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Sjögren’s syndrome (SS) is an autoimmune disease in which lymphocytes invade salivary and tear glands, leading to dry mouth and dry eyes. It is also associated with extraglandular manifestations. The most frequently reported neurological extraglandular manifestations are sensory polyneuropathies. Movement disorders have been reported in only 2% of primary SS patients. In this case, we present a case of primary SS initially manifesting as generalized chorea, which was initially misdiagnosed as tardive dyskinesia.
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The Korean Dementia Association (KDA) has been organizing biennial international academic conferences since 2019, with the International Conference of the KDA (IC-KDA) 2023 held in Busan under the theme ‘Beyond Boundaries: Advancing Global Dementia Solutions.’ The conference comprised 6 scientific sessions, 3 plenary lectures, and 4luncheon symposiums, drawing 804 participants from 35 countries. Notably, a Korea– Taiwan Joint Symposium addressed insights into Alzheimer’s disease (AD). Plenary lectures by renowned scholars explored topics such as microbiome-related AD pathogenesis, social cognition in neurodegenerative diseases, and genetic frontotemporal dementia (FTD). On the first day, specific presentations covered subjects like the gut–brain axis and neuroinflammation in dementia, blood-based biomarkers in AD, and updates in AD therapeutics. The second day’s presentations addressed recent issues in clinical neuropsychology, FTD cohort studies, and the pathogenesis of non-AD dementia. The Academic Committee of the KDA compiles lecture summaries to provide comprehensive understanding of the advanced dementia knowledge presented at IC-KDA 2023.
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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 18 F-Fluorodeoxyglucose Positron Emission Tomography ( 18 F-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 18 F-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@#18 F-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.
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Capecitabine, a precursor of 5-fluorouracil (5-FU), is a well-tolerated and safe chemotherapy that has been used to treat breast, pancreatic, and colorectal cancers. Albeit rare, both 5-FU and capecitbine have been reported to be associated with acute central nervous system toxicity, including cerebellar dysfunction, posterior reversible encephalopathy syndrome, and transient leukoencephalopathies. Here we present a case of 32-year-old female, demonstrating transient mutism related to capecitabine induced acute toxic leukoencephalopathy.
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no abstract available.
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Background@#and Purpose: In this study we aimed to find the association between neuropsychological performance and body mass index (BMI) in patients with mild cognitive impairment (MCI). In addition, we investigated the effects of the apolipoprotein E (APOE) genotype in the relationship between the BMI and cognition in MCI. @*Methods@#We enrolled a cohort of 3,038 subjects with MCI aged 65–90 from the Clinical Research Center for Dementia of South Korea and a dementia cohort of the Ewha Womans University Mokdong Hospital. MCI patients were classified into three subgroups according to the Asian standard of BMI. We compared cognitive performances between groups by one-way analysis of variance. To investigate the effects of the APOE genotype, we used multivariate linear regression models after adjusting for possible confounders. @*Results@#Even though normal BMI groups were younger, had more females, and had less comorbidities, the higher BMI groups had better cognitive functions. Among subjects with APOE ε4 carriers, there was a positive relationship between the BMI and the memory task alone. @*Conclusions@#Our findings suggested that higher BMI in patients with MCI were associated with better cognitive performance. The effects of the APOE ε4 genotype in the associations between BMI and cognition were distinguishing. Therefore, according to physical status, APOE ε4 genotype-specific strategies in the assessments and treatments may be necessary in elderly patients with MCI.
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Recently, aducanumab, a beta amyloid targeted immunotherapy, has been approved by the US Food and Drug Administration for the treatment of Alzheimer’s dementia (AD). Although many questions need to be answered, this approval provides a promising hope for the development of AD drugs that could be supported by new biomarkers such as blood-based ones and composite neuropsychological tests that can confirm pathologic changes in early stages of AD. It is important to elucidate the complexity of AD which is known to be associated with other factors such as vascular etiologies and neuro-inflammation. Through the second international conference of the Korean Dementia Association (KDA), researchers from all over the world have participated in the exchange of opinions with KDA members on the most up-to-date topics. The Academic Committee of the KDA summarizes lectures to provide the depth of the conference as well as discussions. This will be an important milestone to widen the latest knowledge in the research of AD’s diagnosis, therapeutics, pathogenesis that can lead to the establishment of future directions.
