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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.
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Doença de Parkinson/diagnóstico , Transtornos do Sono-Vigília/diagnóstico , Idoso , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Parkinson/complicações , Doença de Parkinson/patologia , Reprodutibilidade dos Testes , República da Coreia , Índice de Gravidade de Doença , Transtornos do Sono-Vigília/complicações , Transtornos do Sono-Vigília/patologia , Inquéritos e Questionários , TraduçãoRESUMO
We aimed to compare Dysport (abobotulinumtoxinA, Ipsen Biopharm, Slough, UK) and Botox (onabotulinumtoxinA, Allergan, Irvine, CA, USA) at a 2.5:1 ratio in the treatment of cervical dystonia (CD). A Dysport/Botox ratio of lower than 3:1 was suggested as a more appropriate conversion ratio, considering its higher efficacy and more frequent incidence of adverse effects not only in the treatment of CD but also in other focal movement disorders. A randomized, double-blind, multicenter, non-inferiority, two-period crossover study was done in CD, with a duration of at least 18 months. Patients were randomly assigned to treatment for the first period with Dysport or Botox, and they were followed up for 16 weeks after the injection. After a 4-week washout period, they were switched to the other formulation and then followed up for 16 weeks. The primary outcome was the changes in the Tsui scale between the baseline value and that at 1 month after each injection. A total of 103 patients were enrolled, and 94 completed the study. Mean changes in the Tsui scale between baseline and 4 weeks after each injection tended to favor Botox; however, this was not statistically significant (4.0 ± 3.9 points for the Dysport treatment vs. 4.8 ± 4.1 points for Botox; 95% confidence interval, -0.1-1.7; P = 0.091). The mean change of the Toronto western spasmodic torticollis rating scale score, the proportion of improvement in clinical global impression and patient global impression, and the incidences of adverse events were not significantly different between the two treatments. With regard to safety and efficacy, Dysport was not inferior to Botox in patients with CD at a conversion factor of 2.5:1. [clinicaltrial.gov: NCT00950664]
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Inibidores da Liberação da Acetilcolina/uso terapêutico , Toxinas Botulínicas Tipo A/uso terapêutico , Torcicolo/tratamento farmacológico , Adulto , Idoso , Método Duplo-Cego , Combinação de Medicamentos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Dor/etiologia , Medição da Dor , Torcicolo/complicações , Resultado do Tratamento , Adulto JovemRESUMO
Drug-induced parkinsonism (DIP) is the common cause of parkinsonism. It is difficult to make a differentiation between DIP and Parkinson's disease (PD) because there are no notable differences in the clinical characteristics between the two entities. In this study, we examined the relationship between the characteristics of [(18)F] fluorinated-N-3-fluoropropyl-2-ß-carboxymethoxy-3-ß-(4-iodophenyl)nortropane (FP-CIT) positron emission tomography (PET) images and clinical features in DIP patients. We retrospectively studied 76 patients with DIP who underwent [(18)F] FP-CIT PET. We also enrolled 16 healthy controls who underwent it. We compared the clinical characteristics between the DIP patients with normal [(18)F] FP-CIT PET scans and those with abnormal ones. Symmetric parkinsonism was more frequent in the patients with normal [(18)F] FP-CIT PET scans as compared with those with abnormal ones. Interval from drug intake to onset of parkinsonism was longer in the patients with abnormal [(18)F] FP-CIT PET scans as compared with those with normal ones. A semi-quantitative analysis showed that specific to non-specific binding ratios in the putamen was lower in the patients with abnormal [(18)F] FP-CIT PET scans as compared with those with normal ones and the age-matched control group. Our results suggest that symmetric parkinsonism was more prevalent, and the duration of drug exposure before the onset of parkinsonism was shorter in the patients with normal [(18)F] FP-CIT PET scans as compared with those with abnormal ones.
