Background: Creutzfeldt-Jakob disease (CJD) is a rare and a rapidly progressive, invariably fatal neurodegenerative disorder believed to be caused by an abnormal isoform of a cellular glycoprotein known as the prion protein. The disease develops in very different ways, such as sporadic, genetic, and iatrogenic. We aim to identify the incidence of CJD genotypes registered in the statutory infectious disease surveillance system from 2017 to 2023 and analyze the types and characteristics of domestic pathogenic CJD genotypes to serve as a basis for preventing transmission. Methods: We analyzed the characteristics of hereditary CDJ among subjects reported through the Korean Centers for Disease Control and Prevention's statutory infectious disease surveillance system from 2017 to 2023. Results: In total, six pathogenic genotypes were identified in Korean patients, with P102L belonging to Gerstmann-Sträussler-Scheinker syndrome being the most common (16, 30.2%), followed by V180I (13, 24.5%), M232R (10, 18.9%), and E200K (seven, 18.9%), which are highly prevalent in Asia, and D178N (six, 11.3%) and V203I (one, 1.9%), which belong to fatal familial insomnia. Conclusions The significance of this study lies in the identification of P102L as the predominant genotype observed among individuals in their 30s and 40s in Korea. Moreover, it highlights that the occurrence of visual symptoms at an early stage is more prevalent in this age group compared to individuals over 50. Therefore, if a young individual reports a subjective decline in visual acuity not adequately accounted for by structural abnormalities of the eye, it becomes imperative to confirm the presence of CJD before undergoing ophthalmic procedures (such as corneal or retinal surgery), as these procedures involve high-risk organs for CJD transmission.

Background: and Purpose: This clinical practice guideline provides evidence-based recommendations for treatment of dementia, focusing on cholinesterase inhibitors and N-methyl-D-aspartate (NMDA) receptor antagonists for Alzheimer’s disease (AD) and other types of dementia. Methods: Using the Population, Intervention, Comparison, Outcomes (PICO) framework, we developed key clinical questions and conducted systematic literature reviews. A multidisciplinary panel of experts, organized by the Korean Dementia Association, evaluated randomized controlled trials and observational studies. Recommendations were graded for evidence quality and strength using Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) methodology. Results: Three main recommendations are presented: (1) For AD, cholinesterase inhibitors (donepezil, rivastigmine, galantamine) are strongly recommended for improving cognition and daily function based on moderate evidence; (2) Cholinesterase inhibitors are conditionally recommended for vascular dementia and Parkinson’s disease dementia, with a strong recommendation for Lewy body dementia; (3) For moderate to severe AD, NMDA receptor antagonist (memantine) is strongly recommended, demonstrating significant cognitive and functional improvements. Both drug classes showed favorable safety profiles with manageable side effects. Conclusions This guideline offers standardized, evidence-based pharmacologic recommendations for dementia management, with specific guidance on cholinesterase inhibitors and NMDA receptor antagonists. It aims to support clinical decision-making and improve patient outcomes in dementia care. Further updates will address emerging treatments, including amyloid-targeting therapies, to reflect advances in dementia management.

Background: Creutzfeldt-Jakob disease (CJD) is a rare and a rapidly progressive, invariably fatal neurodegenerative disorder believed to be caused by an abnormal isoform of a cellular glycoprotein known as the prion protein. The disease develops in very different ways, such as sporadic, genetic, and iatrogenic. We aim to identify the incidence of CJD genotypes registered in the statutory infectious disease surveillance system from 2017 to 2023 and analyze the types and characteristics of domestic pathogenic CJD genotypes to serve as a basis for preventing transmission. Methods: We analyzed the characteristics of hereditary CDJ among subjects reported through the Korean Centers for Disease Control and Prevention's statutory infectious disease surveillance system from 2017 to 2023. Results: In total, six pathogenic genotypes were identified in Korean patients, with P102L belonging to Gerstmann-Sträussler-Scheinker syndrome being the most common (16, 30.2%), followed by V180I (13, 24.5%), M232R (10, 18.9%), and E200K (seven, 18.9%), which are highly prevalent in Asia, and D178N (six, 11.3%) and V203I (one, 1.9%), which belong to fatal familial insomnia. Conclusions The significance of this study lies in the identification of P102L as the predominant genotype observed among individuals in their 30s and 40s in Korea. Moreover, it highlights that the occurrence of visual symptoms at an early stage is more prevalent in this age group compared to individuals over 50. Therefore, if a young individual reports a subjective decline in visual acuity not adequately accounted for by structural abnormalities of the eye, it becomes imperative to confirm the presence of CJD before undergoing ophthalmic procedures (such as corneal or retinal surgery), as these procedures involve high-risk organs for CJD transmission.

