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. @*Methods@#Altogether, 362 patients in 19 centers were recruited for this study. We translated the MDS-UPDRS to Korean using the translation-back translation method and cognitive pretesting. We performed both confirmatory and exploratory factor analyses to validate the scale.We calculated the comparative fit index (CFI) for confirmatory factor analysis, and used unweighted least squares for exploratory factor analysis. @*Results@#The CFI was higher than 0.90 for all parts of the scale. Exploratory factor analysis also showed that the Korean MDS-UPDRS has the same number of factors in each part as the English version. @*Conclusions@#The Korean MDS-UPDRS has the same overall structure as the English MDSUPDRS. Our translated scale can be designated as the official Korean MDS-UPDRS.

Validation of the Korean Version of the Questionnaire for Impulsive-Compulsive Disorders in Parkinson's Disease Rating Scale

Background@#and PurposeImpulse-control disorder is an important nonmotor symptom of Parkinson's disease (PD) that can lead to financial and social problems, and be related to a poor quality of life. A nationwide multicenter prospective study was performed with the aim of validating the Korean Version of the Questionnaire for Impulsive-Compulsive Disorders in Parkinson's Disease Rating Scale (K-QUIP-RS). @*Methods@#The K-QUIP-RS was constructed using forward and backward translation, and pretesting of the prefinal version. PD patients on stable medical condition were recruited from 27 movement-disorder clinics. Participants were assessed using the K-QUIP-RS and evaluated for parkinsonian motor and nonmotor statuses and for PD-related quality of life using a predefined evaluation battery. The test–retest reliability of the K-QUIP-RS was assessed over an interval of 10–14 days, and correlations between the KQUIP-RS and other clinical scales were analyzed. @*Results@#This study enrolled 136 patients. The internal consistency of the K-QUIP-RS was indicated by a Cronbach's α coefficient of 0.846, as was the test–retest reliability by a Guttman split-half coefficient of 0.808. The total K-QUIP-RS score was positively correlated with the scores for depression and motivation items on the Unified PD Rating Scale (UPDRS), Montgomery-Asberg Depression Scale, and Rapid-Eye-Movement Sleep-Behavior-Disorders Questionnaire. The total K-QUIP-RS score was also correlated with the scores on part II of the UPDRS and the PD Quality of Life-39 questionnaire, and the dopaminergic medication dose. @*Conclusions@#The K-QUIP-RS appears to be a reliable assessment tool for impulse-control and related behavioral disturbances in the Korean PD population.

Subtypes of Sleep Disturbance in Parkinson's Disease Based on the Cross-Culturally Validated Korean Version of Parkinson's Disease Sleep Scale-2

Magnetic Resonance Image Findings of Cerebral Amyloid Angiopathy Related Inflammation

No abstract available.

A Man with Adult-onset Progressive Spastic Paraparesis: Genetically Confirmed as Novel Mutation of ABCD1 Gene

Spastic paraparesis is caused by various etiologies such as autoimmune, infection, genetic and metabolic disorder. Adrenomyeloneuropathy (AMN) is very rare but one of important causes in spastic paraparesis. We experienced a patient presenting with adult-onset progressive spastic paraparesis, who was diagnosed as AMN with hemizygous c.431C>T (p.A144V), a novel mutation in exon1. The level of very long chain fatty acid should be included in diagnostic work-up for patients presenting with adult-onset progressive spastic paraparesis.

