• Title/Summary/Keyword: multiple system atrophy

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Clinical Usefulness of Dopamine Transporter Imaging (도파민운반체 영상의 임상적 유용성)

  • Kim, Jong-Min;Kim, Yu-Kyeong;Kim, Sang-Eun;Jeon, Beom-S.
    • Nuclear Medicine and Molecular Imaging
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    • v.41 no.2
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    • pp.152-157
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    • 2007
  • Imaging of the dopamine transporter (DAT) provides a marker for the integrity of presynaptic nigrostriatal dopaminergic system. DAT density is reduced in Parkinson disease, multiple system atrophy, and progressive supranuclear palsy. In patients with suspicious parkinsonism, normal DAT imaging suggests an alternative diagnosis such as essential tremor, vascular parkinsonism, or drug-induced parkinsonism. DAT imaging is a useful tool to aid clinician's differential diagnosis in parkinsonism.

Vegetative endocarditis associated with Arcanobacterium haemolyticum in a Holstein cow: a case report

  • Jung, Ji-Youl;Lee, Kyoung-Kap;Kim, Jae-Hoon
    • Korean Journal of Veterinary Research
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    • v.61 no.3
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    • pp.24.1-24.5
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    • 2021
  • A 4-year-old Holstein cow with progressive atrophy and ataxia was submitted for diagnosis. The postmortem examination revealed multifocal yellowish nodules in the pulmonary parenchyma and vegetative masses in the mitral and tricuspid valve of the heart. Both kidneys were severely enlarged, with multiple yellow nodules on the parenchyma. Histopathologically, pulmonary abscesses, vegetative endocarditis, suppurative glomerulonephritis, and fibrino-purulent arthritis were observed. The tiny β-hemolytic bacterial colonies were isolated from the lesions and identified as Arcanobacterium haemolyticum by the VITEK 2 system (bioMérieux, USA). This is the first documented report of an A. haemolyticum infection in a Holstein cow in Korea.

Fishing for synucleinopathy models

  • Noor, Suzita Mohd;Norazit, Anwar
    • Fisheries and Aquatic Sciences
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    • v.25 no.3
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    • pp.117-139
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    • 2022
  • Synucleinopathies such as Parkinson's disease (PD) are incurable neurodegenerative conditions characterised by the abnormal aggregation of α-synuclein protein in neuronal cells. In PD, fibrillary synuclein aggregation forms Lewy bodies and Lewy neurites in the substantia nigra and cortex on the brain. Dementia with Lewy bodies and multiple system atrophy are also associated with α-synuclein protein abnormalities. α-synuclein is one of three synuclein proteins, and while its precise function is still unknown, one hypothesis posits that α-synuclein propagates from the enteric nervous system through the vagus nerve and into the brain, resulting in synucleinopathy. Studies on synucleinopathies should thus encompass not only the central nervous system but must necessarily include the gut and microbiome. The zebrafish (Danio rerio) is a well-established model for human neuronal pathologies and have been used in studies ranging from genetic models of hereditary disorders to neurotoxin-induced neurodegeneration as well as gut-brain-axis studies. There is significant genetic homology between zebrafish and mammalian vertebrates which is what makes the zebrafish so amenable to modelling human conditions but in the case of synucleinopathies, the zebrafish notably does not possess an α-synuclein homolog. Synuclein orthologs are present in the zebrafish however, and transgenic zebrafish that carry human α-synuclein have been generated. In addition, the zebrafish is a highly advantageous model and ideal replacement for reducing the use of mammalian models. This review discusses the application of the zebrafish as a model for synucleinopathies in efforts to further understand synuclein function and explore therapeutic strategies.

Comparison of Clinical Characteristics and Polysomnographic Findings between REM Sleep Behavior Disorder with and without Associated Central Nervous System Disorders (중추신경계질환 동반 여부에 따른 렘수면 행동장애의 임상 특성과 수면다원기록소견 소견 비교)

