• The Journal of Internal Korean Medicine
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• v.38 no.2
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• pp.146-151
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• 2017

The patient was a 69-year-old Korean male with Lewy's body dementia and insomnia. He was treated with acupuncture and a herbal medicine, Ukan-san. To evaluate insomnia, the patient's total sleeping time was recorded. The patient's sleeping time increased after the administration of the Korean medicine treatments. Treatment with Korean medicine may be effective in treating insomnia in cases of Lewy's body dementia.

• Journal of Korean Biological Nursing Science
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• v.22 no.3
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• pp.172-175
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• 2020

Purpose: The purpose of this paper is to provide nurses with a concise review on neurodegenrative dementias. This review includes pathophysiology, clinical course, and tips on management of dementias from Alzheimer's disease (AD), Parkinson disease (PD) and lewy body dementia (LBD). Considering increasing numbers of dementia cases among older adults, nurses who are cognizant about dementia care are instrumental in maximizing daily activities and quality of life of patients with cognitive impairment and dementia.

• The Journal of Internal Korean Medicine
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• v.42 no.5
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• pp.833-845
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• 2021

Parkinson's syndrome is a degenerative brain disease that presents characteristic motor symptoms of tremor, rigidity, and gait disturbance. In addition to these motor symptoms, Parkinson's syndrome also presents non-motor symptoms (NMSs) such as sleep disturbance and cognitive decline. NMSs reduce patient's quality of life and psychosocial functioning and cause economic burden on the patient, so appropriate evaluation and treatment are required. Lewy body dementia is one of the several diseases belonging to Parkinson's syndrome. Its symptoms such as cognitive function, memory impairment, and hallucinations occur with Parkinsonism. Although drug therapy is being used with drug treatment to treat non-motor symptoms, it has limitations such as side effects, which stimulated interest in other complementary treatment methods such as oriental medicine treatment, dance, and yoga. The patient in this case complained of tremor in the right upper extremity, muscle hypertension and pain, and persistent vision, memory, and cognitive decline. The patient was diagnosed with probable Lewy body dementia. The patient was hospitalized for 4 months and received acupuncture and herbal medicines. After treatment, the patient's NMS scale scores decreased from 90 to 63, and the Unified Parkinson's Disease Rating Scale scores (summed I, II, and III) decreased from 17 points to 8 points. The Beck Depression Inventory score decreased from 22 points to 13 points. In addition, the patient's subjective evaluation revealed improvement. In this case, a patient diagnosed with probable Lewy body dementia who did not respond to the standard treatment and did not want to take medications showed improvement in not only motor symptoms but also NMSs after integrative Korean medicine treatment.

• Dementia and Neurocognitive Disorders
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• v.23 no.1
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• pp.11-21
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• 2024

Background and Purpose: Although dementia with Lewy bodies (DLB) is the second most common cause of neurodegenerative dementia, its clinical prevalence is low. We developed a short and easy-to-complete DLB screening questionnaire (DLBSQ) to raise diagnostic sensitivity in routine clinical settings. Methods: A total of 501 participants were retrospectively enrolled, including 71 controls, 184 patients without DLB, and 246 patients with probable DLB. All patients underwent clinical evaluation, including core features of DLB, the DLBSQ, brain magnetic resonance imaging, and detailed neuropsychological assessments. The diagnostic performance of the DLBSQ for probable DLB was investigated using a receiver operating characteristic curve analysis. Results: Total DLBSQ score was associated with visuospatial and frontal/executive dysfunction and the diagnosis of probable DLB. The area under the receiver operating characteristic curve for total DLBSQ score was 0.727. Youden's method revealed an optimal cutoff value of 3. The sensitivity and specificity of the DLBSQ were 68.7% and 62.4%, respectively. Its discriminating performance improved when cognitive test profiles were additionally considered (area under the curve: 0.822, sensitivity: 80.6%, and specificity: 70.4%). Conclusions: The DLBSQ might be a useful screening tool for DLB in routine clinical practice with good sensitivity and specificity.

• Dementia and Neurocognitive Disorders
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• v.23 no.1
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• pp.54-66
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• 2024

Background and Purpose: Dementia subtypes, including Alzheimer's dementia (AD), dementia with Lewy bodies (DLB), and frontotemporal dementia (FTD), pose diagnostic challenges. This review examines the effectiveness of ¹⁸F-Fluorodeoxyglucose Positron Emission Tomography (¹⁸F-FDG PET) in differentiating these subtypes for precise treatment and management. Methods: A systematic review following Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines was conducted using databases like PubMed and Embase to identify studies on the diagnostic utility of ¹⁸F-FDG PET in dementia. The search included studies up to November 16, 2022, focusing on peer-reviewed journals and applying the goldstandard clinical diagnosis for dementia subtypes. Results: From 12,815 articles, 14 were selected for final analysis. For AD versus FTD, the sensitivity was 0.96 (95% confidence interval [CI], 0.88-0.98) and specificity was 0.84 (95% CI, 0.70-0.92). In the case of AD versus DLB, ¹⁸F-FDG PET showed a sensitivity of 0.93 (95% CI 0.88-0.98) and specificity of 0.92 (95% CI, 0.70-0.92). Lastly, when differentiating AD from non-AD dementias, the sensitivity was 0.86 (95% CI, 0.80-0.91) and the specificity was 0.88 (95% CI, 0.80-0.91). The studies mostly used case-control designs with visual and quantitative assessments. Conclusions: ¹⁸F-FDG PET exhibits high sensitivity and specificity in differentiating dementia subtypes, particularly AD, FTD, and DLB. This method, while not a standalone diagnostic tool, significantly enhances diagnostic accuracy in uncertain cases, complementing clinical assessments and structural imaging.