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Encéfalo/diagnóstico por imagem , Doença de Parkinson Secundária/induzido quimicamente , Doença de Parkinson Secundária/diagnóstico por imagem , Idoso , Idoso de 80 Anos ou mais , Encéfalo/fisiopatologia , Mapeamento Encefálico , Feminino , Radioisótopos de Flúor , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Parkinson Secundária/fisiopatologia , Tomografia por Emissão de Pósitrons , Compostos Radiofarmacêuticos , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , TropanosRESUMO
OBJECTIVE: The Scales for Outcomes in Parkinson's Disease-Cognition (SCOPA-Cog) was developed to assess cognition in patients with Parkinson's disease (PD). In this study, we aimed to evaluate the validity and reliability of the Korean version of the SCOPACog (K-SCOPA-Cog). METHODS: We enrolled 129 PD patients with movement disorders from 31 clinics in South Korea. The original version of the SCOPA-Cog was translated into Korean using the translation-retranslation method. The test-retest method with an intraclass correlation coefficient (ICC) and Cronbach's alpha coefficient were used to assess reliability. Spearman's rank correlation analysis with the Montreal Cognitive Assessment-Korean version (MOCA-K) and the Korean Mini-Mental State Examination (K-MMSE) were used to assess concurrent validity. RESULTS: The Cronbach's alpha coefficient was 0.797, and the ICC was 0.887. Spearman's rank correlation analysis revealed a significant correlation with the K-MMSE and MOCA-K scores (r = 0.546 and r = 0.683, respectively). CONCLUSION: Our. RESULTS: demonstrate that the K-SCOPA-Cog has good reliability and validity.
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Recent genome-wide association studies (GWAS) have discovered several Alzheimer disease (AD) susceptibility loci. However, the identified susceptibility loci are substantially inconsistent across GWAS. We aimed to investigate the association of top associated variants in GWAS with AD in Korean population. We selected 86 single-nucleotide polymorphisms (SNPs) selected from 12 genes (ABCA7, APOE, BIN1, CD2AP, CD33, CLU, CR1, EPHA1, LRAT, MS4A6A, PCDH11X, and PICALM) and genotyped in 290 AD cases and 554 unrelated controls from the same region. Three SNPs [rs429358 in APOE: odds ratio (OR)=4.24, 95% confidence interval (CI)=3.01-5.96, P=1.23×10; rs2075650 in APOE: OR=3.57, 95% CI=2.51-5.06, P=1.23×10; and rs677909 in PICALM: OR=0.63, 95% CI=0.49-0.81, P=0.00036, log additive model] were significantly associated with AD susceptibility after correction for multiple testing. Six additional PICALM SNPs, 3 ABCA7 SNPs, and 1 APOE, CD33, and BIN1 SNPs each had significant uncorrected P values. There was no significant association for age at onset of AD. Our results confirm the association of PICALM gene (encoding phosphatidylinositol-binding protein) in addition to APOE gene with AD susceptibility in Korean population but did not show significant associations of other susceptibility loci with AD.
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Doença de Alzheimer/genética , Povo Asiático/genética , Predisposição Genética para Doença/genética , Estudo de Associação Genômica Ampla , Proteínas Monoméricas de Montagem de Clatrina/genética , Idade de Início , Idoso , Idoso de 80 Anos ou mais , Doença de Alzheimer/epidemiologia , Estudos de Casos e Controles , Feminino , Genótipo , Humanos , Masculino , Pessoa de Meia-Idade , Polimorfismo de Nucleotídeo ÚnicoRESUMO
Background: Studies of secondary movement disorder (MD) caused by cerebrovascular diseases have primarily focused on post-stroke MD. However, MD can also result from cerebral artery stenosis (CAS) without clinical manifestations of stroke. In this study, we aimed to investigate the clinical characteristics of MD associated with CAS. Materials and Methods: A nationwide multicenter retrospective analysis was performed based on the data from patients with CAS-associated MDs from 16 MD specialized clinics in South Korea, available between January 1999 and September 2019. CAS was defined as the >50% luminal stenosis of the major cerebral arteries. The association between MD and CAS was determined by MD specialists using pre-defined clinical criteria. The collected clinical information included baseline demographics, features of MD, characteristics of CAS, treatment, and MD outcomes. Statistical analyses were performed to identify factors associated with the MD outcomes. Results: The data from a total of 81 patients with CAS-associated MD were analyzed. The mean age of MD onset was 60.5 ± 19.7 years. Chorea was the most common MD (57%), followed by tremor/limb-shaking, myoclonus, and dystonia. Atherosclerosis was the most common etiology of CAS (78%), with the remaining cases attributed to moyamoya disease (MMD). Relative to patients with atherosclerosis, those with MMD developed MD at a younger age (p < 0.001) and had a more chronic mode of onset (p = 0.001) and less acute ischemic lesion (p = 0.021). Eight patients who underwent surgical treatment for CAS showed positive outcomes. Patients with acute MD onset had a better outcome than those with subacute-to-chronic MD onset (p = 0.008). Conclusions: This study highlights the spectrum of CAS-associated with MD across the country. A progressive, age-dependent functional neuronal modulation in the basal ganglia due to CAS may underlie this condition.