Validation of the Korean Version of the Scales for Outcomes in Parkinson's Disease-Sleep

BACKGROUND: Sleep problems commonly occur in patients with Parkinson's disease (PD), and are associated with a lower quality of life. The aim of the current study was to translate the English version of the Scales for Outcomes in Parkinson's Disease-Sleep (SCOPA-S) into the Korean version of SCOPA-S (K-SCOPA-S), and to evaluate its reliability and validity for use by Korean-speaking patients with PD. METHODS: In total, 136 patients with PD from 27 movement disorder centres of university-affiliated hospitals in Korea were enrolled in this study. They were assessed using SCOPA, Hoehn and Yahr Scale (HYS), Unified Parkinson's Disease Rating Scale (UPDRS), Parkinson's Disease Sleep Scale 2nd version (PDSS-2), Non-motor Symptoms Scale (NMSS), Montgomery Asberg Depression Scale (MADS), 39-item Parkinson's Disease Questionnaire (PDQ39), Neurogenic Orthostatic Hypotension Questionnaire (NOHQ), and Rapid Eye Movement Sleep Behaviour Disorder Questionnaire (RBDQ). The test-retest reliability was assessed over a time interval of 10–14 days. RESULTS: The internal consistency (Cronbach's α-coefficients) of K-SCOPA-S was 0.88 for nighttime sleep (NS) and 0.75 for daytime sleepiness (DS). Test-retest reliability was 0.88 and 0.85 for the NS and DS, respectively. There was a moderate correlation between the NS sub-score and PDSS-2 total score. The NS and DS sub-scores of K-SCOPA-S were correlated with motor scale such as HYS, and non-motor scales such as UPDRS I, UPDRS II, MADS, NMSS, PDQ39, and NOHQ while the DS sub-score was with RBDQ. CONCLUSION: The K-SCOPA-S exhibited good reliability and validity for the assessment of sleep problems in the Korean patients with PD.

Erratum to: The KMDS-NATION Study: Korean Movement Disorders Society Multicenter Assessment of Non-Motor Symptoms and Quality of Life in Parkinson's Disease NATION Study Group

The original version of this article contained wrong informations of some authors which should be changed.

Spontaneous Cerebral Air Embolism

No abstract available.

Dementia-Free Survival and Risk Factors for Dementia in a Hospital-Based Korean Parkinson's Disease Cohort

BACKGROUND AND PURPOSE: Few studies of dementia in Parkinson's disease (PD) have had long-term follow-ups. Moreover, information on the duration from the onset to the development of dementia in patients with PD is lacking. The aim of this study was to determine the median dementia-free survival time from the onset of PD to the development of dementia. METHODS: In total, 1,193 Korean patients with PD were recruited and assessed at regular intervals of 3–6 months. We interviewed the patients and other informants to identify impairments in the activities of daily living. The Hoehn and Yahr stage and scores on the Unified Parkinson's Disease Rating Scale and Mini Mental State Examination were evaluated annually. We used Kaplan-Meier survival analysis to estimate the cumulative proportion of dementia-free patients over time. Risk factors predicting dementia were also evaluated using Cox proportional-hazards regression models. RESULTS: The median dementia-free survival time in the Korean PD population was 19.9 years. Among the 119 patients who subsequently developed dementia, the mean duration from the onset of PD to the development of dementia was 10.6 years. A multivariate analysis identified age at onset and education period as the significant predictors of dementia. CONCLUSIONS: This is the first report on dementia-free survival in patients with PD based on longitudinal data analysis from the disease onset. The median dementia-free survival time in Korean PD patients was found to be longer than expected.

Validation of the Korean Version of the Scale for Outcomes in Parkinson's Disease-Autonomic

