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Objective@#The International Cooperative Ataxia Rating Scale (ICARS) is a semiquantitative clinical scale for ataxia that is widely used in numerous countries. The purpose of this study was to investigate the validity and reliability of the Korean-translated version of the ICARS. @*Methods@#Eighty-eight patients who presented with cerebellar ataxia were enrolled. We investigated the construct validity using exploratory factor analysis (EFA) and confirmatory factor analysis (CFA). We also investigated the internal consistency using Cronbach’s α and intrarater and interrater reliability using intraclass correlation coefficients. @*Results@#The Korean-translated ICARS showed satisfactory construct validity using EFA and CFA. It also revealed good interrater and intrarater reliability and showed acceptable internal consistency. However, subscale 4 for assessing oculomotor disorder showed moderate internal consistency. @*Conclusion@#This is the first report to investigate the validity and reliability of the Korean-translated ICARS. Our results showed excellent construct and convergent validity. The reliability is also acceptable.
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Background@#and PurposeThis study aimed to determine the updated 10-year prevalence of Huntington’s disease (HD) in South Korea and the medical and economic burdens across the duration of the disease. @*Methods@#Data from the National Health Insurance database during 2010–2019 were analyzed. We identified HD cases using predefined criteria. Information on age at diagnosis, sex, and common nonneurological comorbidities were collected. We analyzed individual patterns of the use of medical services and yearly medical expenditure. Incidence rates, 10-year prevalence rates, and longitudinal medical expenditure changes were assessed. @*Results@#New patients with HD (average=152.10) were detected every year, with an annual incidence of 0.29 per 100,000. The estimated 10-year prevalence of HD was 2.2 per 100,000. The most common ages at the time of diagnosis were 50–59 years (23.3%). In 2019, 56.4% of patients with HD were followed-up at referral or general hospitals, and 32.2% were managed at long-term-care hospitals. The annual medical cost for an individual was KRW 6,569,341±895,097 (mean±SD) (mean≈USD 5,653). Medical expenditure was the highest in those aged 60–79 years, and lowest in those younger than 30 years. However, in all age groups, the annual medical expenditure was highest during the 9 years following a diagnosis. @*Conclusions@#This study found that the actual prevalence of HD in South Korea was higher than previously thought and that patients are in a situation with high medical expenditure that persists over time.
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Nongenetic movement disorders are common throughout the world. The movement disorders encountered may vary depending on the prevalence of certain disorders across various geographical regions. In this paper, we review historical and more common nongenetic movement disorders in Asia. The underlying causes of these movement disorders are diverse and include, among others, nutritional deficiencies, toxic and metabolic causes, and cultural Latah syndrome, contributed by geographical, economic, and cultural differences across Asia. The industrial revolution in Japan and Korea has led to diseases related to environmental toxin poisoning, such as Minamata disease and β-fluoroethyl acetate-associated cerebellar degeneration, respectively, while religious dietary restriction in the Indian subcontinent has led to infantile tremor syndrome related to vitamin B12 deficiency. In this review, we identify the salient features and key contributing factors in the development of these disorders.
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Clinical case studies and reporting are important to the discovery of new disorders and the advancement of medical sciences. Both clinicians and basic scientists play equally important roles leading to treatment discoveries for both cures and symptoms. In the field of movement disorders, exceptional observation of patients from clinicians is imperative, not just for phenomenology but also for the variable occurrences of these disorders, along with other signs and symptoms, throughout the day and the disease course. The Movement Disorders in Asia Task Force (TF) was formed to help enhance and promote collaboration and research on movement disorders within the region. As a start, the TF has reviewed the original studies of the movement disorders that were preliminarily described in the region. These include nine disorders that were first described in Asia: Segawa disease, PARK-Parkin, X-linked dystonia-parkinsonism, dentatorubral-pallidoluysian atrophy, Woodhouse-Sakati syndrome, benign adult familial myoclonic epilepsy, Kufor-Rakeb disease, tremulous dystonia associated with mutation of the calmodulin-binding transcription activator 2 gene, and paroxysmal kinesigenic dyskinesia. We hope that the information provided will honor the original researchers and help us learn and understand how earlier neurologists and basic scientists together discovered new disorders and made advances in the field, which impact us all to this day.
