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.Eligibility for Lecanemab Treatment in the Republic of Korea:Real-World Data From Memory Clinics
Sung Hoon KANG ; Jee Hyang JEONG ; Jung-Min PYUN ; Geon Ha KIM ; Young Ho PARK ; YongSoo SHIM ; Seong-Ho KOH ; Chi-Hun KIM ; Young Chul YOUN ; Dong Won YANG ; Hyuk-je LEE ; Han LEE ; Dain KIM ; Kyunghwa SUN ; So Young MOON ; Kee Hyung PARK ; Seong Hye CHOI
Journal of Clinical Neurology 2025;21(3):182-189
Background:
and Purpose We aimed to determine the proportion of Korean patients with early Alzheimer’s disease (AD) who are eligible to receive lecanemab based on the United States Appropriate Use Recommendations (US AUR), and also identify the barriers to this treatment.
Methods:
We retrospectively enrolled 6,132 patients with amnestic mild cognitive impairment or mild amnestic dementia at 13 hospitals from June 2023 to May 2024. Among them, 2,058 patients underwent amyloid positron emission tomography (PET) and 1,199 (58.3%) of these patients were amyloid-positive on PET. We excluded 732 patients who did not undergo brain magnetic resonance imaging between June 2023 and May 2024. Finally, 467 patients were included in the present study.
Results:
When applying the criteria of the US AUR, approximately 50% of patients with early AD were eligible to receive lecanemab treatment. Among the 467 included patients, 36.8% did not meet the inclusion criterion of a Mini-Mental State Examination (MMSE) score of ≥22.
Conclusions
Eligibility for lecanemab treatment was not restricted to Korean patients with early AD except for those with an MMSE score of ≥22. The MMSE criteria should therefore be reconsidered in areas with a higher proportion of older people, who tend to have lower levels of education.
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.Eligibility for Lecanemab Treatment in the Republic of Korea:Real-World Data From Memory Clinics
Sung Hoon KANG ; Jee Hyang JEONG ; Jung-Min PYUN ; Geon Ha KIM ; Young Ho PARK ; YongSoo SHIM ; Seong-Ho KOH ; Chi-Hun KIM ; Young Chul YOUN ; Dong Won YANG ; Hyuk-je LEE ; Han LEE ; Dain KIM ; Kyunghwa SUN ; So Young MOON ; Kee Hyung PARK ; Seong Hye CHOI
Journal of Clinical Neurology 2025;21(3):182-189
Background:
and Purpose We aimed to determine the proportion of Korean patients with early Alzheimer’s disease (AD) who are eligible to receive lecanemab based on the United States Appropriate Use Recommendations (US AUR), and also identify the barriers to this treatment.
Methods:
We retrospectively enrolled 6,132 patients with amnestic mild cognitive impairment or mild amnestic dementia at 13 hospitals from June 2023 to May 2024. Among them, 2,058 patients underwent amyloid positron emission tomography (PET) and 1,199 (58.3%) of these patients were amyloid-positive on PET. We excluded 732 patients who did not undergo brain magnetic resonance imaging between June 2023 and May 2024. Finally, 467 patients were included in the present study.
Results:
When applying the criteria of the US AUR, approximately 50% of patients with early AD were eligible to receive lecanemab treatment. Among the 467 included patients, 36.8% did not meet the inclusion criterion of a Mini-Mental State Examination (MMSE) score of ≥22.
Conclusions
Eligibility for lecanemab treatment was not restricted to Korean patients with early AD except for those with an MMSE score of ≥22. The MMSE criteria should therefore be reconsidered in areas with a higher proportion of older people, who tend to have lower levels of education.
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.Eligibility for Lecanemab Treatment in the Republic of Korea:Real-World Data From Memory Clinics
Sung Hoon KANG ; Jee Hyang JEONG ; Jung-Min PYUN ; Geon Ha KIM ; Young Ho PARK ; YongSoo SHIM ; Seong-Ho KOH ; Chi-Hun KIM ; Young Chul YOUN ; Dong Won YANG ; Hyuk-je LEE ; Han LEE ; Dain KIM ; Kyunghwa SUN ; So Young MOON ; Kee Hyung PARK ; Seong Hye CHOI
Journal of Clinical Neurology 2025;21(3):182-189
Background:
and Purpose We aimed to determine the proportion of Korean patients with early Alzheimer’s disease (AD) who are eligible to receive lecanemab based on the United States Appropriate Use Recommendations (US AUR), and also identify the barriers to this treatment.
Methods:
We retrospectively enrolled 6,132 patients with amnestic mild cognitive impairment or mild amnestic dementia at 13 hospitals from June 2023 to May 2024. Among them, 2,058 patients underwent amyloid positron emission tomography (PET) and 1,199 (58.3%) of these patients were amyloid-positive on PET. We excluded 732 patients who did not undergo brain magnetic resonance imaging between June 2023 and May 2024. Finally, 467 patients were included in the present study.
