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.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.
3.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.
4.Antiproliferative Activity of Piceamycin by Regulating Alpha-Actinin-4 in Gemcitabine-Resistant Pancreatic Cancer Cells
Jee-Hyung LEE ; Jin Ho CHOI ; Kyung-Min LEE ; Min Woo LEE ; Ja-Lok KU ; Dong-Chan OH ; Yern-Hyerk SHIN ; Dae Hyun KIM ; In Rae CHO ; Woo Hyun PAIK ; Ji Kon RYU ; Yong-Tae KIM ; Sang Hyub LEE ; Sang Kook LEE
Biomolecules & Therapeutics 2024;32(1):123-135
Although gemcitabine-based regimens are widely used as an effective treatment for pancreatic cancer, acquired resistance to gemcitabine has become an increasingly common problem. Therefore, a novel therapeutic strategy to treat gemcitabine-resistant pancreatic cancer is urgently required. Piceamycin has been reported to exhibit antiproliferative activity against various cancer cells; however, its underlying molecular mechanism for anticancer activity in pancreatic cancer cells remains unexplored. Therefore, the present study evaluated the antiproliferation activity of piceamycin in a gemcitabine-resistant pancreatic cancer cell line and patient-derived pancreatic cancer organoids. Piceamycin effectively inhibited the proliferation and suppressed the expression of alpha-actinin-4, a gene that plays a pivotal role in tumorigenesis and metastasis of various cancers, in gemcitabine-resistant cells. Long-term exposure to piceamycin induced cell cycle arrest at the G0/G1 phase and caused apoptosis. Piceamycin alsoinhibited the invasion and migration of gemcitabine-resistant cells by modulating focal adhesion and epithelial-mesenchymal transition biomarkers. Moreover, the combination of piceamycin and gemcitabine exhibited a synergistic antiproliferative activity in gemcitabine-resistant cells. Piceamycin also effectively inhibited patient-derived pancreatic cancer organoid growth and induced apoptosis in the organoids. Taken together, these findings demonstrate that piceamycin may be an effective agent for overcoming gemcitabine resistance in pancreatic cancer.
5.Sex Differences in Chronic Cough Epidemiology: The Korean Cough Study Group
Jiyeon KANG ; Woo Jung SEO ; Jieun KANG ; Jung Gon KIM ; Sung Jun CHUNG ; Hyung Koo KANG ; Sung-Soon LEE ; Tai Joon AN ; Hyonsoo JOO ; Hyun LEE ; Youlim KIM ; Ina JEONG ; Jinkyeong PARK ; Sung-Kyoung KIM ; Jong-Wook SHIN ; Chin Kook RHEE ; Yee Hyung KIM ; Kyung Hoon MIN ; Ji-Yong MOON ; Deog Kyeom KIM ; Seung Hun JANG ; Kwang Ha YOO ; Jin Woo KIM ; Hyoung Kyu YOON ; Hyeon-Kyoung KOO
Journal of Korean Medical Science 2024;39(38):e273-
Background:
Chronic cough is a common symptom encountered by healthcare practitioners.The global prevalence of chronic cough is 9.6%, with a female predominance. The aim of our study is to reveal the sex differences in prevalence and severity of chronic cough in South Korea, stratified by age and etiology.
Methods:
This study included adult patients with chronic cough who were recruited from 19 respiratory centers in South Korea. Patients completed the cough numeric rating scale (NRS) and COugh Assessment Test (COAT) questionnaire to assess the severity and multidimensional impact of cough.
