Erratum: Validation Study of the Official Korean Version of the Movement Disorder Society-Unified Parkinson's Disease Rating Scale.

This corrects the article on p. 633 in vol. 16, PMID: 33029970.

Validation Study of the Official Korean Version of the Movement Disorder Society-Unified Parkinson's Disease Rating Scale.

Background And Purpose: The Movement Disorder Society-Sponsored Revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS) is widely used for estimating the symptoms of Parkinson's disease. Translation and validation of the MDS-UPDRS is necessary for non-English speaking countries and regions. The aim of this study was to validate the Korean version of the MDS-UPDRS.

Correlation of electrode position and clinical outcomes in globus pallidus stimulation for dystonia.

Background: The correlation between the electrode location and the clinical outcome for internal globus pallidus (GPi) deep brain stimulation (DBS) has not been fully elucidated.

Objective: The aim of this study was to determine the discrepancies between the theoretical target planned by magnetic resonance imaging (MRI) and the actual electrode location in postoperative MRI, as well as to find the correlation between the final electrode locations and the clinical outcome after GPi DBS.

Methods: Thirty-six patients who underwent GPi DBS for dystonia were included in this retrospective study.

Pregnancy and Delivery in a Generalized Dystonia Patient Treated with Internal Globus Pallidal Deep Brain Stimulation: a Case Report.

Internal globus pallidus (GPi) deep brain stimulation (DBS) has been widely accepted as an effective treatment modality of medically refractory dystonia. However, there have been few studies regarding the safety issue of pregnancy and childbirth related with DBS. This report describes a female patient who was pregnant and delivered a baby after GPi DBS surgery.

Clinical Heterogeneity of Atypical Pantothenate Kinase-Associated Neurodegeneration in Koreans.

Objective: Neurodegeneration with brain iron accumulation (NBIA) represents a group of inherited movement disorders characterized by iron accumulation in the basal ganglia. Recent advances have included the identification of new causative genes and highlighted the wide phenotypic variation between and within the specific NBIA subtypes. This study aimed to investigate the current status of NBIA in Korea.

Long-Term Clinical Outcome of Internal Globus Pallidus Deep Brain Stimulation for Dystonia.

Background: GPi (Internal globus pallidus) DBS (deep brain stimulation) is recognized as a safe, reliable, reversible and adjustable treatment in patients with medically refractory dystonia.

Objectives: This report describes the long-term clinical outcome of 36 patients implanted with GPi DBS at the Neurosurgery Department of Seoul National University Hospital.

Methods: Nine patients with a known genetic cause, 12 patients with acquired dystonia, and 15 patients with isolated dystonia without a known genetic cause were included.

The Pathogenic Role of Low Range Repeats in SCA17.

Introduction: SCA17 is an autosomal dominant cerebellar ataxia with expansion of the CAG/CAA trinucleotide repeats in the TATA-binding protein (TBP) gene. SCA17 can have various clinical presentations including parkinsonism, ataxia, chorea and dystonia. SCA17 is diagnosed by detecting the expanded CAG repeats in the TBP gene; however, in the literature, pathologic repeat numbers as low as 41 overlap with normal repeat numbers.

Rotigotine transdermal system as add-on to oral dopamine agonist in advanced Parkinson's disease: an open-label study.

Background: Achieving optimal symptom control with minimal side effects is a major goal in clinical practice. Dual-agent dopamine receptor agonist (DA) therapy in Parkinson's disease (PD) may represent a promising approach to treatment, as the combination of different pharmacokinetic/pharmacological profiles may result in a lesser need for high dosages and, accordingly, may be well tolerated. The objective of the current study was to investigate safety and efficacy of rotigotine transdermal system as add-on to oral DA in patients with advanced PD inadequately controlled with levodopa and low-dose oral DA.

Switch from oral pramipexole or ropinirole to rotigotine transdermal system in advanced Parkinson's disease: an open-label study.

Objective: Investigate safety, feasibility and efficacy of switching therapy in patients with advanced-stage Parkinson's disease (PD) inadequately controlled with pramipexole (≤ 3.5 mg/day) or ropinirole (≤ 14 mg/day) to rotigotine transdermal system (≤ 14 mg/24 h; dose adjustments ≤ 16 mg/24 h permitted).

Methods: PD0009 (ClinicalTrials.

Whole-brain diffusion-tensor changes in parkinsonian patients with impulse control disorders.

Background And Purpose: The aim of this study was to determine the changes in diffusion-tensor images associated with medication-related impulse control disorder (ICD) in Parkinson's disease (PD) patients undergoing chronic dopamine-replacement therapy.

Methods: Nineteen PD patients, comprising 10 with ICD (PD-ICD) and 9 without ICD (PD-nonICD), and 18 age-matched healthy controls (HCs) with no cognitive or other psychiatric disorders were analyzed. All subjects underwent 3-T magnetic resonance diffusion-tensor imaging.

Vestibular Performance During High-Acceleration Stimuli Correlates with Clinical Decline in SCA6.

