Cholinergic degeneration and early cognitive signs in prodromal Lewy body dementia.

Introduction: Isolated REM sleep behavior disorder (iRBD) is a strong prodromal marker of Lewy body diseases (LBDs) - Parkinson's disease (PD) and dementia with Lewy bodies (DLB). Cholinergic loss is linked to cognitive decline in these conditions, but its trajectory remains unclear.

Methods: In a cohort of 92 iRBD participants with baseline MRI, cholinergic basal forebrain (cBF) volume was measured, with longitudinal changes analyzed in 49 with follow-up scans.

Application of elastic net for clinical outcome prediction and classification in progressive supranuclear palsy: A multicenter cohort study.

Background: Previous studies have used machine learning to identify clinically relevant atrophic regions in progressive supranuclear palsy (PSP). This study applied Elastic Net (EN) in PSP to uncover key atrophic patterns, offering a novel approach to understanding its pathology.

Methods: This study included baseline data from 74 patients with PSP enrolled in the Study of Comprehensive ANd multimodal marker-based cohort of PSP (SCAN-PSP, NCT05579301) in Seoul between January 2022 and August 2023.

Hospital utilization and telemedicine preferences in patients with late-stage Parkinson's disease and caregivers.

Background: There remains a significant gap in systematic research on healthcare utilization behaviors and the influencing factors for patients with Parkinson's disease (PD), particularly those in late stages.

Methods: PD patients in late stage (Hoehn and Yahr (HY) stages 4 and 5) and their caregivers from Seoul National University Hospital Movement Disorders Clinic participated. A total of 103 respondents completed a questionnaire covering medical utilization behaviors, perceptions of face-to-face and telemedicine consultations, and additional medical service needs.

Mild behavioral impairment in idiopathic REM sleep behavior disorder and Lewy body disease continuum.

To investigate the clinical impact of mild behavioral impairment (MBI) in a predefined cohort with Lewy body disease (LBD) continuum. Eighty-four patients in the LBD continuum participated in this study, including 35 patients with video-polysomnography-confirmed idiopathic REM sleep behavior disorder (iRBD) and 49 clinically established LBD. Evaluations included the Movement Disorder Society-Unified Parkinson's Disease Rating Scale (MDS-UPDRS), neuropsychological tests, and MBI Checklist (MBI-C).

Prediction of phenoconversion into alpha-synucleinopathy in patients with isolated REM sleep behavior disorder using event-related potentials during visuospatial attention tasks.

Study Objectives: Isolated rapid eye movement sleep behavior disorder (iRBD) is recognized as a prodromal stage of alpha-synucleinopathies. Predicting phenoconversion in iRBD patients remains a key challenge. We aimed to investigate whether event-related potentials (ERPs) recorded during visuospatial attention tasks can serve as predictors of phenoconversion in iRBD patients.

Spatiotemporal Gait Parameters During Turning and Imbalance in Parkinson's Disease: Video-Based Analysis From a Single Camera.

Objective: This study aims to objectively evaluate turning gait parameters in Parkinson's disease (PD) patients using 2D-RGB video-based analysis and explore their relationships with imbalance.

Methods: We prospectively enrolled PD patients for clinical assessment, balance analysis and gait with 180º turning. Spatiotemporal gait parameters during turning were derived using video-based analysis and correlated with modified Hoehn and Yahr (mHY) stages and center of pressure (COP) oscillations.

Development of a cerebellar ataxia diagnosis model using conditional GAN-based synthetic data generation for visuomotor adaptation task.

This study proposes a synthetic data generation model to create a classification framework for cerebellar ataxia patients using trajectory data from the visuomotor adaptation task. The classification objectives include patients with cerebellar ataxia, age-matched normal individuals, and young healthy subjects. Synthetic data for the three classes is generated based on class conditions and random noise by leveraging a combination of conditional adversarial generative neural networks and reconstruction networks.

Progression trajectories from prodromal to overt synucleinopathies: a longitudinal, multicentric brain [F]FDG-PET study.

The phenoconversion trajectory from idiopathic/isolated Rapid eye movement (REM) sleep behavior disorder (iRBD) towards either Parkinson's Disease (PD) or Dementia with Lewy Bodies (DLB) is currently uncertain. We investigated the capability of baseline brain [F]FDG-PET in differentiating between iRBD patients eventually phenoconverting to PD or DLB, by deriving the denovoPDRBD-related pattern (denovoPDRBD-RP) from 32 de novo PD patients; and the denovoDLBRBD-RP from 30 de novo DLB patients, both with evidence of RBD at diagnosis. To explore [F]FDG-PET phenoconversion trajectories prediction power, we applied these two patterns on a group of 115 iRBD patients followed longitudinally.

Clonal hematopoiesis with DNMT3A mutations is associated with multiple system atrophy.

Background: Clonal hematopoiesis of indeterminate potential (CHIP) is associated with cardiovascular diseases and other disorders, possibly via inflammation. Recent research suggests a connection of CHIP with neurodegenerative disorders.

Objective: We aimed to investigate the association between multiple system atrophy (MSA) and CHIP.