Background: and Purpose: This clinical practice guideline provides evidence-based recommendations for treatment of dementia, focusing on cholinesterase inhibitors and N-methyl-D-aspartate (NMDA) receptor antagonists for Alzheimer’s disease (AD) and other types of dementia. Methods: Using the Population, Intervention, Comparison, Outcomes (PICO) framework, we developed key clinical questions and conducted systematic literature reviews. A multidisciplinary panel of experts, organized by the Korean Dementia Association, evaluated randomized controlled trials and observational studies. Recommendations were graded for evidence quality and strength using Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) methodology. Results: Three main recommendations are presented: (1) For AD, cholinesterase inhibitors (donepezil, rivastigmine, galantamine) are strongly recommended for improving cognition and daily function based on moderate evidence; (2) Cholinesterase inhibitors are conditionally recommended for vascular dementia and Parkinson’s disease dementia, with a strong recommendation for Lewy body dementia; (3) For moderate to severe AD, NMDA receptor antagonist (memantine) is strongly recommended, demonstrating significant cognitive and functional improvements. Both drug classes showed favorable safety profiles with manageable side effects. Conclusions This guideline offers standardized, evidence-based pharmacologic recommendations for dementia management, with specific guidance on cholinesterase inhibitors and NMDA receptor antagonists. It aims to support clinical decision-making and improve patient outcomes in dementia care. Further updates will address emerging treatments, including amyloid-targeting therapies, to reflect advances in dementia management.

Background: and Purpose: This clinical practice guideline provides evidence-based recommendations for treatment of dementia, focusing on cholinesterase inhibitors and N-methyl-D-aspartate (NMDA) receptor antagonists for Alzheimer’s disease (AD) and other types of dementia. Methods: Using the Population, Intervention, Comparison, Outcomes (PICO) framework, we developed key clinical questions and conducted systematic literature reviews. A multidisciplinary panel of experts, organized by the Korean Dementia Association, evaluated randomized controlled trials and observational studies. Recommendations were graded for evidence quality and strength using Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) methodology. Results: Three main recommendations are presented: (1) For AD, cholinesterase inhibitors (donepezil, rivastigmine, galantamine) are strongly recommended for improving cognition and daily function based on moderate evidence; (2) Cholinesterase inhibitors are conditionally recommended for vascular dementia and Parkinson’s disease dementia, with a strong recommendation for Lewy body dementia; (3) For moderate to severe AD, NMDA receptor antagonist (memantine) is strongly recommended, demonstrating significant cognitive and functional improvements. Both drug classes showed favorable safety profiles with manageable side effects. Conclusions This guideline offers standardized, evidence-based pharmacologic recommendations for dementia management, with specific guidance on cholinesterase inhibitors and NMDA receptor antagonists. It aims to support clinical decision-making and improve patient outcomes in dementia care. Further updates will address emerging treatments, including amyloid-targeting therapies, to reflect advances in dementia management.

Background: and Purpose: This clinical practice guideline provides evidence-based recommendations for treatment of dementia, focusing on cholinesterase inhibitors and N-methyl-D-aspartate (NMDA) receptor antagonists for Alzheimer’s disease (AD) and other types of dementia. Methods: Using the Population, Intervention, Comparison, Outcomes (PICO) framework, we developed key clinical questions and conducted systematic literature reviews. A multidisciplinary panel of experts, organized by the Korean Dementia Association, evaluated randomized controlled trials and observational studies. Recommendations were graded for evidence quality and strength using Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) methodology. Results: Three main recommendations are presented: (1) For AD, cholinesterase inhibitors (donepezil, rivastigmine, galantamine) are strongly recommended for improving cognition and daily function based on moderate evidence; (2) Cholinesterase inhibitors are conditionally recommended for vascular dementia and Parkinson’s disease dementia, with a strong recommendation for Lewy body dementia; (3) For moderate to severe AD, NMDA receptor antagonist (memantine) is strongly recommended, demonstrating significant cognitive and functional improvements. Both drug classes showed favorable safety profiles with manageable side effects. Conclusions This guideline offers standardized, evidence-based pharmacologic recommendations for dementia management, with specific guidance on cholinesterase inhibitors and NMDA receptor antagonists. It aims to support clinical decision-making and improve patient outcomes in dementia care. Further updates will address emerging treatments, including amyloid-targeting therapies, to reflect advances in dementia management.

