Reliability and Validity of the Korean version of the Center for Neurologic Study Bulbar Function Scale (K-CNS-BFS): An observational study.

Bulbar dysfunction in amyotrophic lateral sclerosis (ALS) significantly affects daily life, leading to weight loss and reduced survival. Methods for evaluating bulbar dysfunction, including videofluoroscopic swallowing studies and the bulbar component of the ALS Functional Rating Scale-Revised (ALSFRS-R), have been employed; however, Korean-specific tools are lacking. The Center for Neurologic Study Bulbar Function Scale (CNS-BFS) comprehensively evaluates bulbar symptoms. This study aimed to develop and validate the Korean version of the CNS-BFS (K-CNS-BFS) to assess bulbar dysfunction in Korean patients with ALS. Twenty-seven patients with ALS were recruited from a tertiary hospital in South Korea based on revised El Escorial criteria. Demographic, clinical, and measurement data were collected. The K-CNS-BFS was evaluated for reliability and validity. Reliability assessment revealed strong internal consistency (Cronbach alpha) for the K-CNS-BFS subscales and total score. Test-retest reliability showed significant correlation. Content validity index was excellent, and convergent validity demonstrated significant correlations between the K-CNS-BFS and relevant measures. Discriminant validity was observed between the K-CNS-BFS and motor/respiratory subscores of the ALSFRS-R. Construct validity demonstrated significant correlations between the K-CNS-BFS subscales and total score. This is the first study to investigate the reliability and validity of the Korean version of the CNS-BFS, which showed consistent and reliable scores that correlated with tests for bulbar or general dysfunction. The K-CNS-BFS effectively measured bulbar dysfunction similar to the original CNS-BFS. The K-CNS-BFS is a reliable and valid tool for assessing bulbar dysfunction in patients with ALS in South Korea.

Mediating role of health literacy in relationship between frailty and health-related quality of life in community-dwelling older adults.

PURPOSE: The aim of this study was to investigate the mediating effects of health literacy on the relationship between frailty and health-related quality of life (HRQOL) among community-dwelling older adults. METHODS: This study used the Korean Frailty and Aging Cohort Database (KFACD) for secondary data analysis. We selected data from 1,631 people without missing main variable values for analysis. Frailty was determined based on the modified Fried's phenotype [MFP], and HRQOL was measured using the Korean version of the 5-level EuroQol questionnaire (EQ-5D-5L). Health literacy was assessed using the questions on the Behavioral Risk Factor Surveillance System (BRFSS) used by the U.S. Center for Disease Control and Prevention. To examine the mediating role of health literacy in the relationship between frailty and HRQOL, Baron & Kenny's three-step mediating effect verification method was utilized. RESULTS: The participants had a mean frailty score of 1.37±1.02, health literacy score of 8.56±2.59, and HRQOL score of 0.84±0.10. Frailty was negatively correlated with health literacy (r = -0.27, p < .001) and HRQOL (r = -0.32, p < .001), while health literacy was positively correlated with HRQOL (r = 0.34, p < .001). We observed that health literacy played a partial mediating role in the relationship between frailty and HRQOL. CONCLUSION: To increase older adults' HRQOL, measures that directly prevent and manage frailty as well as interventions that target the enhancement of health literacy are needed.

Detection of Acute Brain Injury in Intensive Care Unit Patients on ECMO Support Using Ultra-Low-Field Portable MRI: A Retrospective Analysis Compared to Head CT.