Evidence-Based Review on Symptomatic Management of Huntington's Disease.

Huntington's disease (HD) is a neurodegenerative disorder characterized by motor, behavioral, and cognitive impairments and significant impacts on patient quality of life. This evidence-based review, conducted by the Korean Huntington Disease Society task force, systematically examines current pharmacological and nonpharmacological interventions for symptomatic management of HD. Following PRISMA guidelines, databases were searched for studies up to August 2022 that focused on 23 symptoms across four domains: motor, neuropsychological, cognition, and others.

Monoaminergic degeneration, cognition, and autonomic symptom trajectory in early Parkinson's disease.

Introduction: Parkinson's disease (PD) encompasses a range of non-motor symptoms attributed to deficits in various neurotransmitter systems. This study aimed to investigate the associations between cognitive and autonomic symptoms and the degeneration of brainstem monoaminergic nuclei, particularly the serotonergic and noradrenergic nuclei, in a prospective cohort of early PD patients.

Methods: Twenty-eight early PD patients (with an average disease duration of approximately three years) underwent baseline [F]FP-CIT positron emission tomography (PET) scans, Montreal Cognitive Assessment (MoCA), and Composite Autonomic Symptom Scale-31 (COMPASS-31) evaluations, followed by repeat MoCA and COMPASS-31 assessments three years later.

Symmetric and Profound Monoaminergic Degeneration in Parkinson's Disease with Premotor REM Sleep Behavior Disorder.

Background: Rapid eye movement sleep behavior disorder (RBD) may precede or follow motor symptoms in Parkinson's disease (PD). While over 70% of idiopathic RBD cases phenoconvert within a decade, a small subset develops PD after a more extended period or remains nonconverted. These heterogeneous manifestations of RBD in PD prompt subtype investigations.

The prevalence of non-troublesome dyskinesia in Parkinson's disease.

Introduction: Levodopa-induced dyskinesia is a common complication of long-term treatment of Parkinson's disease (PD), but its impact on daily activities is somewhat controversial. This study investigated the prevalence and severity of dyskinesia, particularly non-troublesome dyskinesia, to provide insights into its significance for long-term PD management.

Methods: We reviewed electronic medical records of 2571 PD patients, who had been followed up at Seoul National University Hospital and were seen between January 2016 and June 2017.

Electroencephalographic spectro-spatial covariance patterns related to phenoconversion in isolated rapid eye movement sleep behavior disorder and their longitudinal trajectories in α-synucleinopathies.

Study Objectives: This study aimed to identify electroencephalographic (EEG) spectro-spatial covariance patterns associated with phenoconversion in isolated rapid eye movement sleep behavior disorder (iRBD) patients and explore their longitudinal trajectories within α-synucleinopathies.

Methods: We assessed 47 participants, including 35 patients with iRBD and 12 healthy controls (HC), through baseline eye-closed resting EEGs. Patients with iRBD underwent follow-up EEG assessments and 18 patients with iRBD converted (12 to Parkinson's disease (PD), 6 to dementia with Lewy bodies [DLB]) during follow-up.

Impaired cognitive flexibility and disrupted cognitive cerebellum in degenerative cerebellar ataxias.

There is a clinically unmet need for a neuropsychological tool that reflects the pathophysiology of cognitive dysfunction in cerebellar degeneration. We investigated cognitive flexibility in degenerative cerebellar ataxia patients and aim to identify the pathophysiological correlates of cognitive dysfunction in relation to cerebellar cognitive circuits. We prospectively enrolled degenerative cerebellar ataxia patients with age-matched healthy controls who underwent 3 T 3D and resting-state functional MRI.

Association of high-estimated glomerular filtration rate with the severity of ischemic stroke during non-vitamin K antagonist oral anticoagulants therapy: a nationwide cohort study.

Aim: While the relationship between impaired kidney function and non-vitamin K antagonist oral anticoagulants (NOACs) is well established, there is limited research exploring the association between an elevated estimated glomerular filtration rate (eGFR) and the efficacy of NOACs, especially concerning the outcomes of acute ischemic stroke (AIS). This study aimed to examine the association between higher-than-normal eGFR and the severity of AIS during the use of NOACs using a nationwide multicenter stroke registry in Korea.

Material And Methods: This study utilized data from the Korean Stroke Registry (KSR) database, examining information from 2,379 patients with AIS, who had atrial fibrillation (AF) and a history of utilizing NOACs prior to hospitalization due to incident stroke occurring between 2016 and 2021.

Brain olfactory-related atrophy in isolated rapid eye movement sleep behavior disorder.

Objective: To investigate structural and functional connectivity changes in brain olfactory-related structures in a longitudinal prospective cohort of isolated REM sleep behavior disorder (iRBD) and their clinical correlations, longitudinal evolution, and predictive values for phenoconversion to overt synucleinopathies, especially Lewy body diseases.

Methods: The cohort included polysomnography-confirmed iRBD patients and controls. Participants underwent baseline assessments including olfactory tests, neuropsychological evaluations, the Movement Disorders Society-Unified Parkinson's Disease Rating Scale, 3T brain MRI, and F-FP-CIT PET scans.