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We performed pupillometer testing on 132 patients with Parkinson's disease, stratified into two groups according to the disease stage. Neurological examinations and pupillometry were performed in the ON state. Patients in the Hoehn and Yahr stages 1 and 2 comprised the early group, and patients in stages 3-5 formed the late group. We performed age- and sex-matched (2:1) propensity score matching to compensate for the effect of age on pupil light reflex. Eight pupillometer parameters were measured and compared between the two groups. After the propensity score matching, the early group had 64 patients and the late group had 32 patients. The late group had a longer disease duration and took a higher levodopa equivalent dose than the early group. The constriction velocity (P = 0.006) and maximum constriction velocity (P = 0.005) were significantly faster in the early group than in the late group. Pupil size, minimum diameter, and dilation velocity were similar in both groups. The pupillary contraction velocity decreased with the disease progression, suggesting that the progression of Parkinson's disease could be identified by the pupil constriction velocity.
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Progressão da Doença , Exame Neurológico , Doença de Parkinson/patologia , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Exame Neurológico/métodos , Doença de Parkinson/diagnóstico , Doença de Parkinson/tratamento farmacológico , Pupila/efeitos dos fármacos , Reflexo Pupilar/fisiologia , Visão Ocular/efeitos dos fármacos , Visão Ocular/fisiologiaRESUMO
123I-metaiodobenzylguanidine (MIBG) cardiac scintigraphy was performed to assess cardiac autonomic dysfunction and demonstrate its correlation with clinical and polysomnographic characteristics in patients with isolated rapid eye movement (REM) sleep behavior disorder. All subjects including 39 patients with isolated REM sleep behavior disorder and 17 healthy controls underwent MIBG cardiac scintigraphy for cardiac autonomic dysfunction assessment. The isolated REM sleep behavior disorder was confirmed by in-lab overnight polysomnography. A receiver operating curve was constructed to determine the cut-off value of the early and delayed heart-to-mediastinum ratio in patients with isolated REM sleep behavior disorder. Based on each cut-off value, a comparison analysis of REM sleep without atonia was performed by dividing isolated REM sleep behavior disorder patients into two groups. MIBG uptake below the cut-off value was associated with higher REM sleep without atonia. The lower heart-to-mediastinum ratio had significantly higher REM sleep without atonia (%), both with cut-off values of early (11.0 ± 5.6 vs. 29.3 ± 23.2%, p = 0.018) and delayed heart-to-mediastinum ratio (9.1 ± 4.3 vs. 30.0 ± 22.9%, p = 0.011). These findings indicate that reduced MIBG uptake is associated with higher REM sleep without atonia in isolated REM sleep behavior disorder.