Background: Creutzfeldt-Jakob disease (CJD) is a rare and a rapidly progressive, invariably fatal neurodegenerative disorder believed to be caused by an abnormal isoform of a cellular glycoprotein known as the prion protein. The disease develops in very different ways, such as sporadic, genetic, and iatrogenic. We aim to identify the incidence of CJD genotypes registered in the statutory infectious disease surveillance system from 2017 to 2023 and analyze the types and characteristics of domestic pathogenic CJD genotypes to serve as a basis for preventing transmission. Methods: We analyzed the characteristics of hereditary CDJ among subjects reported through the Korean Centers for Disease Control and Prevention's statutory infectious disease surveillance system from 2017 to 2023. Results: In total, six pathogenic genotypes were identified in Korean patients, with P102L belonging to Gerstmann-Sträussler-Scheinker syndrome being the most common (16, 30.2%), followed by V180I (13, 24.5%), M232R (10, 18.9%), and E200K (seven, 18.9%), which are highly prevalent in Asia, and D178N (six, 11.3%) and V203I (one, 1.9%), which belong to fatal familial insomnia. Conclusions The significance of this study lies in the identification of P102L as the predominant genotype observed among individuals in their 30s and 40s in Korea. Moreover, it highlights that the occurrence of visual symptoms at an early stage is more prevalent in this age group compared to individuals over 50. Therefore, if a young individual reports a subjective decline in visual acuity not adequately accounted for by structural abnormalities of the eye, it becomes imperative to confirm the presence of CJD before undergoing ophthalmic procedures (such as corneal or retinal surgery), as these procedures involve high-risk organs for CJD transmission.

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.

Lecanemab (product name Leqembi ® ) is an anti-amyloid monoclonal antibody treatment approved for use in Korea for patients with mild cognitive impairment (MCI) or mild dementia due to Alzheimer's disease. The Korean Dementia Association has created recommendations for the appropriate use of lecanemab to assist clinicians. These recommendations include selecting patients for administration, necessary pre-administration tests and preparations,administration methods, monitoring for amyloid related imaging abnormalities (ARIA), and communication with patients and caregivers. Lecanemab is recommended for patients with MCI or mild dementia who confirmed positive amyloid biomarkers, and should not be administered to patients with severe hypersensitivity to lecanemab or those unable to undergo magnetic resonance imaging (MRI) evaluation. To predict the risk of ARIA before administration, apolipoprotein E genotyping is conducted, and regular brain MRI evaluations are recommended to monitor for ARIA during treatment. The most common adverse reactions are infusion-related reactions, which require appropriate management upon occurrence. Additional caution is needed when co-administering with anticoagulants or tissue plasminogen activator due to the risk of macrohemorrhage. Clinicians should consider the efficacy and necessary conditions for administration, as well as the safety of lecanemab, to make a comprehensive decision regarding its use.

Lecanemab (product name Leqembi ® ) is an anti-amyloid monoclonal antibody treatment approved for use in Korea for patients with mild cognitive impairment (MCI) or mild dementia due to Alzheimer's disease. The Korean Dementia Association has created recommendations for the appropriate use of lecanemab to assist clinicians. These recommendations include selecting patients for administration, necessary pre-administration tests and preparations,administration methods, monitoring for amyloid related imaging abnormalities (ARIA), and communication with patients and caregivers. Lecanemab is recommended for patients with MCI or mild dementia who confirmed positive amyloid biomarkers, and should not be administered to patients with severe hypersensitivity to lecanemab or those unable to undergo magnetic resonance imaging (MRI) evaluation. To predict the risk of ARIA before administration, apolipoprotein E genotyping is conducted, and regular brain MRI evaluations are recommended to monitor for ARIA during treatment. The most common adverse reactions are infusion-related reactions, which require appropriate management upon occurrence. Additional caution is needed when co-administering with anticoagulants or tissue plasminogen activator due to the risk of macrohemorrhage. Clinicians should consider the efficacy and necessary conditions for administration, as well as the safety of lecanemab, to make a comprehensive decision regarding its use.