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Background@#and Purpose: The Korean-Color Word Stroop Test: Color Reading (K-CWST:CR) included in the Seoul Neuropsychological Screening Battery, 2nd Edition (SNSB-II) examines inhibitory control deficit. It provides normative data for both 60- and 120-second conditions, but the validity of the 60-second condition has not yet been proven. This study examined the validity of the 60-second condition by observing concordance between the performances in cognitively normal, MCI, and mild dementia groups. @*Methods@#There were 1,336 patients performed the SNSB-II, including the K-CWST: CR.Based on the cognitive test results, activities of daily living, and clinical interview, the patients were assigned to normal cognition (n=104), MCI (n=884), or mild dementia (n=348) groups. Abnormal performance on the K-CWST: CR was operationally defined as 1SD below the normative mean. The receiver operating characteristic curve analyses were conducted to compare the discriminability between the 60- and 120-second conditions. @*Results@#The percentages of abnormal performance in the MCI group were 41.5% and 42.3%, and those in the mild dementia group were 82.7% and 82.4% for the 60- and 120-second conditions, respectively. The areas under the curve for the 60- and 120-seconds were as follows; 0.80 and 0.81 in differentiating normal from MCI; 0.95 and 0.96 in normal from mild dementia; and 0.77 and 0.77 in MCI from mild dementia. @*Conclusions@#The 60-second condition of the K-CWST showed very similar results, not statistically different from the 120-second condition. Therefore, the 60-second condition could be used interchangeably with the 120-second condition in a clinical setting.
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Baclofen, a gamma-aminobutyric acid B agonist, is frequently used in the treatment of various neurologic diseases including spasticity, dystonia and trigeminal neuralgia. This drug usually acts at the presynaptic motor neuron to induce an antispastic response. Oral baclofen toxicity is extremely rare, but is often reported to affect patients with renal disease. Here, we report a patient with chronic kidney disease who was treated with relatively low doses of baclofen (5 mg twice per day) and experienced nonconvulsive status epilepticus.
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Background@#and Purpose: Rapid population aging and an increase in the demented elderly became major social concerns in South Korea. Environmental design is increasingly recognized as an important aid for long-term care of patients with dementia as well as pharmacotherapy. We did a pilot study to investigate the effect of the Seoul Dementia Healing Design Project In-House Design (S-DHDP-IHD) in improving the quality of life of the cognitively impaired patients and of the S-DHDP Environmental Design (S-DHDP-ED) in increasing daily outdoor activities for cognitively impaired individuals and not cognitively impaired (NCI) elderly residents. @*Methods@#We applied the S-DHDP-IHD to 2 households of patients with mild cognitive impairment (MCI) and early-stage vascular dementia (VD). We assessed the effectiveness of intervention by surveys and video recordings of daily tasks. Additionally, we applied the S-DHDP-ED to 5 community facilities and randomly selected 287 residents over 65 years old (32 dementia caregivers and 255 NCI elderly) to participate in surveys. @*Results@#S-DHDP-IHD intervention showed improved instrumental activities in MCI patient and early-stage VD patient. Also, the satisfaction with an intervened home environment was increased. Following S-DHDP-ED intervention, non-demented residents engaged in more outdoor and social activities. They were also satisfied with the function and design of the installed facilities. @*Conclusions@#S-DHDP encompassing both home and environmental improvements was effective in readapting cognitively impaired individuals and could achieve a customized, holistic approach to dementia caregiving by means of the improved design.