OBJECTIVE: Autonomic symptoms are commonly observed in patients with Parkinson's disease (PD) and often limit the activities of daily living. The Scale for Outcomes in Parkinson's disease-Autonomic (SCOPA-AUT) was developed to evaluate and quantify autonomic symptoms in PD. The goal of this study was to translate the original SCOPA-AUT, which was written in English, into Korean and to evaluate its reliability and validity for Korean PD patients. METHODS: For the translation, the following processes were performed: forward translation, backward translation, expert review, pretest of the pre-final version and development of the final Korean version of SCOPA-AUT (K-SCOPA-AUT). In total, 127 patients with PD from 31 movement disorder clinics of university-affiliated hospitals in Korea were enrolled in this study. All patients were assessed using the K-SCOPA-AUT and other motor, non-motor, and quality of life scores. Test-retest reliability for the K-SCOPA-AUT was assessed over a time interval of 10−14 days. RESULTS: The internal consistency and reliability of the K-SCOPA-AUT was 0.727 as measured by the mean Cronbach's α-coefficient. The test-retest correlation reliability was 0.859 by the Guttman split-half coefficient. The total K-SCOPA-AUT score showed a positive correlation with other non-motor symptoms [the Korean version of non-motor symptom scale (K-NMSS)], activities of daily living (Unified Parkinson's Disease Rating Scale part II) and quality of life [the Korean version of Parkinson's Disease Quality of Life 39 (K-PDQ39)]. CONCLUSION: The K-SCOPA-AUT had good reliability and validity for the assessment of autonomic dysfunction in Korean PD patients. Autonomic symptom severities were associated with many other motor and non-motor impairments and influenced quality of life.

Two Siblings with Adolescent/Adult Onset Niemann-Pick Disease Type C in Korea

Niemann-Pick disease, type C (NP-C), is caused by NPC1 or NPC2 gene mutations. Progressive neurological, psychiatric, and visceral symptoms are characteristic. Here, we present cases of a brother (Case 1) and sister (Case 2) in their mid-20s with gait disturbance and psychosis. For the Case 1, neurological examination revealed dystonia, ataxia, vertical supranuclear-gaze palsy (VSGP), and global cognitive impairment. Case 2 showed milder, but similar symptoms, with cortical atrophy. Abdominal computed tomography showed hepatosplenomegaly in both cases. NPC1 gene sequencing revealed compound heterozygote for exon 9 (c.1552C>T [R518W]) and exon 18 (c.2780C>T [A927V]). Filipin-staining tests were also positive. When a young patient with ataxia or dystonia shows VSGP, NP-C should be considered.

The KMDS-NATION Study: Korean Movement Disorders Society Multicenter Assessment of Non-Motor Symptoms and Quality of Life in Parkinson's Disease NATION Study Group

BACKGROUND AND PURPOSE: Nonmotor symptoms (NMS) in Parkinson's disease (PD) have multisystem origins with heterogeneous manifestations that develop throughout the course of PD. NMS are increasingly recognized as having a significant impact on the health-related quality of life (HrQoL). We aimed to determine the NMS presentation according to PD status, and the associations of NMS with other clinical variables and the HrQoL of Korean PD patients. METHODS: We surveyed patients in 37 movement-disorders clinics throughout Korea. In total, 323 PD patients were recruited for assessment of disease severity and duration, NMS, HrQoL, and other clinical variables including demographics, cognition, sleep scale, fatigability, and symptoms. RESULTS: In total, 98.1% of enrolled PD subjects suffered from various kinds of NMS. The prevalence of NMS and scores in each NMS domain were significantly higher in the PD group, and the NMS worsened as the disease progressed. Among clinical variables, disease duration and depressive mood showed significant correlations with all NMS domains (p<0.001). NMS status impacted HrQoL in PD (rS=0.329, p<0.01), and the association patterns differed with the disease stage. CONCLUSIONS: The results of our survey suggest that NMS in PD are not simply isolated symptoms of degenerative disease, but rather exert significant influences throughout the disease course. A novel clinical approach focused on NMS to develop tailored management strategies is warranted to improve the HrQoL in PD patients.