• Korean Journal of Radiology
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• v.24 no.3
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• pp.247-258
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• 2023

Objective: To localize the neuroanatomical substrate of rapid eye movement sleep behavior disorder (RBD) and to investigate the neuroanatomical locational relationship between RBD and α-synucleinopathy neurodegenerative diseases. Materials and Methods: Using a systematic PubMed search, we identified 19 patients with lesions in different brain regions that caused RBD. First, lesion network mapping was applied to confirm whether the lesion locations causing RBD corresponded to a common brain network. Second, the literature-based RBD lesion network map was validated using neuroimaging findings and locations of brain pathologies at post-mortem in patients with idiopathic RBD (iRBD) who were identified by independent systematic literature search using PubMed. Finally, we assessed the locational relationship between the sites of pathological alterations at the preclinical stage in α-synucleinopathy neurodegenerative diseases and the brain network for RBD. Results: The lesion network mapping showed lesions causing RBD to be localized to a common brain network defined by connectivity to the pons (including the locus coeruleus, dorsal raphe nucleus, central superior nucleus, and ventrolateral periaqueductal gray), regardless of the lesion location. The positive regions in the pons were replicated by the neuroimaging findings in an independent group of patients with iRBD and it coincided with the reported pathological alterations at post-mortem in patients with iRBD. Furthermore, all brain pathological sites at preclinical stages (Braak stages 1-2) in Parkinson's disease (PD) and at brainstem Lewy body disease in dementia with Lewy bodies (DLB) were involved in the brain network identified for RBD. Conclusion: The brain network defined by connectivity to positive pons regions might be the regulatory network loop inducing RBD in humans. In addition, our results suggested that the underlying cause of high phenoconversion rate from iRBD to neurodegenerative α-synucleinopathy might be pathological changes in the preclinical stage of α-synucleinopathy located at the regulatory network loop of RBD.

• 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.

• Journal of mucopolysaccharidosis and rare diseases
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• v.5 no.1
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• pp.1-7
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• 2021

Gaucher disease (GD, OMIM #230800 OMIM#230800) is a rare, autosomal recessive inherited metabolic disorder caused by mutation in GBA1 encoding the lysosomal enzyme, glucocerebrosidase. The deficiency of glucocerebrosidase leads to an accumulation of its substrate, glucosylceramide in macrophages of various tissues. Common clinical manifestations include cytopenia, splenomegaly, hepatomegaly, and bone lesions. The phenotype of GD is classified into three clinical categories: Type 1 (non-neuronopathic) is characterized by involvements on the viscera, whereas types 2 and 3 (neuronopathic) are associated with not only visceral symptoms but also neurological impairment, either severe in type 2 or variable in type 3. A diagnosis of GD can be confirmed by demonstrating the deficiency of acid glucocerebrosidase activity in leukocytes. Mutations in the GBA1 should be identified as they may be of prognostic value in some cases. Biomarkers including Chitotriosidase, CCL18, and glucosylsphingosine (lyso-GL1) are useful in diagnosis and treatment monitoring. Currently available disease-specific treatment in Korea consists of intravenous enzyme replacement therapy and substrate reduction therapy. For enhancing long-term prognosis, the onset of Parkinson's disease and Lewy body dementia, or the occurrence of a blood disease or cancer (hepatocellular carcinoma) should be monitored in older patients. The development of new strategies that can modify the neurological phenotype are expected, especially in Asia including Korea, where the prevalence of neuronopathic GD is relatively higher than that in western countries.

• 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.

• Nuclear Medicine and Molecular Imaging
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• v.41 no.4
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• pp.272-279
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• 2007

Purpose: The aim of this study was to evaluate the discriminating nature of $^{123}I-FP-CIT$ SPECT in patients with parkinsonism. Methods: $^{123}I-FP-CIT$ SPECT images acquired from the 18 normal controls; NC ($60.4{\pm}10.0$ yr) and 237 patients with parkinsonism ($65.9{\pm}9.2$ yr) were analyzed. From spatialIy normalized images, regional counts of the caudate, putamen, and occipital lobe were obtained using region of interest method. Binding potential (BP) was calculated with the ratio of specific to nonspecific binding activity at equilibrium. Additionally, the BP ratio of putamen to caudate (PCR) and asymmetric Index (ASI) were measured. Results: BPs of NC $3.37{\pm}0.57,\; 3.10{\pm}0.41,\; 3.23{\pm}0.48$ for caudate, putamen, whole striatum, respectively) had no significant difference with those of essential tremor; ET ($3.31{\pm}0.64,\; 3.06{\pm}0.61,\; 3.14{\pm}0.63$) and Alzheimer's disease; AD (3.33 $\pm$0.60, 3.29$\pm$0.79, 3.31$\pm$0.70), but were higher than those of Parkinson's disease; PD (1.92$\pm$0.74, 1.39$\pm$0.68, 1.64$\pm$0.68), multiple system atrophy; MSA (2.36$\pm$1.07, 2.16$\pm$0.91, 2.26$\pm$0.96), and dementia with Lewy body; DLB (1.95$\pm$0.72, 1.64$\pm$0.65, 1.79$\pm$0.66)(p<0.005). PD had statisticalIy lower values of PER and higher values of ASI than those of NC (p<0.005). And PD had significantIy lower value of PCR, higher ASI and lower BP in the putamen and whole striatum than MSA (p<0.05). Conclusion: Dopamine transporter image of $^{123}I-FP-CIT$ SPECT was a good value in differential diagnosis of parkinsonism.