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Uncontrolled blood pressure (BP) in patients with chronic kidney disease (CKD) can lead to serious adverse outcomes. To prevent the occurrence of cardiovascular events (CVEs), and end-stage kidney disease, achieving an optimal BP level is important. Recently, there has been a paradigm shift in the management of BP largely as a result of the Systolic Blood Pressure Intervention Trial (SPRINT), which showed a reduction in CVEs by lowering systolic BP to 120 mmHg. A lower systolic blood pressure (SBP) target has been accepted by the Kidney Disease: Improving Global Outcomes (KDIGO) 2021 guidelines. However, whether intensive control of SBP targeting < 120 mmHg is also effective in patients with CKD is controversial. Notably, this lower target SBP is associated with a higher risk of adverse kidney outcomes. Unfortunately, there have been no randomized controlled trials on this issue involving only patients with CKD, particularly those with advanced CKD. In this review, we discuss the optimal control of BP in patients with CKD in terms of reduction in death and CVEs as well as attenuation of CKD progression based on the evidence-based literature.
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Objective@#The distinction between idiopathic normal pressure hydrocephalus (iNPH) and hydrocephalus ex vacuo caused by encephalic volume loss remains to be established. This study aims to investigate radiological parameters as clinically useful tools to discriminate iNPH from hydrocephalus ex vacuo caused by Alzheimer’s disease (AD). @*Methods@#A total of 54 patients with ventriculomegaly (iNPH, 25; hydrocephalus ex vacuo, 29) were recruited in this study. Consequently, nine radiological parameters were compared between iNPH and hydrocephalus ex vacuo using magnetic resonance imaging (MRI). @*Results@#A small callosal angle (CA), the Sylvian fissure dilatation, and absence of narrowing of superior parietal sulci discriminated the iNPH group from the hydrocephalus ex vacuo group (p<0.05). The final binary logistic regression model included narrowing of superior parietal sulci, degrees of the CA, and height of the Sylvian fissure after controlling for age and global Clinical Dementia Rating (CDR). The composite score made from these three indicators (narrowing of superior parietal sulci, degrees of the CA, and height of the Sylvian fissure) was statistically different between iNPH and hydrocephalus ex vacuo. @*Conclusion@#The narrowing of the CA, dilatation of the Sylvain fissure, and narrowing of superior parietal sulci may be used as radiological key indices and noninvasive tools for the differential diagnosis of iNPH from hydrocephalus ex vacuo.
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Objective@#To assess nocturnal hypokinesia using the Korean version of the Nocturnal Hypokinesia Questionnaire (NHQ-K) in Parkinson’s disease (PD) patients across disease stages. @*Methods@#We developed the NHQ-K and performed questionnaire-based interviews with 108 PD patients from three referral hospitals. Clinical associations of nocturnal hypokinesia and its impact on health-related quality of life (HRQoL) were also analyzed. @*Results@#The NHQ-K showed acceptable internal consistency (0.83) and interrater reliability (0.95). Nocturnal hypokinesia significantly affected HRQoL in PD patients at both the early and advanced stages (adjusted p < 0.001). Increased severity of nocturnal hypokinesia was associated with dyskinesias, off-period disability, apathy, and anxious mood in PD patients (adjusted p < 0.01) after controlling for disease severity and medication dose. @*Conclusion@#The NHQ-K is useful for screening nocturnal hypokinesia in PD patients. Given the high impact of nocturnal hypokinesia on HRQoL, comprehensive management of nocturnal disability is needed for PD patients.
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Background@#and Purpose Premanifest mutation carriers with spinocerebellar ataxia (SCA) can exhibit subtle abnormalities before developing ataxia. We summarized the preataxic manifestations of SCA1, -2, -3, and -6, and their associations with ataxia onset. @*Methods@#We included studies of the premanifest carriers of SCA published between January 1998 and December 2019 identified in Scopus and PubMed by searching for terms including ‘spinocerebellar ataxia’ and several synonyms of ‘preataxic manifestation’. We systematically reviewed the results obtained in studies categorized based on clinical, imaging, and laboratory markers. @*Results@#We finally performed a qualitative analysis of 48 papers. Common preataxic manifestations appearing in multiple SCA subtypes were muscle cramps, abnormal muscle reflexes, instability in gait and posture, lower Composite Cerebellar Functional Severity scores, abnormalities in video-oculography and transcranial magnetic stimulation, and gray-matter loss and volume reduction in the brainstem and cerebellar structures. Also, decreased sensory amplitudes in nerve conduction studies were observed in SCA2. Eotaxin and neurofilament lightchain levels were revealed as sensitive blood biomarkers in SCA3. Concerning potential predictive markers, hyporeflexia and abnormalities of somatosensory evoked potentials showed correlations with the time to ataxia onset in SCA2 carriers. However, no longitudinal data were found for the other SCA gene carriers. @*Conclusions@#Our results suggest that preataxic manifestations vary among SCA1, -2, -3, and -6, with some subtypes sharing specific features. Combining various markers into a standardized index for premanifest carriers may be useful for early screening and assessing the risk of disease progression in SCA carriers.