Restless Legs Syndrome in Children and Adolescents with Type 1 Diabetes / 수면정신생리

OBJECTIVES: Restless legs syndrome (RLS) is a common sleep disorder in adults with diabetes. This study investigated the frequency of RLS and clinical correlations in children and adolescents with type 1 diabetes. METHODS: This study included 55 consecutive patients (21 males, age 12.6 +/- 3.4 years) with type I diabetes that were regularly treated at the Department of Pediatric Endocrinology. RLS was diagnosed by intensive interviews which also included the Epworth Sleepiness Scale (ESS) and International RLS Rating Scale (IRLSRS). Patients also received neurological examinations and laboratory tests for diabetes, iron metabolism and renal function. RESULTS: Thirteen patients (23.6%, 6 males) were compatible for the diagnostic criteria of RLS. None of the RLS patients showed abnormal findings in neurological evaluations and 7 patients had familial history of RLS. Demographic and laboratory findings were not different between the patients with or without RLS. The RLS group showed significantly increased ESS and IRLSRS scores. CONCLUSION: RLS was prevalent in children and adolescents with type I diabetes. The association between RLS and diabetes-related laboratory findings requires further investigation.

Effect of Carotid Artery Stenting on Cognitive Function in Patients with Carotid Artery Stenosis: A Prospective, 3-Month-Follow-Up Study

BACKGROUND AND PURPOSE: Carotid artery stenting (CAS) is emerging as an alternative to carotid endarterectomy for the treatment of carotid artery stenosis (CS), but the effect of CAS on the cognitive function of patients with severe CS has not been fully investigated. The aim of this study was to use comprehensive neuropsychological tests to determine the effect of CAS on cognitive function from baseline to 3 months postprocedure in patients with severe CS. METHODS: Thirty-one patients due to undergo CAS due to high-grade CS (> or =70%) and 11 control subjects who were diagnosed with CS, but who did not undergo CAS, and who visited the clinic or emergency room between February 2009 and February 2012 were recruited consecutively at baseline (i.e., pre-CAS). Follow-up neuropsychological evaluations after 3 months were completed by 23 of the 31 patients who underwent CAS, and by 10 of the 11 control subjects. The primary cognitive outcome was assessed using a neuropsychological test containing subcategories designed to test general cognitive function, attention, visuospatial function, language and related functions, memory, and frontal lobe/executive function. RESULTS: Of the 23 patients undergoing CAS who completed the 3-month follow-up tests, 12 had asymptomatic CS. During the 3-month follow-up period, the patients who underwent CAS and those with asymptomatic CS achieved similar results to the control group on all cognitive tests. However, symptomatic CS patients (n=11) who underwent CAS exhibited improvements in visuospatial function (p=0.046) and total Seoul Neuropsychological Screening Battery-Dementia Version scores (p=0.010) in comparison with both the asymptomatic CS patients and the control group. CONCLUSIONS: The findings of this study suggest that CAS has a positive effect on cognitive function in patients with symptomatic CS over a 3-month follow-up period. A long-term, multicenter, prospective case-control study would be helpful to predict quality of life and prognoses for patients undergoing CAS.

Sleep Disturbances in Patients with Parkinson's Disease according to Disease Severity / 대한임상신경생리학회지

BACKGROUND: Sleep-related disturbances and sleep disorders are common in Parkinson's disease (PD) and have a great impact on daily life of PD patients. This study was done to find the sleep characteristics and sleep disturbing factors in PD patients according to disease severity through clinical interview and polysomnographic (PSG) study. METHODS: Fifty patients with PD (22 males, age 60.6 +/- 6.4, Hoehn and Yahr (HY) stage 2.7 +/- 1.0) were recruited and thoroughly interviewed about their sleep. PSG was performed on the patients taking routine antiparkinsonian medications. Patients were grouped into mild and moderate/severe group according to HY stage, and the results were compared between each group. RESULTS: Ninety-four percent of total patients had one or more sleep-related disturbances based on the interview or PSG. On interview, the moderate/severe group complained more insomnia and REM sleep behavior disorder (RBD) than mild group. In PSG findings, the moderate/severe group showed lower sleep efficiency, longer sleep latency, REM sleep latency, waking time after sleep onset, and higher prevalence of RBD. CONCLUSIONS: In this study, most patients with PD had sleep disturbances. Clinical interview and PSG findings revealed deterioration of sleep quality along the disease severity. Our results suggest that sleep disturbances in PD patients are prevalent and warrant clinical attention, especially to the patients with advanced disease.