  • Lee, Yu-Jin;Jeong, Do-Un
    • Sleep Medicine and Psychophysiology
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    • v.12 no.1
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    • pp.58-63
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    • 2005
  • Objectives: REM sleep behavior disorder (RBD), characterized by excessive motor activity during REM sleep, is associated with loss of muscle atonia. In recent years, it has been reported that RBD has high co-morbidity with CNS disorders (especially, Parkinson's disease, dementia, multiple system atrophy, etc.). We aimed to assess differences in clinical and polysomnographic findings among RBD patients, depending on the presence or absence of central nervous system (CNS) disorders. Methods: The medical records and polysomnographic data of 81 patients who had been diagnosed as having RBD were reviewed. The patients were classified into two groups: associated RBD (aRBD, i.e., with a clinical history and/or brain MRI evidence of CNS disorder) and idiopathic RBD (iRBD, i.e., without a clinical history and/or brain MRI evidence of CNS disorder) groups. Twenty-one patients (25.9%) belonged to the aRBD group and 60 patients (74.1%) belonged to the iRBD group. The clinical characteristics and polysomnographic findings of the two groups were compared. Results: Periodic limb movement disorder (PLMD), i.e., PLMI (periodic limb movement index)>5, was observed more frequently in the aRBD group than in the iRBD group (p<0.001, Fisher's exact test). Also, obstructive sleep apnea syndrome (OSAS), i.e., RDI (respiratory disturbance index)>5, was found more frequently in the aRBD group (p=0.0042, Fisher's exact test). The percentages for slow wave sleep and sleep efficiency were significantly lower in the aRBD group than in the iRBD group. Conclusion: We found that 1 out of 4 RBD patients had associated CNS disorders, warranting more careful neurological evaluation and follow-up in this category of RBD. In this category of RBD patients, we also found more frequent PLMD and OSAS. These patients were also found to have lower slow wave sleep and sleep efficiency. In summary, RBD patients with associated CNS disorders suffer from more disturbed sleep than those without them.

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Voice Tremor in Parkinsonism : A Preliminary Study for Differential Diagnosis (파킨슨증의 음성진전 : 감별진단을 위한 예비연구)

  • Choi, Seong-Hee;Kim, Hyang-Hee;Lee, Won-Yong;Choi, Hong-Shik
    • Speech Sciences
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    • v.12 no.3
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    • pp.19-33
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    • 2005
  • Tremor is a main factor of parkinsonism. Voice tremor may be the first, later or the only symptom of a neurological disease and its frequency, amplitude, and regularity may differ among the diseases of different neural subsystems. Differential diagnosis between idiopathic Parkinson's disease (IPD) and multiple system atrophy (MSA) has been difficult. This study included three groups: (1) 6 IPD patients; (2) 6 MSA patients; and (3) 20 ageand sex-matched normal controls. The MDVP (Multidimensional Voice Program) was used to analyze the sustained /a/phonation. The results were as follows: (1) frequency perturbation parameters (jitter, sPPQ, Vf0) and FTRI of tremor parameter of two patient groups were statistically different from those of the controls (p < .01); (2) measures were higher in short-term and long-term f0 and amplitude perturbation in MSA than IPD; (3) however, any acoustic parameters between IPD and MSA were not statistically different; except for the rate of frequency tremor, 4$\sim$5 Hz in IPD, 5$\sim$11 Hz in MSA and (4) the pattern of regularity for voice tremor through histogram indicated that amplitude of IPD was irregular while both f0 and amplitude of MSA were irregular. In conclusion, F0, rate of frequency tremor, and pattern of f0 regularity may be predictors for differential diagnosis. These findings might signify that voice tremor of parkinsonism was resulted from modulation of f0.

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Role of post-translational modifications on the alpha-synuclein aggregation-related pathogenesis of Parkinson's disease

  • Yoo, Hajung;Lee, Jeongmin;Kim, Bokwang;Moon, Heechang;Jeong, Huisu;Lee, Kyungmi;Song, Woo Jeung;Hur, Junho K.;Oh, Yohan
    • BMB Reports
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    • v.55 no.7
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    • pp.323-335
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    • 2022
  • Together with neuronal loss, the existence of insoluble inclusions of alpha-synuclein (α-syn) in the brain is widely accepted as a hallmark of synucleinopathies including Parkinson's disease (PD), multiple system atrophy, and dementia with Lewy body. Because the α-syn aggregates are deeply involved in the pathogenesis, there have been many attempts to demonstrate the mechanism of the aggregation and its potential causative factors including post-translational modifications (PTMs). Although no concrete conclusions have been made based on the previous study results, growing evidence suggests that modifications such as phosphorylation and ubiquitination can alter α-syn characteristics to have certain effects on the aggregation process in PD; either facilitating or inhibiting fibrillization. In the present work, we reviewed studies showing the significant impacts of PTMs on α-syn aggregation. Furthermore, the PTMs modulating α-syn aggregation-induced cell death have been discussed.

A Case-Study of Taeeumin Patient with Atypical Parkinsonian Disorders Having Dizziness and Gait Disturbance (비정형파킨슨증후군 태음인의 어지럼증과 보행장애 치험례)

  • Park, Jieun;Kim, Kukhwa;Lee, Seul;Lee, Yong-jae;Lee, Jeongyun
    • Journal of Sasang Constitutional Medicine
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    • v.34 no.3
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    • pp.69-80
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    • 2022
  • A 52-year-old female diagnosed with Atypical Parkinsonian Disorders (APD) about one year ago suffered from dizziness and gait disturbance. The patient was identified as Taeeumin esophagus-cold lung-dry symptomatology pattern and was treated with Jowisengcheong-tang. The patient's symptoms of dizziness and night sweats were assessed using Global Assessment Scale (GAS). And, the Unified Multiple System Atrophy Rating Scale (UMSARS) and the Unified Parkinson's Disease Rating Scale (MDS-UPDRS) were used to assess the overall function of the patient. The patient's symptoms of dizziness and night sweats were significantly improved, and UMSARS and MDS-UPDRS scores were decreased. This case showed that Sasang constitutional medicine treatment can improve APD symptoms and improve the quality of life of the patient.