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Uncontrolled blood pressure (BP) in patients with chronic kidney disease (CKD) can lead to serious adverse outcomes. To prevent the occurrence of cardiovascular events (CVEs), and end-stage kidney disease, achieving an optimal BP level is important. Recently, there has been a paradigm shift in the management of BP largely as a result of the Systolic Blood Pressure Intervention Trial (SPRINT), which showed a reduction in CVEs by lowering systolic BP to 120 mmHg. A lower systolic blood pressure (SBP) target has been accepted by the Kidney Disease: Improving Global Outcomes (KDIGO) 2021 guidelines. However, whether intensive control of SBP targeting < 120 mmHg is also effective in patients with CKD is controversial. Notably, this lower target SBP is associated with a higher risk of adverse kidney outcomes. Unfortunately, there have been no randomized controlled trials on this issue involving only patients with CKD, particularly those with advanced CKD. In this review, we discuss the optimal control of BP in patients with CKD in terms of reduction in death and CVEs as well as attenuation of CKD progression based on the evidence-based literature.
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Objective@#The distinction between idiopathic normal pressure hydrocephalus (iNPH) and hydrocephalus ex vacuo caused by encephalic volume loss remains to be established. This study aims to investigate radiological parameters as clinically useful tools to discriminate iNPH from hydrocephalus ex vacuo caused by Alzheimer’s disease (AD). @*Methods@#A total of 54 patients with ventriculomegaly (iNPH, 25; hydrocephalus ex vacuo, 29) were recruited in this study. Consequently, nine radiological parameters were compared between iNPH and hydrocephalus ex vacuo using magnetic resonance imaging (MRI). @*Results@#A small callosal angle (CA), the Sylvian fissure dilatation, and absence of narrowing of superior parietal sulci discriminated the iNPH group from the hydrocephalus ex vacuo group (p<0.05). The final binary logistic regression model included narrowing of superior parietal sulci, degrees of the CA, and height of the Sylvian fissure after controlling for age and global Clinical Dementia Rating (CDR). The composite score made from these three indicators (narrowing of superior parietal sulci, degrees of the CA, and height of the Sylvian fissure) was statistically different between iNPH and hydrocephalus ex vacuo. @*Conclusion@#The narrowing of the CA, dilatation of the Sylvain fissure, and narrowing of superior parietal sulci may be used as radiological key indices and noninvasive tools for the differential diagnosis of iNPH from hydrocephalus ex vacuo.
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Objective@#To assess nocturnal hypokinesia using the Korean version of the Nocturnal Hypokinesia Questionnaire (NHQ-K) in Parkinson’s disease (PD) patients across disease stages. @*Methods@#We developed the NHQ-K and performed questionnaire-based interviews with 108 PD patients from three referral hospitals. Clinical associations of nocturnal hypokinesia and its impact on health-related quality of life (HRQoL) were also analyzed. @*Results@#The NHQ-K showed acceptable internal consistency (0.83) and interrater reliability (0.95). Nocturnal hypokinesia significantly affected HRQoL in PD patients at both the early and advanced stages (adjusted p < 0.001). Increased severity of nocturnal hypokinesia was associated with dyskinesias, off-period disability, apathy, and anxious mood in PD patients (adjusted p < 0.01) after controlling for disease severity and medication dose. @*Conclusion@#The NHQ-K is useful for screening nocturnal hypokinesia in PD patients. Given the high impact of nocturnal hypokinesia on HRQoL, comprehensive management of nocturnal disability is needed for PD patients.
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Background@#and Purpose Premanifest mutation carriers with spinocerebellar ataxia (SCA) can exhibit subtle abnormalities before developing ataxia. We summarized the preataxic manifestations of SCA1, -2, -3, and -6, and their associations with ataxia onset. @*Methods@#We included studies of the premanifest carriers of SCA published between January 1998 and December 2019 identified in Scopus and PubMed by searching for terms including ‘spinocerebellar ataxia’ and several synonyms of ‘preataxic manifestation’. We systematically reviewed the results obtained in studies categorized based on clinical, imaging, and laboratory markers. @*Results@#We finally performed a qualitative analysis of 48 papers. Common preataxic manifestations appearing in multiple SCA subtypes were muscle cramps, abnormal muscle reflexes, instability in gait and posture, lower Composite Cerebellar Functional Severity scores, abnormalities in video-oculography and transcranial magnetic stimulation, and gray-matter loss and volume reduction in the brainstem and cerebellar structures. Also, decreased sensory amplitudes in nerve conduction studies were observed in SCA2. Eotaxin and neurofilament lightchain levels were revealed as sensitive blood biomarkers in SCA3. Concerning potential predictive markers, hyporeflexia and abnormalities of somatosensory evoked potentials showed correlations with the time to ataxia onset in SCA2 carriers. However, no longitudinal data were found for the other SCA gene carriers. @*Conclusions@#Our results suggest that preataxic manifestations vary among SCA1, -2, -3, and -6, with some subtypes sharing specific features. Combining various markers into a standardized index for premanifest carriers may be useful for early screening and assessing the risk of disease progression in SCA carriers.
