PURPOSE: Obtaining brain tissue is critical to definite diagnosis and to furthering understanding of neurodegenerative diseases. The present authors have maintained the National Neuropathology Reference and Diagnostic Laboratories for Dementia in South Korea since 2016. We have built a nationwide brain bank network and are collecting brain tissues from patients with neurodegenerative diseases. We are aiming to facilitate analyses of clinic-pathological and image-pathological correlations of neurodegenerative disease and to broaden understanding thereof. MATERIALS AND METHODS: We recruited participants through two routes: from memory clinics and the community. As a baseline evaluation, clinical interviews, a neurological examination, laboratory tests, neuropsychological tests, and MRI were undertaken. Some patients also underwent amyloid PET. RESULTS: We recruited 105 participants, 70 from clinics and 35 from the community. Among them, 11 died and were autopsied. The clinical diagnoses of the autopsied patients included four with Alzheimer's disease (AD), two with subcortical vascular dementia, two with non-fluent variant primary progressive aphasia, one with leukoencephalopathy, one with frontotemporal dementia (FTD), and one with Creutzfeldt-Jakob disease (CJD). Five patients underwent amyloid PET: two with AD, one with mixed dementia, one with FTD, and one with CJD. CONCLUSION: The clinical and neuropathological information to be obtained from this cohort in the future will provide a deeper understanding of the neuropathological mechanisms of cognitive impairment in Asia, especially Korea.
Alzheimer Disease ; Amyloid ; Aphasia, Primary Progressive ; Asia ; Brain* ; Cognition Disorders ; Cohort Studies ; Creutzfeldt-Jakob Syndrome ; Dementia ; Dementia, Vascular ; Diagnosis ; Frontotemporal Dementia ; Humans ; Korea* ; Leukoencephalopathies ; Magnetic Resonance Imaging ; Memory ; Neurodegenerative Diseases ; Neurologic Examination ; Neuropathology ; Neuropsychological Tests

Dementias can be calssified into cortical, subcortical, cortical-subcortical and multifocal ones based on the major pathological distribution within the brain. The literatures of recent knowledge about clinical features of other dementias than Alzheimer's and vascular ones, which were most frequently experienced by many clinicians were reviewed. That is, cortical dementias such as Pick's disease, frontal lobe type dementia and non-Alzheimer's type lobar atrophy including fronto-temporal dementia, progressive dysphasia, fronto-temporal dementia with motor neuron disease, and alcohol-related dementia were reviewed. Subcortical dementias such as dementias accompanying Parkinson's disease, Huntington's disease and progressive supranuclear palsy, and cortical-subcortical dementias such as Lewy body dementiaq and cortical-basal degeneration were also reviewed. As multifocal dementias, prion dementias including KUru, Creutzfeldt-Jakob disease, fatal familial insomnia and Gerstmann-Strussler-Sheinker syndrone, and AIDS dementia were also reviewed.
Aphasia ; Atrophy ; Brain ; Creutzfeldt-Jakob Syndrome ; Dementia* ; Frontal Lobe ; Frontotemporal Dementia ; Huntington Disease ; Insomnia, Fatal Familial ; Kuru ; Lewy Bodies ; Lewy Body Disease ; Motor Neuron Disease ; Parkinson Disease ; Pick Disease of the Brain ; Supranuclear Palsy, Progressive