Results:
When applying the criteria of the US AUR, approximately 50% of patients with early AD were eligible to receive lecanemab treatment. Among the 467 included patients, 36.8% did not meet the inclusion criterion of a Mini-Mental State Examination (MMSE) score of ≥22.
Conclusions
Eligibility for lecanemab treatment was not restricted to Korean patients with early AD except for those with an MMSE score of ≥22. The MMSE criteria should therefore be reconsidered in areas with a higher proportion of older people, who tend to have lower levels of education.
7.Clonal Distribution and Its Association With the Carbapenem Resistance Mechanisms of Carbapenem-Non-Susceptible Pseudomonas aeruginosa Isolates From Korean Hospitals
Nayeong KIM ; Seo Yeon KO ; Seong Yong PARK ; Seong Yeob KIM ; Da Eun LEE ; Ki Tae KWON ; Yu Kyung KIM ; Je Chul LEE
Annals of Laboratory Medicine 2024;44(5):410-417
Background:
Carbapenem resistance in Pseudomonas aeruginosa is a serious global health problem. We investigated the clonal distribution and its association with the carbapenem resistance mechanisms of carbapenem-non-susceptible P. aeruginosa isolates from three Korean hospitals.
Methods:
A total of 155 carbapenem-non-susceptible P. aeruginosa isolates collected between 2011 and 2019 were analyzed for sequence types (STs), antimicrobial susceptibility, and carbapenem resistance mechanisms, including carbapenemase production, the presence of resistance genes, OprD mutations, and the hyperproduction of AmpC β-lactamase.
Results:
Sixty STs were identified in carbapenem-non-susceptible P. aeruginosa isolates.Two high-risk clones, ST235 (N = 41) and ST111 (N = 20), were predominant; however, sporadic STs were more prevalent than high-risk clones. The resistance rate to amikacin was the lowest (49.7%), whereas that to piperacillin was the highest (92.3%). Of the 155 carbapenem-non-susceptible isolates, 43 (27.7%) produced carbapenemases. Three metalloβ-lactamase (MBL) genes, blaIMP-6 (N = 38), blaVIM-2 (N = 3), and blaNDM-1 (N = 2), were detected. blaIMP-6 was detected in clonal complex 235 isolates. Two ST773 isolates carried blaNDM-1 and rmtB. Frameshift mutations in oprD were identified in all isolates tested, regardless of the presence of MBL genes. Hyperproduction of AmpC was detected in MBL gene–negative isolates.
Conclusions
Frameshift mutations in oprD combined with MBL production or hyperproduction of AmpC are responsible for carbapenem resistance in P. aeruginosa. Further attention is required to curb the emergence and spread of new carbapenem-resistant P. aeruginosa clones.
8.Clonal Distribution and Its Association With the Carbapenem Resistance Mechanisms of Carbapenem-Non-Susceptible Pseudomonas aeruginosa Isolates From Korean Hospitals
Nayeong KIM ; Seo Yeon KO ; Seong Yong PARK ; Seong Yeob KIM ; Da Eun LEE ; Ki Tae KWON ; Yu Kyung KIM ; Je Chul LEE
Annals of Laboratory Medicine 2024;44(5):410-417
Background:
Carbapenem resistance in Pseudomonas aeruginosa is a serious global health problem. We investigated the clonal distribution and its association with the carbapenem resistance mechanisms of carbapenem-non-susceptible P. aeruginosa isolates from three Korean hospitals.
Methods:
A total of 155 carbapenem-non-susceptible P. aeruginosa isolates collected between 2011 and 2019 were analyzed for sequence types (STs), antimicrobial susceptibility, and carbapenem resistance mechanisms, including carbapenemase production, the presence of resistance genes, OprD mutations, and the hyperproduction of AmpC β-lactamase.
Results:
Sixty STs were identified in carbapenem-non-susceptible P. aeruginosa isolates.Two high-risk clones, ST235 (N = 41) and ST111 (N = 20), were predominant; however, sporadic STs were more prevalent than high-risk clones. The resistance rate to amikacin was the lowest (49.7%), whereas that to piperacillin was the highest (92.3%). Of the 155 carbapenem-non-susceptible isolates, 43 (27.7%) produced carbapenemases. Three metalloβ-lactamase (MBL) genes, blaIMP-6 (N = 38), blaVIM-2 (N = 3), and blaNDM-1 (N = 2), were detected. blaIMP-6 was detected in clonal complex 235 isolates. Two ST773 isolates carried blaNDM-1 and rmtB. Frameshift mutations in oprD were identified in all isolates tested, regardless of the presence of MBL genes. Hyperproduction of AmpC was detected in MBL gene–negative isolates.