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Background@#and Purpose: To determine whether the postcessation weight gain modifies the protective effect of smoking on the development of Parkinson’s disease (PD). @*Methods@#This nationwide cohort study included 3,908,687 Korean males aged ≥40 years who underwent at least 2 health checkups biennially between 2009 and 2015. They were grouped into current smokers; quitters with body mass index (BMI) increase, maintenance, and decrease; and never smokers. The occurrence of incident PD was tracked, and Cox proportional-hazard models were used to adjust for potential confounding factors. We also analyzed the impact of weight change regardless of smoking status in the study population. @*Results@#There were 6,871 incident PD cases observed during the 13,059,208 person-years of follow-up. The overall risk of PD was significantly lower in quitters than in never smokers [hazard ratio (HR)=0.78, 95% confidence interval (CI)=0.70–0.86]. The risk of PD was still lower in quitters with BMI increase (HR=0.80, 95% CI=0.65–0.98) and in those with BMI maintenance (HR=0.77, 95% CI=0.68–0.87). This tendency was also observed in quitters with BMI decrease (HR=0.76, 95% CI=0.55–1.06), although it was not as robust as in the other two groups.With respect to weight change alone, BMI increase (HR=1.10, 95% CI=1.02–1.18) but not BMI decrease (HR=1.06, 95% CI=0.98–1.14) significantly increased the PD risk compared to BMI maintenance. @*Conclusions@#Postcessation weight gain in males did not offset the protective impact of smoking on PD development, although overall weight gain predicted an increased risk of PD.
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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.
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Although DNA damage responses (DDRs) are reported to be involved in nitric oxide (NO) production in response to genotoxic stresses, the precise mechanism of DDR-mediated NO production has not been fully understood. Using a genotoxic agent aphidicolin, we investigated how DDRs regulate NO production in bovine aortic endothelial cells. Prolonged (over 24 h) treatment with aphidicolin increased NO production and endothelial NO synthase (eNOS) protein expression, which was accompanied by increased eNOS dimer/monomer ratio, tetrahydrobiopterin levels, and eNOS mRNA expression. A promoter assay using 5’-serially deleted eNOS promoters revealed that Tax-responsive element site, located at −962 to −873 of the eNOS promoter, was respon-sible for aphidicolin-stimulated eNOS gene expression. Aphidicolin increased CREB activity and ectopic expression of dominantnegative inhibitor of CREB, A-CREB, repressed the stimulatory effects of aphidicolin on eNOS gene expression and its promoter activity. Co-treatment with LY294002 decreased the aphidicolin-stimulated increase in p-CREB-Ser133 level, eNOS expression, and NO production. Furthermore, ectopic expression of dominant-negative Akt construct attenuated aphidicolin-stimulated NO production. Aphidicolin increased p-ATM-Ser1981 and the knockdown of ATM using siRNA attenuated all stimulatory effects of aphidicolin on p-Akt-Ser473 , p-CREB-Ser133 , eNOS expression, and NO production. Additionally, these stimulatory effects of aphidicolin were similarly observed in human umbilical vein endothelial cells. Lastly, aphidicolin increased acetylcholine-induced vessel relaxation in rat aortas, which was accompanied by increased p-ATM-Ser1981 , p-Akt-Ser473 , p-CREB-Ser 133 , and eNOS expression. In conclusion, our results demonstrate that in response to aphidicolin, activation of ATM/Akt/CREB/eNOS signaling cascade mediates increase of NO production and vessel relaxation in endothelial cells and rat aortas.