BACKGROUND AND PURPOSE: The closing-in phenomenon is the tendency to draw near or on the target when copying figures, which has been found mostly in patients with Alzheimer's disease (AD). We attempted to quantify the degree of closing-in and to compare it between patients with AD and vascular dementia (VaD). METHODS: The subjects (55 AD, 39 VaD and 38 normal controls) were asked to copy the figure of alternating square and triangle, starting at the designated point and continuing from left to right. The patients with AD and VaD did not differ in age, education, severity of dementia or Rey Complex Figure Test copy score. The proximity (Y-axis) of the subject's drawing to the target was plotted at intervals of 2 mm along the X-axis and the degree of closing-in was computed from the slope of the regression line. RESULTS: The AD and VaD patients showed a steeper slope than the controls. There was no significant difference, however, in the magnitude of closing-in of the AD and VaD patients. When closing-in was defined as a slope that was greater than the mean+2SD of the slope observed for the controls, 32.7% of the AD and 25.6% of the VaD patients showed closing-in. CONCLUSIONS: Our study, using a new method of measuring the degree of closing-in, suggests that this phenomenon is not specific to AD.
Alzheimer Disease* ; Dementia ; Dementia, Vascular* ; Education ; Humans

Background: Antiretroviral treatment (ART) can decreased the incidence of HIV dementia, but milder cognitive impairment may not resolve when patients receive ART. In Indonesia, cognitive screening of HIV patients is not routinely performed before starting ART. Here we assess cognitive impairment in ART- naïve HIV patients beginning treatment in Jakarta. Methods: This is a cross sectional study with inclusion criteria: HIV positive, ART naïve, CD4 T-cells below 200 cells/uL, Karnofsky Performance Score (KPS) above 70. HIV-associated neurocognitive disorder (HAND) was defined by performance at least 1 Standard Deviation (SD) below the mean of demographically adjusted normative scores in at least two cognitive areas. Results: We studied 82 subjects with median (range) age 31 (19-48) years. Fifty six subjects (68%) were males. HAND was found in 42 subjects (51%). Eight subjects (19%) had impairment in 4 domains, 15 subjects (36%) in 3 and 19 (45%) in 2. The most common domain affected was memory (63%). Conclusion: Our results show the prevalence of HAND is high among HIV naïve patients in Jakarta. This establishes the need for screening of cognitive function before initiating ART.
AIDS Dementia Complex ; HIV

Differential diagnosis of Alzheimer's disease (AD) and vascular dementia(VaD) has an important bearing on the diagnosis and management of patients with dementia. This article provides a guideline for the differential diagnosis through 1) history taking, 2) neurological examination, 3) neuropsychological tests, and 4) neuroimaging studies. VaD consists of etiologically and clinically heterogeneous subtypes that include multi-infarct dementia (MID), single strategic infarct dementia, and subcortical vascular dementia. Patients with MID and single infarct dementia con be easily differentiated from patients with AD. However, clinical manifestations of subcortical vascular dementia can mimic those of AD, which may lead primary physicians to misdiagnose subcortical vascular dementia as AD. The issue of differential diagnosis is further complicated by the fact that many patients may have AD with concomitant VaD (mixed dementia).
Alzheimer Disease* ; Dementia ; Dementia, Multi-Infarct ; Dementia, Vascular* ; Diagnosis ; Diagnosis, Differential* ; Humans ; Neuroimaging ; Neurologic Examination ; Neuropsychological Tests

Psychosis in patients with dementia contributes substantially to patient morbidity and caregiver distress. The concept of psychosis of Alzheimer's disease (AD) and other types of dementia is developed with respect to prevalence, incidence, clinical characteristics, clinical course, and potential response to treatment. This article provides an overview of concept of psychosis in dementia. Published prevalence estimates of psychosis in patients with AD range from 10 to 73% within clinical populations. There is a continuing persistence of psychotic symptomatology among people with AD;most patients with psychosis continue to fulfill criteria for psychosis of dementia over at least 3 months, and over a half may have psychotic symptoms persist over a year. Among people with AD who have no psychotic symptoms there appears to be an annualized incidence of psychosis of about 20% in outpatients, and a much higher rate in nursing home patients. Frontal hypometabolism and greater frontal neuropsycological deficits occur in AD patients with psychosis in comparison to those without. There is some evidence that psychotic symptoms improve modestly with antipsychotic medication treatment, although optimal treatment guidelines have been elusive. The characteristics of psychosis in Parkinson's disease, Lewy body dementia, frontotemporal dementia and vascular dementia were also reviewed. Conclusively, further research to support the validity of a syndrome of psychosis in various types of dementia, as well as AD is needed.
Alzheimer Disease ; Caregivers ; Dementia ; Dementia, Vascular ; Frontotemporal Dementia ; Humans ; Incidence ; Lewy Body Disease ; Nursing Homes ; Outpatients ; Parkinson Disease ; Prevalence ; Psychotic Disorders