Development of the Korean Version of the Sleep Apnea Quality of Life Index

OBJECTIVES: Obstructive sleep apnea (OSA) is a disorder characterized by repetitive partial or complete occlusion of the upper airway during sleep that affects quality of life. The aim of this study was to develop the Korean version of the sleep apnea quality of life index (K-SAQLI) and apply it in Korean patients with OSA. METHODS: Ninety-three patients with OSA completed the K-SAQLI. Its construct validity and responsiveness were tested by comparing the baseline and change scores obtained in each domain (i.e., daily functioning, social interactions, emotional functioning, and symptoms) using the medical outcome survey-short form 36 (SF-36). RESULTS: The Cronbach alpha coefficients of internal reliability exceeded 0.60 in all the domains (daily functioning, 0.89; social interactions, 0.88; emotional functioning, 0.92; symptoms, 0.67; and total, 0.94). The K-SAQLI had a high test-retest correlation coefficient of 0.73 in the 20 randomized selected patients. The construct validity was confirmed by significant correlations with SF-36 subscale scores. CONCLUSION: The results of this study demonstrate that the K-SAQLI may be applicable for clinical purposes.

The Efficacy of Exercise Programs for Parkinson's Disease: Tai Chi versus Combined Exercise

BACKGROUND AND PURPOSE: Exercise is recommended for every patient with Parkinson's disease (PD). The effectiveness of two different forms of exercise for PD, Tai Chi and combined stretching-strengthening exercise, was compared. METHODS: Patients with mild-to-moderate PD were recruited to join either the combined stretching-strengthening exercise group (n=7), the Tai Chi group (n=9), or the control (nonintervention) group (n=7). Exercise was performed three times a week over a period of 8 weeks. The Tai Chi exercise was led by certified instructors based on a Tai-Chi-for-arthritis program. The combined stretching-strengthening exercise comprised folk dancing, stepping, and elastic-band exercises. The subjects' functional fitness, parkinsonian symptoms, quality of life (QoL), and depression were evaluated. RESULTS: Both exercise groups yielded better results in their overall functional fitness after the intervention. However, no improvement with exercise was found for parkinsonian symptoms, as evaluated using the Unified Parkinson's Disease Rating Scale. With respect to the domains of QoL, the combined stretching-strengthening exercise group fared better in the social domain of QoL, and the Tai Chi group fared better in the emotional domain, while QoL and depression worsened in the control group. The postintervention QoL was improved relative to the control condition only for the Tai Chi group. Although the exercise interventions did not have any effect on depression, the control group was associated with a significant deterioration. CONCLUSIONS: Exercise improved the functional fitness and QoL of PD patients, with Tai Chi yielding better results in QoL and favorable results in functional fitness. These findings suggest that Tai Chi could be a good exercise strategy for patients with PD.

Elevated Levels of alpha-Synuclein Oligomer in the Cerebrospinal Fluid of Drug-Naive Patients with Parkinson's Disease

BACKGROUND AND PURPOSE: The detection of alpha-synuclein in the body fluids of patients with synucleinopathy has yielded promising but inconclusive results, in part because of conformational changes of alpha-synuclein in response to environmental conditions. The aim of this study was to determine the feasibility of using alpha-synuclein as a biological marker for Parkinson's disease (PD). METHODS: Twenty-three drug-naive patients with PD (age 62.4+/-12.7 years, mean+/-SD; 11 males) and 29 age- and sex-matched neurologic control subjects (age 60.1+/-16.2 years; 16 males) were recruited. The levels of oligomeric and total alpha-synuclein in the cerebrospinal fluid (CSF) and plasma were measured using two simultaneous enzyme-linked immunosorbent assays. RESULTS: The level of alpha-synuclein oligomer in the CSF of PD patients was significantly higher in PD patients than in neurological controls, but other findings (plasma alpha-synuclein oligomer and total alpha-synuclein in CSF and plasma) did not differ significantly between the two groups. When the control subjects were divided into a symptomatic control group (11 patients who complained of parkinsonian symptoms and were diagnosed with hydrocephalus and drug-induced or vascular parkinsonism) and a neurologic control group (10 normal subjects and 8 patients with diabetic ophthalmoplegia), the level of alpha-synuclein oligomer in the CSF was still significantly higher in PD patients than in both of the control subgroups. CONCLUSIONS: These findings provide further evidence for a pathogenic role of the alpha-synuclein oligomer and suggest that CSF levels of alpha-synuclein oligomer can be a reliable marker for PD.