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INTRODUCTION: Nocturnal stridor, a life-threatening condition linked to respiratory failure and sudden death during sleep, is a serious issue in patients with multiple system atrophy (MSA). However, little is known about polysomnographic findings and clinical features of MSA patients with nocturnal stridor. Hence, we investigated video-polysomnography (VPSG) findings and clinical features associated with nocturnal stridor in patients with MSA. METHODS: We retrospectively analyzed the clinical data of patients with MSA (nâ¯=â¯49) who underwent overnight VPSG for the evaluation of sleep-disordered breathing. The presence of nocturnal stridor was confirmed based on overnight VPSG findings. Clinical data, including VPSG findings and clinical features, were compared between MSA patients with and without nocturnal stridor. RESULTS: Nocturnal stridor was present in 31 (63.3%) patients with MSA. Patients with stridor showed significantly higher apnea-hypopnea, respiratory disturbance, and oxygen desaturation indices than those without stridor (Pâ¯=â¯0.024, Pâ¯=â¯0.049, and Pâ¯=â¯0.006, respectively). Patients with stridor had more severe axial motor features, more impaired activities of daily living, and longer disease duration than those without stridor (Pâ¯=â¯0.012, Pâ¯=â¯0.036, and Pâ¯=â¯0.003, respectively). However, there were no significant between-group differences in sex, age at disease onset, MSA subtype, parkinsonian features, cerebellar ataxia, residual urine volume, or systolic and diastolic blood pressure change. CONCLUSIONS: MSA with nocturnal stridor is related to higher apnea indices in conjunction with higher O2 desaturation index, more severe axial motor features, more impaired activities of daily living, and longer disease duration.
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Atrofia de Múltiplos Sistemas , Sons Respiratórios , Síndromes da Apneia do Sono , Atividades Cotidianas , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Atrofia de Múltiplos Sistemas/complicações , Atrofia de Múltiplos Sistemas/fisiopatologia , Polissonografia , Sons Respiratórios/diagnóstico , Sons Respiratórios/etiologia , Sons Respiratórios/fisiopatologia , Estudos Retrospectivos , Síndromes da Apneia do Sono/diagnóstico , Síndromes da Apneia do Sono/etiologia , Síndromes da Apneia do Sono/fisiopatologia , Fatores de Tempo , Gravação em VídeoRESUMO
BACKGROUND: There have been several pathologic data that support an association between postural instability (PI) and autonomic dysfunction in Parkinson's disease (PD). The purpose of this study was to investigate the correlation of PI and autonomic deficits in early PD. METHODS: We collected 17 patients with a diagnosis of early PD. PI was assessed by computerized dynamic posturography (CDP). Standardized autonomic function test (AFT) and time and frequency domain spectral analysis of heart rate variability (HRV) were performed. CDP data obtained from the 21 patients were compared to that from age- and sex-matched healthy controls. We collected HRV data from 18 other age- and sex-matched controls. All patients were evaluated in the "OFF" state. We used Mann-Whitney U-test to compare parameters of CDP between the early PD and control groups. Spearman correlation was used for correlation analysis between parameters of CDP and autonomic function test in PD patients. RESULTS: Most patients (76.5%) showed mild or moderate autonomic dysfunction in the standardized AFT. In CDP, sensory ratios of equilibrium score (e.g., visual and vestibular) and composite scores were significantly lower in PD patients than in controls. In HRV, the low-frequency/high-frequency ratio during the tilt and the gap of low- frequency/high-frequency ratio from supine to tilt were significantly different in both groups. The parameters of time and frequency domains of HRV reflecting parasympathetic function were correlated with equilibrium scores for somatosensory organization test in CDP. DISCUSSION: PI was associated with parasympathetic autonomic dysfunction in early PD. This result was in accordance with a previous assumption that PI in PD is related to parasympathetic cholinergic neuron loss in the brainstem.