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Background@#and Purpose: Rapid population aging and an increase in the demented elderly became major social concerns in South Korea. Environmental design is increasingly recognized as an important aid for long-term care of patients with dementia as well as pharmacotherapy. We did a pilot study to investigate the effect of the Seoul Dementia Healing Design Project In-House Design (S-DHDP-IHD) in improving the quality of life of the cognitively impaired patients and of the S-DHDP Environmental Design (S-DHDP-ED) in increasing daily outdoor activities for cognitively impaired individuals and not cognitively impaired (NCI) elderly residents. @*Methods@#We applied the S-DHDP-IHD to 2 households of patients with mild cognitive impairment (MCI) and early-stage vascular dementia (VD). We assessed the effectiveness of intervention by surveys and video recordings of daily tasks. Additionally, we applied the S-DHDP-ED to 5 community facilities and randomly selected 287 residents over 65 years old (32 dementia caregivers and 255 NCI elderly) to participate in surveys. @*Results@#S-DHDP-IHD intervention showed improved instrumental activities in MCI patient and early-stage VD patient. Also, the satisfaction with an intervened home environment was increased. Following S-DHDP-ED intervention, non-demented residents engaged in more outdoor and social activities. They were also satisfied with the function and design of the installed facilities. @*Conclusions@#S-DHDP encompassing both home and environmental improvements was effective in readapting cognitively impaired individuals and could achieve a customized, holistic approach to dementia caregiving by means of the improved design.
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Background@#and Purpose: Previous studies suggest that cognitive intervention can mitigate the development of dementia in patients with mild cognitive impairment (MCI). However, the previous cognitive intervention was mostly provided as a group session, in which MCI patients sometimes had difficulty in regularly attending sessions or were reluctant to participate in group-based classes. Additionally, experienced instructors for traditional cognitive intervention may be unavailable in some chronic-care facilities or community centers. Considering these reasons, we have developed 5 programs for home-based cognitive intervention using a personal robot for MCI patients. In this preliminary study, we aimed to demonstrate the effects of our newly developed home-based cognitive intervention with robots on cognitive function in MCI patients. @*Methods@#We conducted a single-blind randomized controlled trial enrolling 46 MCI patients. Participants were randomized into 2 groups: the robot cognitive intervention (robot) (n=24) group and without cognitive intervention (control) (n=22) group. The interventions comprised 60-min sessions per day for 4 weeks. The primary outcome was the change in cognitive function measured using the Cambridge Neuropsychological Test Automated Battery. @*Results@#There were no significant baseline demographic or clinical differences between the robot and control groups. After the 4-week cognitive intervention, the robot group showed greater improvement in working memory than did the control group. @*Conclusions@#Our home-based cognitive intervention with a personal robot improved the working memory in MCI patients. Further studies with larger samples and longer study periods are required to demonstrate the effects of these programs in other cognitive domains in MCI patients.
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At the request of the authors, the NRF-support number in ‘Acknowledgements’ is modified.
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Unfortunately, the original version of this article had errors in the data displayed in flow diagram.
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BACKGROUND AND PURPOSE: Visual assessment of medial temporal-lobe atrophy (MTA) has been quick, reliable, and easy to apply in routine clinical practice. However, one of the limitations in visual assessments of MTA is the lack of widely accepted age-adjusted norms and cutoff scores for MTA for a diagnosis of Alzheimer's disease (AD). This study aimed to determine the optimal cutoff score on a T1-weighted axial MTA Visual Rating Scale (VRS) for differentiating patients with AD from cognitively normal elderly people. METHODS: The 3,430 recruited subjects comprising 1,427 with no cognitive impairment (NC) and 2003 AD patients were divided into age ranges of 50–59, 60–69, 70–79, and 80–89 years. Of these, 446 participants (218 in the NC group and 228 in the AD group) were chosen by random sampling for inclusion in this study. Each decade age group included 57 individuals, with the exception of 47 subjects being included in the 80- to 89-year NC group. The scores on the T1-weighted axial MTA VRS were graded by two neurologists. The cutoff values were evaluated from the area under the receiver operating characteristic curve. RESULTS: The optimal axial MTA VRS cutoff score from discriminating AD from NC increased with age: it was ≥as ≥1, ≥2, and ≥3 in subjects aged 50–59, 60–69, 70–79, and 80–89 years, respectively (all p < 0.001). CONCLUSIONS: These results show that the optimal cutoff score on the axial MTA VRS for diagnosing of AD differed according to the decade age group. This information could be of practical usefulness in the clinical setting.