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Humans , Follow-Up Studies , Parkinson Disease , Quality of Life , Weights and MeasuresABSTRACT
BACKGROUND@#In South Korea, as an aged society, an understanding of dementia and its risk factors is important from clinical and healthcare policy perspectives. Relationship between cognitive impairment and body weight or weight changes have been reported, but these were contradictory. We have evaluated the association between weight changes and cognitive decline using national level longitudinal data.@*METHODS@#Data from the Korean Longitudinal Study of Ageing from 2006 to 2012 were used. Association between weight changes and decline in cognitive function as measured by K-MMSE (the Korean version of the Minimental state examination) score was assessed by multivariate logistic regression. Weight changes were calculated from 1st wave and 3rd wave survey data, and classified into five groups as stable, increases, decreases of >10%, or 5%–10%.@*RESULTS@#About 37% of the total participants (n=4,512) were 65 years or older. These participants made up the largest proportion of the groups with weight change exceeding 10%. Multivariate logistic regression analyses revealed that weight changes exceeding 10% (10% increase vs stable, adjusted OR [aOR] 1.47, 95% confidence interval (CI) 1.11–1.95; 10% decrease vs stable, aOR 1.44, 95% CI 1.11–1.88) were significant predictive factors for decline in cognitive function. In subgroup analyses, the association between weight changes and cognitive decline was significant in males aged over 65 years and in normal BMI groups.@*CONCLUSION@#Weight changes, both increases and decreases exceeding 10% of baseline, were significantly associated with declines in cognitive function among older adults in South Korea.
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OBJECTIVE: It is unclear whether the decline in dopamine transporters (DAT) differs among idiopathic rapid eye movement sleep behavior disorder (iRBD) patients with different levels of olfactory impairment. This study aimed to characterize DAT changes in relation to nonmotor features in iRBD patients by olfactory loss. METHODS: This prospective cohort study consisted of three age-matched groups: 30 polysomnography-confirmed iRBD patients, 30 drug-naïve Parkinson's disease patients, and 19 healthy controls without olfactory impairment. The iRBD group was divided into two groups based on olfactory testing results. Participants were evaluated for reported prodromal markers and then underwent 18F-FP-CIT positron emission tomography and 3T MRI. Tracer uptakes were analyzed in the caudate, anterior and posterior putamen, substantia nigra, and raphe nuclei. RESULTS: Olfactory impairment was defined in 38.5% of iRBD patients. Mild parkinsonian signs and cognitive functions were not different between the two iRBD subgroups; however, additional prodromal features, constipation, and urinary and sexual dysfunctions were found in iRBD patients with olfactory impairment but not in those without. Tracer uptake showed significant group differences in all brain regions, except the raphe nuclei. The iRBD patients with olfactory impairment had uptake reductions in the anterior and posterior putamen, caudate, and substantia nigra (p < 0.016 in all, adjusted for age), which ranged from 0.6 to 0.8 of age-normative values. In contrast, those without olfactory impairment had insignificant changes in all regions ranging above 0.8. CONCLUSION: There was a clear distinction in DAT loss and nonmotor profiles by olfactory status in iRBD.
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Humans , Brain , Cognition , Cohort Studies , Constipation , Dopamine Plasma Membrane Transport Proteins , Dopamine , Magnetic Resonance Imaging , Parkinson Disease , Positron-Emission Tomography , Prospective Studies , Putamen , Raphe Nuclei , REM Sleep Behavior Disorder , Sleep, REM , Smell , Substantia NigraABSTRACT
OBJECTIVE: To determine the prevalence of osteoporosis (OP) and osteoporotic vertebral fracture (OVF) in people with Parkinson’s disease (PD) in Korea and its association with socioeconomic status.METHODS: Using Health Insurance Review and Assessment Service-National Inpatient Sample (HIRA-NIS) data from 2009 to 2013, we estimated the annual prevalence of PD, OP, and OVF and investigated its association with socioeconomic status using data from National Health Insurance (NHI) beneficiaries and Medical Aid (MA) recipients. This study was supported by research funding from Korean Society for Bone and Mineral Research 2015. There were no study-specific biases related to conflicts of interest.RESULTS: The number of PD patients in the HIRA-NIS increased each year from 2009 to 2013. Among patients with PD, the standardized prevalence rates of OP and OVF increased from 2009 to 2013; from 23.2 to 27.8 and from 2.8 to 4.2, respectively. Among patients with PD with OP, the prevalence of OVF were 12.2% and 15.1% in 2009 and 2013, respectively. The standardized prevalence rates of PD with OP and PD with OVF were significantly higher in MA recipients than in NHI beneficiaries.CONCLUSION: The prevalence of PD both with OP and with OVF increased and the prevalence was higher in MA recipients than in NHI beneficiaries. These findings may suggest that age over 65 years, female and low income may be a significant factor related to PD occurring with OP and OVF.