Results:
Among the 625 patients, 419 (67.0%) were females, with a male-to-female ratio of 1:2.03. The mean age was 49.4 years, and the median duration of cough was 12 weeks. The mean NRS and COAT scores were 5.5 ± 1.8 and 9.5 ± 3.6, respectively. Female patients were older (45.3 ± 15.4 vs. 51.6 ± 15.2, P < 0.001) and more likely to have asthma/cough variant asthma (CVA) (26.7% vs. 40.8%, P = 0.001) than male patients. There was no difference in the duration or severity of cough between sexes, regardless of the cause. The male-tofemale ratio was lower for upper airway cough syndrome (UACS), asthma/CVA, and gastroesophageal reflux disease (GERD), but not for eosinophilic bronchitis (EB) or unexplained cough. The mean age of female patients was higher in UACS and asthma/CVA, but not in EB, GERD, or unexplained cough. The majority (24.2%) fell within the age category of 50s. The proportion of females with cough increased with age, with a significant rise in the 50s, 60s, and 70–89 age groups. The severity of cough decreased in the 50s, 60s, and 70–89 age groups, with no significant sex differences within the same age group.
Conclusion
The sex disparities in prevalence and severity of cough varied significantly depending on the age category and etiology. Understanding the specific sex-based difference could enhance comprehension of cough-related pathophysiology and treatment strategies.
6.Telemedicine Protocols for the Management of Patients with Acute Spontaneous Intracerebral Hemorrhage in Rural and Medically Underserved Areas in Gangwon State : Recommendations for Doctors with Less Expertise at Local Emergency Rooms
Hyo Sub JUN ; Kuhyun YANG ; Jongyeon KIM ; Jin Pyeong JEON ; Sun Jeong KIM ; Jun Hyong AHN ; Seung Jin LEE ; Hyuk Jai CHOI ; In Bok CHANG ; Jeong Jin PARK ; Jong-Kook RHIM ; Sung-Chul JIN ; Sung Min CHO ; Sung-Pil JOO ; Seung Hun SHEEN ; Sang Hyung LEE ;
Journal of Korean Neurosurgical Society 2024;67(4):385-396
Previously, we reported the concept of a cloud-based telemedicine platform for patients with intracerebral hemorrhage (ICH) at local emergency rooms in rural and medically underserved areas in Gangwon state by combining artificial intelligence and remote consultation with a neurosurgeon. Developing a telemedicine ICH treatment protocol exclusively for doctors with less ICH expertise working in emergency rooms should be part of establishing this system. Difficulties arise in providing appropriate early treatment for ICH in rural and underserved areas before the patient is transferred to a nearby hub hospital with stroke specialists. This has been an unmet medical need for decade. The available reporting ICH guidelines are realistically possible in university hospitals with a well-equipped infrastructure. However, it is very difficult for doctors inexperienced with ICH treatment to appropriately select and deliver ICH treatment based on the guidelines. To address these issues, we developed an ICH telemedicine protocol. Neurosurgeons from four university hospitals in Gangwon state first wrote the guidelines, and professors with extensive ICH expertise across the country revised them. Guidelines and recommendations for ICH management were described as simply as possible to allow more doctors to use them easily. We hope that our effort in developing the telemedicine protocols will ultimately improve the quality of ICH treatment in local emergency rooms in rural and underserved areas in Gangwon state.