In spinocerebellar ataxia type 6 (SCA6), the vestibular dysfunction and its correlation with other clinical parameters require further exploration. We determined vestibular responses over a broad range of stimulus acceleration in 11 patients with SCA6 (six men, age range=33-72 years, mean age±SD=59±12 years) using bithermal caloric irrigations, rotary chair, and head impulse tests. Correlations were also pursued among disability scores, as measured using the International Cooperative Ataxia Rating Scale, disease duration, age at onset, cytosine-adenine-guanine (CAG) repeat length, and the gain of the vestibulo-ocular reflex (VOR).

Comparison of sleep and other non-motor symptoms between SWEDDs patients and de novo Parkinson's disease patients.

Background: SWEDDs (Scans Without Evidence of Dopaminergic Deficits) was defined from a series of pharmaceutical trials on Parkinson's disease (PD). Non-motor features including sleep-related problems are common even in early-stage PD patients; however, little is known about the burden of the non-motor symptoms in SWEDDs patients.

Methods: The Non-motor Symptoms Assessment Scale (NMSS), revised version of the Parkinson's Disease Sleep Scale (PDSS-2), Epworth Sleepiness Scale (ESS), and EuroQol 5-Dimension (EQ-5D) were applied to evaluate 17 SWEDDs patients and 28 de novo PD patients.

Maladaptive reward-learning and impulse control disorders in patients with Parkinson's disease: a clinical overview and pathophysiology update.

Impulse control disorders (ICD) in Parkinson's disease (PD) are a disabling non-motor symptom with frequencies of 13-35% among patients receiving dopamine replacement therapy. ICD in PD is strongly associated with dopaminergic drug use, especially non-ergot dopamine agonists (DA). However, individual susceptibility and disease-related neural changes are also important contributors to the development of ICD.

Nanomolar concentration of alpha-synuclein enhances dopaminergic neuronal survival via Akt pathway.

Although alpha-synuclein is generally thought to have a pathological role in Parkinson's disease, accumulative evidence exists that alpha-synuclein has a neuroprotective effect. The aim of this study was to evaluate the effect of extracellular alpha-synuclein on dopaminergic cell survival. We assessed cell viability using the 3-(4,5-dimethyl-thiazol-2-yl)-2,5-diphenyltertazolium bromide (MTT) assay both in undifferentiated SH-SY5Y (SHSY) cells and neuronally-differentiated SH-SY5Y (ndSHSY) cells after 24 hour treatment with monomeric alpha-synuclein at various concentrations (0 [control], 50, 100 nmol/L, 1 μmol/L).

Clinical spectrum of dopa-responsive dystonia and related disorders.

Dopa-responsive dystonia (DRD) has a classic presentation of childhood or adolescent-onset dystonia, mild parkinsonism, marked diurnal fluctuations, improvement with sleep or rest, and a dramatic and sustained response to low doses of L-dopa without motor fluctuations or dyskinesias. However, there have been many papers on patients with a wide range of features, which report them as DRD mainly because they had dystonic syndromes with L-dopa responsiveness. Many mutations in the dopaminergic system have been found as molecular genetic defects.

Identifying the clusters within nonmotor manifestations in early Parkinson's disease by using unsupervised cluster analysis.

Background: Classical and data-driven classifications of Parkinson's disease (PD) are based primarily on motor symptoms, with little attention being paid to the clustering of nonmotor manifestations.

Methods: Clinical data on demographic, motor and nonmotor features, including the Korean version of the sniffin' stick (KVSS) test results, and responses to the screening questionnaire of the nonmotor features were collected from 56 PD patients with disease onset within 3 years. Nonmotor subgroups were classified using unsupervised hierarchical cluster analysis (HCA).

Alpha-synuclein repeat variants and survival in Parkinson's disease.

Objectives: To determine whether α-synuclein dinucleotide repeat (REP1) genotypes are associated with survival in Parkinson's disease (PD).

Methods: Investigators from the Genetic Epidemiology of Parkinson's Disease Consortium provided REP1 genotypes and baseline and follow-up clinical data for cases. The primary outcome was time to death.

Optical coherence tomography in Parkinson's disease: is the retina a biomarker?

Visual symptoms are a common feature in patients with Parkinson's disease (PD), and retinal dopamine loss and dysfunctions in foveal vision have been described in PD patients. Because visual hallucination (VH) is a specific feature of PD which is differentiated from other parkinsonian disorders, defective visual information processing from both the central and peripheral pathways is suggested to be the pathophysiological mechanisms of VH in PD. Decreased visual acuity as well as impaired contrast sensitivity and color vision is known to be related to the appearance of VH in PD.

Preserved high-centrality hubs but efficient network reorganization during eyes-open state compared with eyes-closed resting state: an MEG study.

A question to be addressed in the present study is how different the eyes-closed (EC) and eyes-open (EO) resting states are across frequency bands in terms of efficiency and centrality of the brain functional network. We investigated both the global and nodal efficiency and betweenness centrality in the EC and EO resting states from 39 volunteers. Mutual information was used to obtain the functional connectivity for each of the four frequency bands (theta, alpha, beta, and gamma).

The clinical impact of precise electrode positioning in STN DBS on three-year outcomes.

Few studies have analyzed the clinical impact of subthalamic nucleus (STN) deep brain stimulation (DBS) as a function of the positioning of the inserted electrode. We investigated retrospectively the three-year outcomes in Parkinson's disease (PD) patients following bilateral STN DBS in terms of the electrode positions. Forty-one advanced PD patients were followed up for over three years following bilateral STN DBS.