Early detection of acute brain injury (ABI) is critical to intensive care unit (ICU) patient management and intervention to decrease major complications. Head CT (HCT) is the standard of care for the assessment of ABI in ICU patients; however, it has limited sensitivity compared to MRI. We retrospectively compared the ability of ultra-low-field portable MR (ULF-pMR) and head HCT, acquired within 24 h of each other, to detect ABI in ICU patients supported on extracorporeal membrane oxygenation (ECMO). A total of 17 adult patients (median age 55 years; 47% male) were included in the analysis. Of the 17 patients assessed, ABI was not observed on either ULF-pMR or HCT in eight patients (47%). ABI was observed in the remaining nine patients with a total of 10 events (8 ischemic, 2 hemorrhagic). Of the eight ischemic events, ULF-pMR observed all eight, while HCT only observed four events. Regarding hemorrhagic stroke, ULF-pMR observed only one of them, while HCT observed both. ULF-pMR outperformed HCT for the detection of ABI, especially ischemic injury, and may offer diagnostic advantages for ICU patients. The lack of sensitivity to hemorrhage may improve with modification of the imaging acquisition program.

Clinical Use of Bedside Portable Low-field Brain Magnetic Resonance Imaging in Patients on ECMO: The Results from Multicenter SAFE MRI ECMO Study.

Purpose: Early detection of acute brain injury (ABI) is critical for improving survival for patients with extracorporeal membrane oxygenation (ECMO) support. We aimed to evaluate the safety of ultra-low-field portable MRI (ULF-pMRI) and the frequency and types of ABI observed during ECMO support. Methods: We conducted a multicenter prospective observational study (NCT05469139) at two academic tertiary centers (August 2022-November 2023). Primary outcomes were safety and validation of ULF-pMRI in ECMO, defined as exam completion without adverse events (AEs); secondary outcomes were ABI frequency and type. Results: ULF-pMRI was performed in 50 patients with 34 (68%) on venoarterial (VA)-ECMO (11 central; 23 peripheral) and 16 (32%) with venovenous (VV)-ECMO (9 single lumen; 7 double lumen). All patients were imaged successfully with ULF-pMRI, demonstrating discernible intracranial pathologies with good quality. AEs occurred in 3 (6%) patients (2 minor; 1 serious) without causing significant clinical issues.ABI was observed in ULF-pMRI scans for 22 patients (44%): ischemic stroke (36%), intracranial hemorrhage (6%), and hypoxic-ischemic brain injury (4%). Of 18 patients with both ULF-pMRI and head CT (HCT) within 24 hours, ABI was observed in 9 patients with 10 events: 8 ischemic (8 observed on ULF-oMRI, 4 on HCT) and 2 hemorrhagic (1 observed on ULF-pMRI, 2 on HCT). Conclusions: ULF-pMRI was shown to be safe and valid in ECMO patients across different ECMO cannulation strategies. The incidence of ABI was high, and ULF-pMRI may more sensitive to ischemic ABI than HCT. ULF-pMRI may benefit both clinical care and future studies of ECMO-associated ABI.

Hypoxic-Ischemic Brain Injury in ECMO: Pathophysiology, Neuromonitoring, and Therapeutic Opportunities.

Extracorporeal membrane oxygenation (ECMO), in conjunction with its life-saving benefits, carries a significant risk of acute brain injury (ABI). Hypoxic-ischemic brain injury (HIBI) is one of the most common types of ABI in ECMO patients. Various risk factors, such as history of hypertension, high day 1 lactate level, low pH, cannulation technique, large peri-cannulation PaCO2 drop (∆PaCO2), and early low pulse pressure, have been associated with the development of HIBI in ECMO patients. The pathogenic mechanisms of HIBI in ECMO are complex and multifactorial, attributing to the underlying pathology requiring initiation of ECMO and the risk of HIBI associated with ECMO itself. HIBI is likely to occur in the peri-cannulation or peri-decannulation time secondary to underlying refractory cardiopulmonary failure before or after ECMO. Current therapeutics target pathological mechanisms, cerebral hypoxia and ischemia, by employing targeted temperature management in the case of extracorporeal cardiopulmonary resuscitation (eCPR), and optimizing cerebral O2 saturations and cerebral perfusion. This review describes the pathophysiology, neuromonitoring, and therapeutic techniques to improve neurological outcomes in ECMO patients in order to prevent and minimize the morbidity of HIBI. Further studies aimed at standardizing the most relevant neuromonitoring techniques, optimizing cerebral perfusion, and minimizing the severity of HIBI once it occurs will improve long-term neurological outcomes in ECMO patients.

