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.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.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.