Conclusions
Frameshift mutations in oprD combined with MBL production or hyperproduction of AmpC are responsible for carbapenem resistance in P. aeruginosa. Further attention is required to curb the emergence and spread of new carbapenem-resistant P. aeruginosa clones.
9.Clonal Distribution and Its Association With the Carbapenem Resistance Mechanisms of Carbapenem-Non-Susceptible Pseudomonas aeruginosa Isolates From Korean Hospitals
Nayeong KIM ; Seo Yeon KO ; Seong Yong PARK ; Seong Yeob KIM ; Da Eun LEE ; Ki Tae KWON ; Yu Kyung KIM ; Je Chul LEE
Annals of Laboratory Medicine 2024;44(5):410-417
Background:
Carbapenem resistance in Pseudomonas aeruginosa is a serious global health problem. We investigated the clonal distribution and its association with the carbapenem resistance mechanisms of carbapenem-non-susceptible P. aeruginosa isolates from three Korean hospitals.
Methods:
A total of 155 carbapenem-non-susceptible P. aeruginosa isolates collected between 2011 and 2019 were analyzed for sequence types (STs), antimicrobial susceptibility, and carbapenem resistance mechanisms, including carbapenemase production, the presence of resistance genes, OprD mutations, and the hyperproduction of AmpC β-lactamase.
Results:
Sixty STs were identified in carbapenem-non-susceptible P. aeruginosa isolates.Two high-risk clones, ST235 (N = 41) and ST111 (N = 20), were predominant; however, sporadic STs were more prevalent than high-risk clones. The resistance rate to amikacin was the lowest (49.7%), whereas that to piperacillin was the highest (92.3%). Of the 155 carbapenem-non-susceptible isolates, 43 (27.7%) produced carbapenemases. Three metalloβ-lactamase (MBL) genes, blaIMP-6 (N = 38), blaVIM-2 (N = 3), and blaNDM-1 (N = 2), were detected. blaIMP-6 was detected in clonal complex 235 isolates. Two ST773 isolates carried blaNDM-1 and rmtB. Frameshift mutations in oprD were identified in all isolates tested, regardless of the presence of MBL genes. Hyperproduction of AmpC was detected in MBL gene–negative isolates.
Conclusions
Frameshift mutations in oprD combined with MBL production or hyperproduction of AmpC are responsible for carbapenem resistance in P. aeruginosa. Further attention is required to curb the emergence and spread of new carbapenem-resistant P. aeruginosa clones.
10.Clonal Distribution and Its Association With the Carbapenem Resistance Mechanisms of Carbapenem-Non-Susceptible Pseudomonas aeruginosa Isolates From Korean Hospitals
Nayeong KIM ; Seo Yeon KO ; Seong Yong PARK ; Seong Yeob KIM ; Da Eun LEE ; Ki Tae KWON ; Yu Kyung KIM ; Je Chul LEE
Annals of Laboratory Medicine 2024;44(5):410-417
Background:
Carbapenem resistance in Pseudomonas aeruginosa is a serious global health problem. We investigated the clonal distribution and its association with the carbapenem resistance mechanisms of carbapenem-non-susceptible P. aeruginosa isolates from three Korean hospitals.
Methods:
A total of 155 carbapenem-non-susceptible P. aeruginosa isolates collected between 2011 and 2019 were analyzed for sequence types (STs), antimicrobial susceptibility, and carbapenem resistance mechanisms, including carbapenemase production, the presence of resistance genes, OprD mutations, and the hyperproduction of AmpC β-lactamase.
Results:
Sixty STs were identified in carbapenem-non-susceptible P. aeruginosa isolates.Two high-risk clones, ST235 (N = 41) and ST111 (N = 20), were predominant; however, sporadic STs were more prevalent than high-risk clones. The resistance rate to amikacin was the lowest (49.7%), whereas that to piperacillin was the highest (92.3%). Of the 155 carbapenem-non-susceptible isolates, 43 (27.7%) produced carbapenemases. Three metalloβ-lactamase (MBL) genes, blaIMP-6 (N = 38), blaVIM-2 (N = 3), and blaNDM-1 (N = 2), were detected. blaIMP-6 was detected in clonal complex 235 isolates. Two ST773 isolates carried blaNDM-1 and rmtB. Frameshift mutations in oprD were identified in all isolates tested, regardless of the presence of MBL genes. Hyperproduction of AmpC was detected in MBL gene–negative isolates.
Conclusions
Frameshift mutations in oprD combined with MBL production or hyperproduction of AmpC are responsible for carbapenem resistance in P. aeruginosa. Further attention is required to curb the emergence and spread of new carbapenem-resistant P. aeruginosa clones.

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