Metabolic Topography of Parkinsonism

  • Kim, Jae-Seung
    • Nuclear Medicine and Molecular Imaging
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    • v.41 no.2
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    • pp.141-151
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    • 2007
  • Parkinson's disease is one of the most frequent neurodegenerative diseases, which mainly affects the elderly. Parkinson's disease is often difficult to differentiate from atypical parkinson diorder such as progressive supranuclear palsy, multiple system atrophy, dementia with Lewy body, and corticobasal ganglionic degeneration, based on the clinical findings because of the similarity of phenotypes and lack of diagnostic markers. The accurate diagnosis of Parkinson's disease and atypical Parkinson disorders is not only important for deciding on treatment regimens and providing prognosis, but also it is critical for studies designed to investigate etiology and pathogenesis of parkinsonism and to develop new therapeutic strategies. Although degeneration of the nigrostriatal dopamine system results in marked loss of striatal dopamine content in most of the diseases causing parkinsonism, pathologic studies revealed different topographies of the neuronal cell loss in Parkisonism. Since the regional cerebral glucose metabolism is a marker of integrated local synaptic activity and as such is sensitive to both direct neuronal/synaptic damage and secondary functional disruption at synapses distant from the primary site of pathology, an assessment of the regional cerebral glucose metabolism with F-18 FDG PET is useful in the differential diagnosis of parkinsonism and evaluating the pathophysiology of parkisonism.

Evaluation of Multiple System Atrophy and Early Parkinson's Disease Using $^{123)I$-FP-CIT SPECT ($^{123)I$-FP-CIT SPECT를 이용한 다중계위축증 및 조기 파킨슨병에서의 평가)

  • Oh, So-Won;Kim, Yu-Kyeong;Lee, Byung-Chul;Kim, Bom-Sahn;Kim, Ji-Sun;Kim, Jong-Min;Kim, Sang-Eun
    • Nuclear Medicine and Molecular Imaging
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    • v.43 no.1
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    • pp.10-18
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    • 2009
  • Purpose: We investigated quantification of dopaminergic transporter (DAT) and serotonergic transporter (SERT) on $^{123}I$-FP-CIT SPECT for differentiating between multiple systemic atrophy (MSA) and idiopathic Parkinson's disease (IPD). Materials and Methods: N-fluoropropyl-$2{\beta}$-carbomethoxy-$3{\beta}$-4-[$^{123}I$]-iodophenylnortropane SPECT ($^{123}I$-FP-CIT SPECT) was performed in 8 patients with MSA (mean age: $64.0{\pm}4.5yrs$, m:f=6:2), 13 with early IPD (mean age: $65.5{\pm}5.3yrs$, m:f=9:4), and 12 healthy controls (mean age: $63.3{\pm}5.7yrs$, m:f=8:4). Standard regions of interests (ROls) of striatum to evaluate DAT, and hypothalamus and midbrain for SERT were drawn on standard template images and applied to each image taken 4 hours after radiotracer injection. Striatal specific binding for DAT and hypothalamic and midbrain specific binding for SERT were calculated using region/reference ratio based on the transient equilibrium method. Group differences were tested using ANOVA with the postHoc analysis. Results: DAT in the whole striatum and striatal subregions were significantly decreased in both patient groups with MSA and early IPD, compared with healthy control (p<0.05 in all). In early IPD, a significant increase in the uptake ratio in anterior and posterior putamen and a trend of increase in caudate to putamen ratio was observed. In MSA, the decrease of DAT was accompanied with no difference in the striatal uptake pattern compared with healthy controls. Regarding the brain regions where $^{123}I$-FP-CIT binding was predominant by SERT, MSA patients showed a decrease in the binding of $^{123}I$-FP-CIT in the pons compared with controls as well as early IPD patients (MSA: $0.22{\pm}0.1$ healthy controls: $0.33{\pm}0.19$, IPD: $0.29{\pm}0.19$), however, it did not reach the statistical significance. Conclusion: In this study, the differential patterns in the reduction of DAT in the striatum and the reduction of pontine $^{123}I$-FP-CIT binding predominant by SERT could be observed in MSA patients on $^{123}I$-FP-CIT SPECT. We suggest that the quantification of SERT as well as DAT using $^{123}I$-FP-CIT SPECT is helpful to differentiate parkinsonian disorders in early stage.