7.Triglyceride-glucose index is an independent predictor of coronary artery calcification progression in patients with chronic kidney disease
Ye Eun KO ; Hyung Woo KIM ; Jung Tak PARK ; Seung Hyeok HAN ; Shin-Wook KANG ; Suah SUNG ; Kyu-Beck LEE ; Joongyub LEE ; Kook-Hwan OH ; Tae-Hyun YOO ;
Kidney Research and Clinical Practice 2024;43(3):381-390
Coronary artery calcification (CAC) is highly prevalent in patients with chronic kidney disease (CKD) and is associated with major adverse cardiovascular events and metabolic disturbances. The triglyceride-glucose index (TyGI), a novel surrogate marker of metabolic syndrome and insulin resistance, is associated with CAC in the general population and in patients with diabetes. This study investigated the association between the TyGI and CAC progression in patients with CKD, which is unknown. Methods: A total of 1,154 patients with CKD (grades 1–5; age, 52.8 ± 11.9 years; male, 688 [59.6%]) were enrolled from the KNOWCKD (KoreaN Cohort Study for Outcomes in Patients With Chronic Kidney Disease). The TyGI was calculated as follows: ln (fasting triglycerides × fasting glucose/2). Patients were classified into tertiles (low, intermediate, high) based on the TyGI. The primary outcome was annualized percentage change in CAC score [(percent change in CAC score + 1)12/follow-up months – 1] of ≥15%, defined as CAC progression. Results: During the 4-year follow-up, the percentage of patients with CAC progression increased across TyGI groups (28.6%, 37.5%, and 46.2% in low, intermediate, and high groups, respectively; p < 0.001). A high TyGI was associated with an increased risk of CAC progression (odds ratio [OR], 2.11; 95% confidence interval [CI], 1.14–3.88; p = 0.02) compared to the low group. Moreover, a 1-point increase in the TyGI was related to increased risk of CAC progression (OR, 1.55; 95% CI, 1.06–1.76; p = 0.02) after adjustment. Conclusion: A high TyGI may be a useful predictor of CAC progression in CKD.
8.Public Perception Towards Drug Abuse in South Korea: The Effects of Overconfidence and Affirmation
Ki Won JANG ; Jiheon KIM ; Han Wool JUNG ; Sang-Kyu LEE ; Byung Joo PARK ; Hoon-Chul KANG ; Chan-Hyung KIM ; Hae Kook LEE ; Daeyoung ROH
Psychiatry Investigation 2024;21(7):746-754
Objective:
The abuse of prescription drugs and over-the-counter medicines has been a major issue addressed as a serious public health problem worldwide. This study explored factors contributing to substance abuse in Korea by examining the status of substance abuse among Korean adults and evaluating their knowledge, attitudes, and intentions toward substance abuse.
Methods:
Data were collected online from a sample of participants 19 years old or older from May 20 to June 1, 2020 (n=1,020). The survey consisted of questions on demographics, perceptions of drug risk, motives for drug use, and attitudes toward drug addiction treatment. Principal component and multiple logistic regression analyses were used to explore the factors contributing to the perception of drug abuse.
Results:
In the multivariate regression analysis, overconfidence in handling drug usage, acceptance of addictive substances, and affirmation of public support for drug abuse were associated with opioid abuse (Nagelkerke R2=0.486), and additionally affirmation of legal cannabis usage and motivation to use diet pills were associated with diet pill abuse (Nagelkerke R2=0.569).
Conclusion
The findings of this study suggest that the actual situation of substance abuse among Korean adults increases awareness of and attitudes toward drug use related to substance abuse.
9.Effect of COVID-19 (SARS-CoV-2) Vaccination on Patients with Atopic Dermatitis Treated with Dupilumab: A Multicenter, Observational Study
Tae Young HAN ; Dong Hyun SHIM ; Yu Jin LEE ; Young Bok LEE ; Ha Yeh Rin KOO ; Min Kyung SHIN ; Tae Eun KIM ; Yong Hyun JANG ; Jin Seon BANG ; Hyung Don KOOK ; Jiyoung AHN ; Hye Jung JUNG ; Chan Ho NA
Annals of Dermatology 2023;35(1):38-45
Background:
Atopic dermatitis (AD) patients usually wonder if their condition will worsen after vaccination or if they should continue with the treatment they are receiving. Considering that many patients treated with dupilumab had previously experienced severe AD symptoms and flares, the concerns are more understandable.
Objective:
This study aimed to investigate the safety of the coronavirus disease 2019 (COVID-19) vaccination in patients with AD treated with dupilumab.
Methods:
We enrolled 133 patients (101 dupilumab-treated and 32 systemic oral agentstreated as control group) with AD from six hospitals. Patients were asked about worsening pruritus and AD (5-point Likert scale) after vaccination. AD variables (eczema area and severity index [EASI], investigator’s global assessment [IGA], itch numerical rating scale [NRS], sleep NRS, and patient-oriented eczema measure [POEM]) were compared pre- and postvaccination. Adverse reactions to the COVID-19 vaccination were observed.