Deep learning enables fast, gentle STED microscopy.

STED microscopy is widely used to image subcellular structures with super-resolution. Here, we report that restoring STED images with deep learning can mitigate photobleaching and photodamage by reducing the pixel dwell time by one or two orders of magnitude. Our method allows for efficient and robust restoration of noisy 2D and 3D STED images with multiple targets and facilitates long-term imaging of mitochondrial dynamics.

Development of a deep learning system to detect glaucoma using macular vertical optical coherence tomography scans of myopic eyes.

Myopia is one of the risk factors for glaucoma, making accurate diagnosis of glaucoma in myopic eyes particularly important. However, diagnosis of glaucoma in myopic eyes is challenging due to the frequent associations of distorted optic disc and distorted parapapillary and macular structures. Macular vertical scan has been suggested as a useful tool to detect glaucomatous retinal nerve fiber layer loss even in highly myopic eyes. The present study was performed to develop and validate a deep learning (DL) system to detect glaucoma in myopic eyes using macular vertical optical coherence tomography (OCT) scans and compare its diagnostic power with that of circumpapillary OCT scans. The study included a training set of 1416 eyes, a validation set of 471 eyes, a test set of 471 eyes, and an external test set of 249 eyes. The ability to diagnose glaucoma in eyes with large myopic parapapillary atrophy was greater with the vertical than the circumpapillary OCT scans, with areas under the receiver operating characteristic curves of 0.976 and 0.914, respectively. These findings suggest that DL artificial intelligence based on macular vertical scans may be a promising tool for diagnosis of glaucoma in myopic eyes.

Retinal Nerve Fiber Layer Defect Associated With Progressive Myopia.

PURPOSE: The purpose of this study was to report a case with bilateral paravascular inner retinal defects (PIRDs) enlarging with progressive myopia, which had different structural characteristics from the glaucomatous retinal nerve fiber layer (RNFL) defect. PATIENTS AND METHODS: A 10-year-old girl with high myopia was referred to a glaucoma clinic for evaluation of RNFL defects shown in color fundus photographs. Fundus photographs and optical coherence tomography examinations were serially reviewed to examine the changes in the RNFL. RESULTS: Cleavage of inner retinal layers involving the layers deeper than the RNFL was identified in optical coherence tomography, which appeared and enlarged in both eyes along with progressive myopia and axial elongation during the follow-up period of 8 years. CONCLUSIONS: PIRD developed and enlarged with progressive myopia and axial elongation in childhood. It should be differentiated from the widening of RNFL defect shown with glaucoma progression.

Deep learning enables fast, gentle STED microscopy.

STED microscopy is widely used to image subcellular structures with super-resolution. Here, we report that denoising STED images with deep learning can mitigate photobleaching and photodamage by reducing the pixel dwell time by one or two orders of magnitude. Our method allows for efficient and robust restoration of noisy 2D and 3D STED images with multiple targets and facilitates long-term imaging of mitochondrial dynamics.

Impact of peripapillary retinoschisis on visual field test results in glaucomatous eyes.

BACKGROUND/AIMS: To investigate the influence of peripapillary retinoschisis (PRS) on visual field (VF) test results in patients with primary open angle glaucoma (POAG). METHODS: Thirty eyes of 30 patients with POAG who had PRS at least once were included. All eyes were followed-up for a minimum 5 years at 4-6-month intervals. The occurrence of PRS was determined by circumpapillary retinal nerve fibre layer B-scan on spectral-domain optical coherence tomography (OCT). The global and regional VF deviations just prior to and immediately after PRS formation, or just prior to and immediately after PRS resolution (if it occurred), were compared. VF sensitivity within the region corresponding to the OCT sector where PRS occurred was determined according to the Garway-Heath map. RESULTS: Global MD (p=0.345) and regional VF deviations (p=0.255) did not differ significantly between immediately after and just prior to PRS formation. Global MD (p=0.846) and regional VF deviations (p=0.758) were also similar between immediately after and just prior to PRS resolution. CONCLUSION: PRS has no short-term effect on the VF sensitivity. Therefore, VF test can still be useful for evaluating glaucomatous damage even in the presence of PRS compared with OCT.