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INTRODUCTION: Long-term efficacy and safety of subthalamic nucleus deep brain stimulation (STN DBS) in patients with young-onset Parkinson's disease (YOPD) and late-onset PD (LOPD) (i.e. motor symptom initial appearance at ages ≤40 and > 40 years, respectively) was compared to identify relationships between PD onset age and the efficacy of DBS. METHODS: Statistical analyses compared specific motor and non-motor features among 13 patients with YOPD and 11 with LOPD. Medication reduction patterns and dyskinesia severity scores at baseline and after 1, 3, 5, and 10 years of follow-up were also analyzed using a repeated measures ANOVA tests. Lastly, a correlation analysis identified relationships between the impact of DBS settings (volume of activated tissue) and levodopa equivalent daily dose (LED), dyskinesia severity scores, Unified Parkinson's Disease Rating Scale (UPDRS) part III, and UPDRS part II (disability) scores. RESULTS: Ten years after DBS surgery, the reduction of LED from baseline (85.9 ± 592.6 mg versus 623.2 ± 464.9 mg; p = .023) and levodopa-induced dyskinesia (LID) scores (Unified Dyskinesia Rating Scale [UDysRS] parts III items 16-22; 1.6 ± 2.8 versus 5.5 ± 4.1; p = .013) were significantly lower in YOPD patients than LOPD patients. There were no significant differences between the two groups regarding UPDRS part III score improvement in response to levodopa, psychosis occurrence, or adverse effects. CONCLUSION: Ten years after STN DBS surgery, LOPD patients showed greater LED reduction, and YOPD patients showed greater LID improvement, although the general long-term outcomes were similar between YOPD and LOPD patients.
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Estimulação Encefálica Profunda , Doença de Parkinson , Núcleo Subtalâmico , Adulto , Seguimentos , Humanos , Doença de Parkinson/terapia , Resultado do TratamentoRESUMO
BACKGROUND AND PURPOSE: Impulse-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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BACKGROUND AND PURPOSE: This study aimed to determine the clinimetric properties of the Korean version of Parkinson's Disease Sleep Scale-2 (K-PDSS-2) and whether distinct subtypes of sleep disturbance can be empirically identified in patients with Parkinson's disease (PD) using the cross-culturally validated K-PDSS-2. METHODS: The internal consistency, test-retest reliability, scale precision, and convergent validity of K-PDSS-2 were assessed in a nationwide, multicenter study of 122 patients with PD. Latent class analysis (LCA) was used to derive subgroups of patients who experienced similar patterns of sleep-related problems and nocturnal disabilities. RESULTS: The total K-PDSS-2 score was 11.67±9.87 (mean±standard deviation) at baseline and 12.61±11.17 at the retest. Cronbach's α coefficients of the total K-PDSS-2 scores at baseline and follow-up were 0.851 and 0.880, respectively. The intraclass correlation coefficients over the 2-week study period ranged from 0.672 to 0.848. The total K-PDSS-2 score was strongly correlated with health-related quality of life measures and other corresponding nonmotor scales. LCA revealed three distinct subtypes of sleep disturbance in the study patients: "less-troubled sleepers," "PD-related nocturnal difficulties," and "disturbed sleepers." CONCLUSIONS: K-PDSS-2 showed good clinimetric attributes in accordance with previous studies that employed the original version of the PDSS-2, therefore confirming the cross-cultural usefulness of the scale. This study has further documented the first application of an LCA approach for identifying subtypes of sleep disturbance in patients with PD.
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BACKGROUND AND PURPOSE: We investigated the frequency and clinical features of restless legs syndrome (RLS) in patients with Parkinson's disease (PD). METHODS: This study included 74 PD patients. RLS was diagnosed in face-to-face assessments of all of the subjects based on diagnostic criteria of the International Restless Legs Syndrome Study Group revised in 2003. We analyzed the clinical features of PD patients with and without RLS and compared the data to idiopathic RLS. RESULTS: The frequency of RLS in the cohort was 21.6% (n=16). Two (12.5%) of the patients with RLS were not treated with dopaminergic drugs, while 14 (24.1%) of the 58 patients without RLS received treatment with dopaminergic drugs. Anxiety, depression, and quality of life (QoL) were significantly worst in patients with RLS. PD patients with RLS had significantly worse sleep quality (p=0.003) and worse scores on the cardiovascular subscale of the Scales for Outcomes in Parkinson's Disease for Autonomic Symptoms (p=0.031) compared to those without RLS. In the group of PD patients with RLS, RLS preceding PD onset was related to a lower Hoehn and Yahr stage. CONCLUSIONS: We found that the frequency of RLS in the present patients with PD was higher than that in our previous study of a general population of RLS subjects. Compared to the PD patients without RLS, the present PD patients with RLS suffered from worse sleep quality and QoL, depression, anxiety, and autonomic disturbances, especially those with cardiovascular problems.