Results:
The incidence of adverse reactions to COVID-19 vaccines and worsening AD symptoms in dupilumab-treated patients were not significantly different compared with that in the control group. The itch NRS score increased significantly after vaccination (p<0.001).However, there were no statistically significant differences between the pre-and post-EASI, IGA, and POEM scores. Eight patients (7.9%) had worse EASI scores and required rescue therapy; however, most were easily managed with low-dose steroids or topical agents. None of the patients discontinued dupilumab treatment.
Conclusion
No serious adverse reactions were observed in patients with AD after COVID-19 vaccination. Exacerbation of pruritus and AD symptoms was observed but was mostly mild and transient.
10.Safety and Efficacy of Everolimus-Eluting Bioresorbable Vascular Scaffold Versus Second-Generation Drug-Eluting Stents in Real-World Practice
Joo Myung LEE ; Hyun Sung JOH ; Ki Hong CHOI ; David HONG ; Taek Kyu PARK ; Jeong Hoon YANG ; Young Bin SONG ; Jin-Ho CHOI ; Seung-Hyuk CHOI ; Jin-Ok JEONG ; Jong-Young LEE ; Young Jin CHOI ; Jei-Keon CHAE ; Seung-Ho HUR ; Jang-Whan BAE ; Ju-Hyeon OH ; Kook-Jin CHUN ; Hyun-Joong KIM ; Byung Ryul CHO ; Doosup SHIN ; Seung Hun LEE ; Doyeon HWANG ; Hyun-Jong LEE ; Ho-Jun JANG ; Hyun Kuk KIM ; Sang Jin HA ; Eun-Seok SHIN ; Joon-Hyung DOH ; Joo-Yong HAHN ; Hyeon-Cheol GWON ; On behalf of the SMART-REWARD Investigators
Journal of Korean Medical Science 2023;38(5):e34-
Background:
The risk of device thrombosis and device-oriented clinical outcomes with bioresorbable vascular scaffold (BVS) was reported to be significantly higher than with contemporary drug-eluting stents (DESs). However, optimal device implantation may improve clinical outcomes in patients receiving BVS. The current study evaluated mid-term safety and efficacy of Absorb BVS with meticulous device optimization under intravascular imaging guidance.
Methods:
The SMART-REWARD and PERSPECTIVE-PCI registries in Korea prospectively enrolled 390 patients with BVS and 675 patients with DES, respectively. The primary endpoint was target vessel failure (TVF) at 2 years and the secondary major endpoint was patientoriented composite outcome (POCO) at 2 years.
Results:
Patient-level pooled analysis evaluated 1,003 patients (377 patients with BVS and 626 patients with DES). Mean scaffold diameter per lesion was 3.24 ± 0.30 mm in BVS group.Most BVSs were implanted with pre-dilatation (90.9%), intravascular imaging guidance (74.9%), and post-dilatation (73.1%) at proximal to mid segment (81.9%) in target vessel.Patients treated with BVS showed comparable risks of 2-year TVF (2.9% vs. 3.7%, adjusted hazard ratio [HR], 1.283, 95% confidence interval [CI], 0.487–3.378, P = 0.615) and 2-year POCO (4.5% vs. 5.9%, adjusted HR, 1.413, 95% CI, 0.663–3.012,P = 0.370) than those with DES. The rate of 2-year definite or probable device thrombosis (0.3% vs. 0.5%, P = 0.424) was also similar. The sensitivity analyses consistently showed comparable risk of TVF and POCO between the 2 groups.
Conclusion
With meticulous device optimization under imaging guidance and avoidance of implantation in small vessels, BVS showed comparable risks of 2-year TVF and device thrombosis with DES.

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