in one, and PSMA+/FDG+ in 39 patients. On site-based analysis, the discordance (PSMA+/FDG- or PSMA-/FDG+) rate was 9.5% (4/42) for prostate/bed, 11.9% (5/42) for lymph nodes, 9.5% (4/42) for bones, and 11.9% (5/42) for visceral organs. FDG uptake was higher than PSMA uptake in at least one tumor site in 54.8% (23/42) of patients. Patients with greater total tumor volume on FDG PET/CT than that on PSMA PET/CT (“FDG-dominant pattern”) accounted for 28.6% (12/42), and they had significantly shorter time from diagnosis (median 25 months vs. 62 months, P = 0.049), and higher aspartate aminotransferase (median 28.5 vs. 22.5, P = 0.027) and lactate dehydrogenase (median 341.5 vs. 224.5, P = 0.010) levels.
Conclusion
Most patients with advanced mCRPC had tumors with positive findings on both FDG and PSMA PET/CT. However, the uptake patterns varied; 54.8% of the patients had tumor(s) with FDG uptake greater than PSMA uptake, and FDGdominant pattern was noted in 28.6% of the patients.

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