Difference in topographic morphology of optic nerve head and neuroretinal rim between normal tension glaucoma and central retinal artery occlusion.

Although central retinal artery occlusion (CRAO) has its own defining pathomechanism and clinical characteristics, morphologic feature of the optic nerve head (ONH) during its later stage is not diagnostic, which makes it difficult to differentiate CRAO from other optic neuropathies. This cross-sectional study was performed to investigate the differences in the topographic morphology of the ONH in eyes with normal-tension glaucoma (NTG) and CRAO. Thirty-one eyes with NTG; 31 eyes with CRAO; and 31 healthy fellow eyes of the subjects with CRAO were included. ONH morphology was evaluated by measuring horizontal rim width (HRW), minimal rim width in the selected horizontal image (MRW), and lamina cribrosa curvature index (LCCI) in horizontal B-scan images obtained using enhanced depth-imaging optical coherence tomography. HRW was smaller and LCCI was larger in NTG eyes than in both CRAO and healthy fellow eyes (both P < 0.001), while both were comparable between CRAO and healthy fellow eyes. MRW differed significantly among the three groups, being smallest in NTG eyes followed by CRAO and healthy fellow eyes (P < 0.001). NTG and CRAO eyes with a similar degree of RNFL loss differed in ONH morphology, indicating that mechanisms of ONH damage differ between these two conditions.

Identification and clinical characterization of Charcot-Marie-Tooth disease type 1C patients with LITAF p.G112S mutation.

BACKGROUND: Charcot-Marie-Tooth disease type 1C (CMT1C) is a rare subtype associated with LITAF gene mutations. Until now, only a few studies have reported the clinical features of CMT1C. OBJECTIVE: This study was performed to find CMT1C patients with mutation of LITAF in a Korean CMT cohort and to characterize their clinical features. METHODS: In total, 1,143 unrelated Korean families with CMT were enrolled in a cohort. We performed whole exome sequencing to identify LITAF mutations, and examined clinical phenotypes including electrophysiological and MRI features for the identified CMT1C patients. RESULTS: We identified 10 CMT1C patients from three unrelated families with p.G112S mutation in LITAF. The frequency of CMT1C among CMT1 patients was 0.59%, which is similar to reports from Western populations. CMT1C patients showed milder symptoms than CMT1A patients. The mean CMT neuropathy score version 2 was 7.7, and the mean functional disability scale was 1.0. Electrophysiological findings showed a conduction block in 22% of affected individuals. Lower extremity MRIs showed that the superficial posterior and anterolateral compartments of the calf were predominantly affected. CONCLUSIONS: We found a conduction block in Korean CMT1C patients with p.G112S mutation and first described the characteristic MRI findings of the lower extremities in patients with LITAF mutation. These findings will be helpful for genotype-phenotype correlation and will widen understanding about the clinical spectrum of CMT1C.

PERIPAPILLARY MICROVASCULATURE OF THE RETINA AND CHORIOCAPILLARIS IN UNINVOLVED FELLOW EYES OF UNILATERAL RETINAL VEIN OCCLUSION PATIENTS.

