Cerebellar ataxia, neuropathy, and vestibular areflexia syndrome (CANVAS) is a neurodegenerative disorder caused by a biallelic expansion of pentanucleotide repeats in the RFC1 gene. Previous studies have reported up to 22% of patients with late-onset ataxia harbor this pathogenic repeat expansion. Despite its relatively high prevalence, CANVAS is often underdiagnosed because the disease is not well recognized and genetic testing is not performed in clinical practice. Here, we present a patient with characteristic clinical features, confirmed by genetic testing.

Background: Despite the critical importance of sleep medicine within neurology, notable gaps in education and clinical practice persist among neurologists. This study aims to explore the extent of involvement and the challenges faced by Korean neurologists in sleep medicine, focusing on awareness of polysomnography training program, the operation of sleep study facilities, and educational exposure. Methods: An online survey collected responses from 233 neurologists, focusing on their demographics, knowledge of and involvement in sleep medicine, operation of sleep study facilities, and participation in residency training. Results: The findings indicated that 84.9% of neurologists were aware of polysomnography training program, primarily through professional societies. Nonetheless, 15.1% reported unfamiliarity with these initiatives, with 72.7% of this subgroup expressing interest in sleep medicine yet lacking access to information. In terms of clinical practice, 74 neurologists operated sleep study facilities, with 63% intending to expand. Key operational challenges included staff management, maintaining patient volumes, and inadequate institutional support. Among respondents from teaching hospitals, only 36 out of 114 reported active resident involvement in sleep study interpretations, predominantly hindered by excessive workloads and insufficient staffing. Conclusions A significant number of neurologists have an interest in sleep medicine; however, substantial challenges impede effective education and clinical practice. These results underscore the need for improved educational resources and institutional support to enhance the growth and effectiveness of sleep medicine practices among neurologists.

Cerebellar ataxia, neuropathy, and vestibular areflexia syndrome (CANVAS) is a neurodegenerative disorder caused by a biallelic expansion of pentanucleotide repeats in the RFC1 gene. Previous studies have reported up to 22% of patients with late-onset ataxia harbor this pathogenic repeat expansion. Despite its relatively high prevalence, CANVAS is often underdiagnosed because the disease is not well recognized and genetic testing is not performed in clinical practice. Here, we present a patient with characteristic clinical features, confirmed by genetic testing.

Background: Despite the critical importance of sleep medicine within neurology, notable gaps in education and clinical practice persist among neurologists. This study aims to explore the extent of involvement and the challenges faced by Korean neurologists in sleep medicine, focusing on awareness of polysomnography training program, the operation of sleep study facilities, and educational exposure. Methods: An online survey collected responses from 233 neurologists, focusing on their demographics, knowledge of and involvement in sleep medicine, operation of sleep study facilities, and participation in residency training. Results: The findings indicated that 84.9% of neurologists were aware of polysomnography training program, primarily through professional societies. Nonetheless, 15.1% reported unfamiliarity with these initiatives, with 72.7% of this subgroup expressing interest in sleep medicine yet lacking access to information. In terms of clinical practice, 74 neurologists operated sleep study facilities, with 63% intending to expand. Key operational challenges included staff management, maintaining patient volumes, and inadequate institutional support. Among respondents from teaching hospitals, only 36 out of 114 reported active resident involvement in sleep study interpretations, predominantly hindered by excessive workloads and insufficient staffing. Conclusions A significant number of neurologists have an interest in sleep medicine; however, substantial challenges impede effective education and clinical practice. These results underscore the need for improved educational resources and institutional support to enhance the growth and effectiveness of sleep medicine practices among neurologists.