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BACKGROUND: The severity of parkinsonism and response to levodopa vary in patients with multiple system atrophy (MSA) because of the heterogeneity of nigrostriatal neuropathology. OBJECTIVE: To investigate the difference in clinical features between MSA patients with predominantly pre-synaptic nigrostriatal dysfunction and those with trans-synaptic nigrostriatal dysfunction. METHODS: We retrospectively analyzed clinical data of 61 patients with MSA who underwent both [18F]FP-CIT-PET and [18F]FDG-PET within 3â¯months of clinical evaluation, and who had ≤3â¯years of disease duration. Tracer uptake of the striatum on [18F]FP-CIT-PET and glucose metabolism of the striatum on [18F]FDG-PET were analyzed using eight striatal subregional volumes-of-interest templates. The patients were classified into two subgroups according to the predominant pre-synaptic tracer uptake loss of the posterior putamen on [18F]FP-CIT-PET (MSA-SNpc, nâ¯=â¯21) and trans-synaptic dopaminergic dysfunction reflected by both [18F]FP-CIT-PET and [18F]FDG-PET (MSA-STR, nâ¯=â¯40). RESULTS: Parkinsonian features were significantly more severe in the MSA-STR group than in the MSA-SNpc group (Pâ¯=â¯.005) and cerebellar ataxia was significantly more severe in the MSA-SNpc group (Pâ¯=â¯.036). The cerebellar type of MSA was significantly more common in the MSA-SNpc group (Pâ¯=â¯.001). There was no difference in age at onset, disease duration at the time of study, or Mini-Mental Status Examination scores between the groups. CONCLUSIONS: Patients with MSA showed distinct clinical features depending on whether the pattern of nigrostriatal dysfunction was predominantly pre-synaptic or trans-synaptic.
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Corpo Estriado/fisiopatologia , Atrofia de Múltiplos Sistemas/fisiopatologia , Putamen/fisiopatologia , Substância Negra/fisiopatologia , Adulto , Idoso , Estudos de Casos e Controles , Ataxia Cerebelar/complicações , Ataxia Cerebelar/fisiopatologia , Corpo Estriado/metabolismo , Feminino , Fluordesoxiglucose F18/metabolismo , Neuroimagem Funcional , Humanos , Masculino , Pessoa de Meia-Idade , Atrofia de Múltiplos Sistemas/complicações , Atrofia de Múltiplos Sistemas/diagnóstico , Vias Neurais/fisiopatologia , Transtornos Parkinsonianos/complicações , Transtornos Parkinsonianos/fisiopatologia , Tomografia por Emissão de Pósitrons , Terminações Pré-Sinápticas/fisiologia , Putamen/metabolismo , Substância Negra/metabolismo , Tropanos/metabolismoRESUMO
BACKGROUND AND PURPOSE: Hand tremor is one of the most frequent symptoms in movement disorders, and differential diagnoses for hand tremor include Parkinson's disease (PD) and essential tremor (ET). However, accurately differentiating between PD and ET in clinical practice remains challenging in patients presenting with hand tremor. We investigated whether a questionnaire-based survey could be useful as a screening tool in patients with hand tremor. METHODS: A questionnaire related to hand tremor consisting of 12 items was prospectively applied to patients with PD or ET in three movement-disorder clinics. Each question was analyzed, and a query-based scoring system was evaluated for differentiating hand tremors between PD and ET. RESULTS: This study enrolled 24 patients with PD and 25 patients with ET. Nine of the 12 questions differed significantly between PD and ET: 1 about resting tremor, 4 questions about action tremor, and 4 about asymmetry. A receiver operating characteristics curve analysis revealed that the 9-item questionnaire showed a good discrimination ability, with a sensitivity of 88% and a specificity of 84%. CONCLUSIONS: The developed Hand Tremor Questionnaire might be a good screening tool for hand tremors in patients with PD and ET.