Dementia with Lewy Bodies versus Alzheimer's Disease and Parkinson's Disease Dementia: A Comparison of Cognitive Profiles

BACKGROUND AND PURPOSE: It is particularly difficult to differentiate dementia with Lewy bodies (DLB) from the related dementias of Alzheimer's disease (AD) and Parkinson's disease dementia (PDD). Few studies have been designed to comparatively analyze detailed neuropsychological assessments of DLB patients and patients with AD and PDD. METHODS: Three groups of patients participated in this study: 10 with DLB, 76 with AD, and 17 with PDD, who had been diagnosed as probable DLB, AD, and PDD, respectively, according to the clinical criteria of the consortium on DLB, National Institute of Neurological and Communicative Diseases and Stroke/Alzheimer's Disease and Related Disorder Association, and the clinical diagnostic criteria for PDD. All patients were evaluated by careful neurological examination with detailed neuropsychological testing. RESULTS: Significant differences among the three groups were found for attention, memory, and executive function, which included tasks of backward digit span, three-word recall, verbal delayed recall, and the Stroop test. Post hoc analysis revealed that the deficiencies of attention on the digit span task were greater in the DLB group than in the AD and PDD groups. The scores for episodic verbal memory tasks were significantly lower in the DLB and AD groups than in the PDD group. The performance in frontal executive function, as indicated by the Stroop test, was significantly worse in the DLB and PDD groups than in the AD group. CONCLUSIONS: The results of the present study show that the pattern of cognitive dysfunction, in terms of attention, episodic memory, and executive functions, differ between patients with DLB and patients with AD and PDD.

Selective Susceptibility of Human Dopaminergic Neural Stem Cells to Dopamine-Induced Apoptosis

Dysfunctions of ubiquitin-proteasome system and toxicity of dopamine have been known as the key mechanisms in the pathogenesis of Parkinson's disease (PD) and proteasome inhibitors are widely used in experimental models of PD to reproduce cell death of dopaminergic neurons. In the present study, immortalized human neural stem cells (HB1.F3, F3) and those transfected with human aromatic acid decarboxylase gene (F3.AADC), were used to investigate the mechanism of selective dopaminergic neuronal cell death mediated by dopamine or proteasome inhibitors. Flow cytometric analysis revealed that F3.AADC was more susceptible to dopamine than parental F3 cell which does not carry dopaminergic phenotype. The dopamine-induced apoptosis was mediated by activation of caspases 3 and 9 and cleavage of PARP. Proteasome inhibitors also induced apoptosis in dose-dependent manner but there was no difference between cell types. Prolonged exposure to subtoxic dose of proteasome inhibitors further enhanced dopamine-induced apoptosis in the F3.AADC, and increased presence of alpha-synuclein and ubiquitin-positive inclusions was noted in the cytoplasm of apoptotic cells by immunocytochemistry. These findings indicate that dopaminergic cells are selectively susceptible to dopamine toxicity and prolonged suppression of proteasome system further enhances selective sensitivity to dopamine toxicity. Chronic subtoxic proteasomal dysfunction of dopaminergic cells might contribute to selective cell death of dopaminergic neurons during the pathogenesis of Parkinson's disease.