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Anciano , Humanos , Enfermedad de Alzheimer , Atrofia , Trastornos del Conocimiento , Demencia , Diagnóstico , Corea (Geográfico) , Pemetrexed , Curva ROCRESUMEN
This study was to investigate the effects of individual and district-level socioeconomic status (SES) on the development of cognitive impairment among the elderly. A 3-year retrospective observational analysis (2010–2013) was conducted which included 136,217 community-dwelling healthy elderly who participated in the Seoul Dementia Management Project. Cognitive impairment was defined as 1.5 standard deviations below the norms on the Mini-mental status examination. In the individual lower SES group, the cumulative incidence rate (CIR) of cognitive impairment was 8.7% (95% confidence interval [CI], 8.64–8.70), whereas the CIR in the individual higher SES group was 4.1% (95% CI, 4.08–4.10). The CIR for lower district-level SES was 4.7% (95% CI, 4.52–4.86), while that in the higher district-level SES was 4.3% (95% CI, 4.06–4.44). There were no additive or synergistic effects between individual and district-level SES. From this study, the individual SES contributed 1.9 times greater to the development of cognitive impairment than the district-level SES, which suggests that individual SES disparities could be considered as one of the important factors in public health related to cognitive impairment in the elderly.
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Anciano , Humanos , Trastornos del Conocimiento , Demencia , Incidencia , Salud Pública , Estudios Retrospectivos , Seúl , Clase SocialRESUMEN
Caring for people with dementia is associated with multiple devastating social, financial, physical, and psychological challenges. There is strong evidence that multicomponent tailored caregiver intervention is effective in improving caregiver well-being and delaying institutionalization. In US, the government is actively funding for developing caregiver program and to prove its efficacy through randomized controlled trials (RCTs) to translate into practice. Even with the introduction of Korean Long-term Care Plan, still, the most of the patients with dementia are being cared by the family members. The distress of caregiving is enormous, but structured therapeutic intervention program which efficacy is proven through RCT is very insufficient in Korea. The purpose of this article is to review the caregiver intervention programs of ongoing clinical trials comparing US and Korea, and to propose a tailored, therapeutic intervention program (I-CARE; A multicenter, randomized trial to assess efficacy of therapeutic intervention programs for decreasing caregiver burden in dementia caregiver) for hospital-care in Korea.
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Humanos , Cuidadores , Demencia , Administración Financiera , Institucionalización , Corea (Geográfico) , Cuidados a Largo PlazoRESUMEN
We would like to correct the grant information.
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Oxidative stress is induced by an imbalanced redox states, involving either excessive generation of reactive oxygen species (ROS) or dysfunction of the antioxidant system. The brain is one of organs especially vulnerable to the effects of ROS because of its high oxygen demand and its abundance of peroxidation-susceptible lipid cells. Previous studies have demonstrated that oxidative stress plays a central role in a common pathophysiology of neurodegenerative diseases such as Alzheimer's disease and Parkinson's disease. Antioxidant therapy has been suggested for the prevention and treatment of neurodegenerative diseases, although the results with regard to their efficacy of treating neurodegenerative disease have been inconsistent. In this review, we will discuss the role of oxidative stress in the pathophysiology of neurodegenerative diseases and in vivo measurement of an index of damage by oxidative stress. Moreover, the present knowledge on antioxidant in the treatment of neurodegenerative diseases and future directions will be outlined.
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Enfermedad de Alzheimer , Encéfalo , Enfermedades Neurodegenerativas , Oxidación-Reducción , Estrés Oxidativo , Oxígeno , Enfermedad de Parkinson , Especies Reactivas de OxígenoRESUMEN
Foix-Chavany-Marie Syndrome (FCMS) is characterized by anarthria and bilateral facio-pharyngo-glosso-masticatory paralysis with an automatic-voluntary dissociation, which usually develops in bilateral opercular lesions. We present a case of FCMS caused by unilateral subcortical lesion. A 54-year-old man was admitted due to acute right hemiparesis with anarthria. He had voluntary facial paresis but automatic-involuntary facial movements were preserved. MRI showed an acute left corona radiata infarction and PET revealed decreased glucose metabolism in left basal ganglia and fronto-parietal lobe.