Background and Objectives: Among various emerging catheter-based treatments for severe tricuspid regurgitation (TR), the spacer device can reduce the regurgitation orifice without manipulating the valve leaflet. However, its clinical application has been hampered by traumatic anchoring to the myocardium and the coaxial alignment of the balloon resulting in insufficient TR reduction. This study aimed to evaluate the early-stage safety, technical feasibility, and preliminary efficacy of the novel atraumatic vertical spacer in patients with isolated severe TR. Methods: All procedures were guided by fluoroscopy and transthoracic echocardiography.The maximum device placement time with an inflated balloon was 24 hours. Changes in the amount of TR, right ventricular function, and patient hemodynamics were measured during balloon deployment. Results: A total of 7 patients (median age 74), underwent successful device implantation without procedure-related complications. During balloon inflation (median 25 minutes), there were no symptoms or signs indicative of TR intolerance. TR was reduced by 1 grade or greater in all patients, with 2 patients exhibiting a reduction of 3 grades, from torrential TR to a moderate degree. Mild TR after balloon inflation was achieved in 3 patients with baseline severe TR. The TR reduction observed during initial balloon deployment was sustained during the subsequent balloon maintenance period. Conclusions The Pivot-balloon procedure was safe, technically feasible, and effective in reducing TR in patients with severe TR. No periprocedural complications or adverse cardiovascular events were reported during device placement with TR reduction observed in all patients. However, longer-term follow-up is needed to confirm safety and treatment effect.

Background and Objectives: Outcomes of deferring percutaneous coronary intervention (PCI) without invasive physiologic assessment for intermediate coronary lesions is uncertain.We sought to compare long-term outcomes between medical treatment and PCI of intermediate lesions without invasive physiologic assessment. Methods: A total of 899 patients with intermediate coronary lesions between 50% and 70% diameter-stenosis were randomized to the conservative group (n=449) or the aggressive group (n=450). For intermediate lesions, PCI was performed in the aggressive group, but was deferred in the conservative group. The primary endpoint was major adverse cardiac events (MACE, a composite of all-cause death, myocardial infarction [MI], or ischemia-driven any revascularization) at 3 years. Results: The number of treated lesions per patient was 0.8±0.9 in the conservative group and 1.7±0.9 in the aggressive group (p=0.001). At 3 years, the conservative group had a significantly higher incidence of MACE than the aggressive group (13.8% vs. 9.3%; hazard ratio [HR], 1.49; 95% confidence interval [CI], 1.00–2.21; p=0.049), mainly driven by revascularization of target intermediate lesion (6.5% vs. 1.1%; HR, 5.69; 95% CI, 2.20–14.73;p<0.001). Between 1 and 3 years after the index procedure, compared to the aggressive group, the conservative group had significantly higher incidence of cardiac death or MI (3.2% vs.0.7%; HR, 4.34; 95% CI, 1.24–15.22; p=0.022) and ischemia-driven any revascularization. Conclusions For intermediate lesions, medical therapy alone, guided only by angiography, was associated with a higher risk of MACE at 3 years compared with performing PCI, mainly due to increased revascularization.

Background: We hypothesized that intranasal combination of dexmedetomidine (2 μg/kg) and ketamine (3 mg/kg) (IN DEXKET) improves the success rate of sedation in pediatric patients compared with chloral hydrate (CH; 50 mg/kg). Methods: This prospective, two-center, single-blinded, randomized controlled trial involved 136 pediatric patients (aged < 7 years) requiring procedural sedation. The participants were randomized to receive CH or IN DEXKET via a mucosal atomizer device. The primary outcome was the success rate of sedation (Pediatric Sedation State Scale, scores 1–3) within 15 min. The secondary outcomes included sedation failure at 30 min and overall complications of first-attempt sedation. Results: After excluding eight patients, 128 were included (CH = 66, IN DEXKET = 62). IN DEXKET showed a similar sedation success rate (75.8% [47/62] vs. 66.7% [44/66]; P = 0.330) but a lower complication rate (3.2% [2/62] vs. 16.7% [11/66]; P = 0.017) than CH. In the subgroup analysis for patients aged < 1 year, IN DEXKET showed a reduced complication rate than CH (2.6% [1/38] vs. 22.9% [8/35]; P = 0.012). In the subgroup analysis of children aged 1–7 years, IN DEXKET showed a higher sedation success rate within 15 min (79.2% [19/24] vs. 51.6% [16/31]; P = 0.049) and a lower sedation failure after 30 min (0% vs. 29.0% [9/31]; P = 0.003) than CH. Conclusions The intranasal combination of dexmedetomidine (2 μg/kg) and ketamine (3 mg/kg) is a safe and effective alternative to CH (50 mg/kg) for sedation in pediatric patients aged < 7 years.