PURPOSE: To investigate peripapillary microvascularity of the retina and choriocapillaris in uninvolved fellow eyes of patients with branch retinal vein occlusion (RVO) compared with that of healthy controls. METHODS: Peripapillary optical coherence tomography angiography bilateral eye scans of 46 patients with unilateral RVO and 46 age-matched control eyes were reviewed. Microvascular density in radial peripapillary capillary plexus (RPCP), superficial capillary plexus, and deep capillary plexus and signal void parameters in choriocapillaris were assessed. Data were statistically adjusted for hypertension. RESULTS: Microvascular densities in the RPCP and superficial capillary plexus were lower in the fellow eyes of patients with RVO than in the controls (RPCP, P = 0.021; superficial capillary plexus, P = 0.040). There were fewer choriocapillaris signal voids in the fellow eyes than in the control (P = 0.043). In patients with a duration of RVO onset >12 months (n = 27), all signal void parameters were lower in the fellow eyes than in the control eyes (size, P = 0.03; number, P = 0.046; proportion of the total area, P = 0.029). CONCLUSION: Uninvolved fellow eyes of patients with unilateral RVO showed microvascular impairment in the retina and choriocapillaris, even after adjusting for age and hypertension, suggesting that an unknown systemic factor might affect both the affected and unaffected eyes.

Brighter spotty lesions on spinal MRI help differentiate AQP4 antibody-positive NMOSD from MOGAD.

In a large acute myelitis cohort, we aimed to determine whether brighter spotty lesions (BSLs)-using the refined terminology-on spinal magnetic resonance imaging (MRI) help distinguish aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (AQP4-NMOSD) from myelin oligodendrocyte glycoprotein antibody disease (MOGAD). An experienced neuro-radiologist and two neurologists independently analyzed 133 spinal MRI scans (65 from MOGAD and 68 from AQP4-NMOSD) acquired within 1 month of attacks. BSLs were observed in 18 of 61 (30%) participants with AQP4-NMOSD, while none of 49 participants with MOGAD showed BSL (p < 0.001). BSL during the acute phase would be useful to differentiate AQP4-NMOSD from MOGAD.

Association between corneal refractive surgery and the prevalence of glaucoma: Korea National Health and Nutrition Examination Survey 2010-2012.

AIM: To determine the relationship between corneal refractive surgery and the prevalence of glaucoma in the Korean population. METHODS: Data were obtained from the Korea National Health and Nutrition Examination Survey (KNHANES), a population-based cross-sectional study using a complex, stratified, multistage, probability-cluster survey. This study included 604 eyes that had undergone myopic corneal refractive surgery, and 3389 control eyes without a history of any ocular surgery and having a spherical equivalent (SE) <-3.00 D, obtained from the KNHANES database for the years 2010-2012. Glaucoma diagnosis was based on the International Society of Geographical and Epidemiological Ophthalmology criteria. The association between a history of corneal refractive surgery and the prevalence of glaucoma was analysed using logistic regression analysis, after adjusting for potential confounding factors. RESULTS: Glaucoma prevalence did not differ between eyes that had and had not undergone corneal refractive surgery (p=0.675). After adjusting for age, sex, SE, and intraocular pressure, multivariate logistic regression analysis found that corneal refractive surgery was significantly associated with an increased risk of glaucoma (OR 9.14, p=0.002; 95% CI 2.22 to 37.69). Subgroup analysis that only included control eyes with a refraction cut-off <-3.70 D found that corneal refractive surgery was not significantly associated with glaucoma. CONCLUSIONS: History of corneal refractive surgery was associated with a higher prevalence of glaucoma in the Korean population. However, this association was not observed in eyes with a higher degree of myopia.

Analysis of microRNA signatures in ischemic stroke thrombus.