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OBJECTIVE: To compare the therapeutic and adverse effects of globus pallidus interna (GPi) and subthalamic nucleus (STN) deep brain stimulation (DBS) for the treatment of advanced Parkinson's disease (PD). METHODS: We retrospectively analyzed the clinical data of patients with PD who underwent GPi (n = 14) or STN (n = 28) DBS surgery between April 2002 and May 2014. The subjects were matched for age at surgery and disease duration. The Unified Parkinson's Disease Rating Scale (UPDRS) scores and levodopa equivalent dose (LED) at baseline and 12 months after surgery were used to assess the therapeutic effects of DBS. Adverse effects were also compared between the two groups. RESULTS: At 12 months, the mean changes in the UPDRS total and part I-IV scores did not differ significantly between the two groups. However, the subscores for gait disturbance/postural instability and dyskinesia were significantly more improved after GPi DBS than those after STN DBS (p = 0.024 and 0.016, respectively). The LED was significantly more reduced in patients after STN DBS than that after GPi DBS (p = 0.004). Serious adverse effects did not differ between the two groups (p = 0.697). CONCLUSION: The patients with PD showed greater improvement in gait disturbance/postural instability and dyskinesia after GPi DBS compared with those after STN DBS, although the patients had a greater reduction in LED after STN DBS. These results may provide useful information for optimal target selection for DBS in PD.
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We investigated the role of mortalin (HSPA9) and its interaction with other mitochondria-related genes (parkin, PINK1, DJ1, and COQ2) as a risk factor for Parkinson's disease (PD) and Alzheimer's disease (AD) in 500 PD, 400 AD, and 500 control subjects. The HSPA9 variants identified by direct sequencing or its interaction with other genes assessed by genetic risk scores did not show a significant association with PD or AD risk. Our findings did not provide a strong evidence for the role of HAPA9 and its interaction with other mitochondria-related genes as a genetic risk factor for PD or AD.
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Doença de Alzheimer/genética , Epistasia Genética/genética , Estudos de Associação Genética , Variação Genética/genética , Proteínas de Choque Térmico HSP70/genética , Mitocôndrias/genética , Proteínas Mitocondriais/genética , Doença de Parkinson/genética , Alquil e Aril Transferases/genética , Feminino , Humanos , Masculino , Proteína Desglicase DJ-1/genética , Proteínas Quinases/genética , Risco , Fatores de Risco , Ubiquitina-Proteína Ligases/genéticaRESUMO
STUDY OBJECTIVES: REM sleep behavior disorder (RBD) is a parasomnia that is commonly associated with neurodegenerative disorders. We aimed to validate the Hong Kong version of the self-reported RBD questionnaire (RBDQ-HK) with the Korean version (RBDQ-KR) and to investigate its clinical usefulness. METHODS: One-hundred five patients with RBD and 105 age- and sex-matched controls were enrolled. Thirty were randomly selected for a retest with the questionnaire after 2 to 4 weeks without any treatment. Receiver operating characteristic curve and exploratory factor analysis were used to evaluate the scale, which had a score ranging from 0 to 100. RESULTS: There was no difference in mean age and sex in patients with RBD and control subjects. Patients with RBD had a significantly higher total RBDQ-KR score than the control group (P < .001). The RBDQ-KR demonstrated high sensitivity (93.3%), specificity (89.5%), positive predictive value (89.9%), and negative predictive value (93.1%). Cronbach α coefficient for internal consistency of the total score of the RBDQ-KR was 0.9, which shows high reliability. Exploratory factor analysis showed two components, dream-related and behavioral factors, consistent with the main clinical features of RBD. Based on the results, the best cutoff for the total score ranging from 0 to 100 was at 18/19 and for factor 2 ranging from 0 to 75 was at 11. CONCLUSIONS: This study showed RBDQ-KR is a valid and reliable questionnaire for RBD symptoms and severity in Korea. It serves as an effective tool to identify patients with RBD and to facilitate future clinical and research studies.