BACKGROUND: We investigated the microRNA expression pattern from thrombus retrieved by mechanical thrombectomy in acute stroke patients to understand the stroke mechanism. METHODS: This study included acute ischemic stroke patients who had undergone intra-arterial thrombectomy at Chung-Ang University Hospital in Seoul, Korea between February 2016 and March 2019. The thrombus was retrieved and stored at -70℃ after obtaining informed consent. MicroRNA microarray analysis was performed for the patients with identified stroke mechanisms including (1) large artery atherosclerosis, (2) cardioembolism with atrial fibrillation, and (3) cardioembolism with valvular heart disease. The microRNAs derived from microarray analysis were validated by quantitative real-time polymerase chain reaction (qRT-PCR) from different patient populations. The correlation analysis was performed between microRNA levels and laboratory data to understand the functional relevance of the altered microRNA. RESULTS: In total, 55 thrombi were obtained from 74 patients, and the microRNAs were analyzed in 45 samples. Microarray analysis of 2578 microRNAs revealed that 50 microRNAs were significantly altered among the three groups. Validation using qRT-PCR showed that miR-378f and miR-450b-5p were significantly elevated among the cardioembolic thrombi; both microRNAs were inversely correlated with the ejection fraction from echocardiography. Thrombi from patients with early neurological deterioration exhibited higher levels of miR-93-5p and lower levels of miR-629-5p than those from neurologically stable patients. CONCLUSIONS: The microRNA expression pattern can provide information regarding the mechanism of stroke by reflecting the underlying pathological status of the organ from which the thrombus was derived.

Adverse Events with the Pfizer-BioNTech COVID-19 Vaccine among Korean Healthcare Workers.

The Pfizer-BioNTech COVID-19 vaccine has shown excellent clinical effectiveness; however, adverse events of the vaccine remain a concern in Korea. We surveyed adverse events in 2498 healthcare workers vaccinated with the Pfizer-BioNTech COVID-19 vaccine at a university hospital. The survey was conducted using a diary card for 7 days following each injection. The questionnaire response rate was 75.1% (1876/2498) for the first dose and 73.8% (1840/2493) for the second dose. Among local reactions, pain was the most commonly reported (84.9% after the first dose and 90.4% after the second dose). After the second dose, two people visited the emergency room due to severe local pain, but no hospitalization or skin necrosis occurred. Among systemic reactions, fatigue was most frequently reported (52.8% after the first dose and 77.0% after the second dose), followed by myalgia (49.0% and 76.1%), headache (28.7% and 59.2%), chills (16.7% and 54.0%), and arthralgia (11.4% and 39.2%). One or more critical adverse events occurred in 0.2% and 0.7% of the vaccinees. Except for urticaria, more adverse events were reported after the second dose than after the first dose. In the future, adverse events should be investigated in older adults, and a future study with a longer observation period should be conducted.

Comparison of Optic Nerve Head Microvasculature Between Normal-Tension Glaucoma and Nonarteritic Anterior Ischemic Optic Neuropathy.

Purpose: To compare the microvasculature of the optic nerve head (ONH) and peripapillary tissues in eyes with normal-tension glaucoma (NTG) and nonarteritic anterior ischemic optic neuropathy (NAION) using optical coherence tomography angiography (OCTA). Methods: Thirty-eight eyes with treatment-naïve NTG, 38 eyes with NAION matched for retinal nerve fiber layer (RNFL) thickness in each superior and inferior quadrant, and 38 healthy eyes matched by age were included. ONH and peripapillary retinal microvasculature was evaluated in en face images obtained using OCTA. Vessel density (VD) was calculated as the percent area occupied by vessels in the measured region in each layer segmented into the prelaminar tissue (PLT), lamina cribrosa (LC), and peripapillary retina (PR). Results: VDs in the PLT and LC were lower in NTG eyes than in both NAION and healthy eyes (P ≤ 0.008), and did not differ between the NAION and healthy eyes. VDs in the PR did not differ between the NTG and NAION eyes. In intersectoral comparisons, VDs in the PLT (P = 0.030) and LC (P = 0.028) were lower in the affected than in the unaffected sector of eyes with NTG, but the differences did not occur in eyes with NAION. VD in the PR was lower in the affected than in the unaffected sector in both NTG and NAION eyes (both P < 0.001). Conclusions: Despite similar degrees of RNFL loss and VD decreases in the PR, VDs in the ONH differed between eyes with NTG and NAION, indicating different mechanisms of vascular impairment and ONH damage in each condition.