BACKGROUND: Clinicians broadly accept the Global Deterioration Scale (GDS) as a staging measure for dementia. The aim of this study is to test the inter-rater reliability and concurrent validity of the Korean version of GDS. METHODS: Participants included 34 subjects without dementia and 41 patients with Alzheimer's disease, in addition to 37 patients with vascular dementia. A rater, using a semi-structured clinical interview, interviewed the participants and their informed collateral sources. To obtain the concurrent validity, all participants received the Korean Mini-Mental State Examination (K-MMSE), the Korean version of Syndrome Kurz Test (K-SKT) and a 15-items abbreviated Korean-Boston Naming Test (K-BNT) on the same day. We also tested the inter-rater reliability among the four raters using a videotape design. RESULTS: The agreements of the overall GDS ratings by the four raters were high (kappa, 0.93-1.0). GDS correlated significantly with K-MMSE, K-SKT, and the abbreviated K-BNT and Clinical Dementia Rating scale. CONCLUSION: The Korean version of GDS may be a reliable and valid tool to be used as a staging measure for Korean patients with Alzheimer's disease as well as vascular dementia.
Alzheimer Disease ; Dementia ; Dementia, Vascular ; Humans ; Videotape Recording

BACKGROUND: The Korean-Boston Naming Test (K-BNT) is a popular measure of the confrontational naming ability. It is particularly sensitive to the early cognitive changes in Alzheimer's disease (AD) and Vascular dementia (VaD). The current study was conducted to develop parallel short forms for the K-BNT used in repeated assessments as well as in situations where the administration of the complete K-BNT is not practical. METHODS: Four 15-item short forms were newly constructed based on the item difficulties of the K-BNT measured from 565 normal elderly aged over 55. The K-BNT was administered to 130 dementia patients (75 AD, 55 VaD) and 130 healthy community-dwelling elderly whose age, sex, and education level were matched with the dementia patients. Scores for the short forms were derived by dividing up the items of the K-BNT. RESULTS: On each short form, normal elderly performed better than dementia patients, and scores on each could be extrapolated to a complete 60-item K-BNT score. Significant correlations were found between short forms and the K-BNT. To compare the probabilities of correctly identifying dementia, the areas under Receiver Operating Characteristic (ROC) curves of each form and the K-BNT were compared. It was found that all the short forms were as efficient as the 60-item K-BNT in identifying dementia. CONCLUSIONS: These results confirm that the four 15-item short forms developed in the current study are parallel and valid as the short forms of the K-BNT.
Aged ; Alzheimer Disease ; Dementia ; Dementia, Vascular ; Education ; Humans ; ROC Curve

BACKGROUND: The neuropsychiatric derangements in dementing patients are common and troublesome in their managements. The purpose of this study is to compare the behavioral changes in patients with subcortical vascular dementia (SVaD) and to those in patients with Alzheimer's disease (AD) by using the Korean version of the neuropsychiatric inventory (K-NPI). METHODS: The K-NPI was administrated to the close caregivers of 19 patients with AD (who met the criteria of the NINCDS-ADRDA for probable AD) and 14 patients with SvaD (who met the criteria of the NINDS-AIREN criteria for probable or possible VaD). Groups were matched for age, education and dementia severity. We evaluated the prevalence, the composite score (frequency X severity) of each behavioral domain in K-NPI between two groups. RESULTS: The most common behavioral disturbances were anxiety (63%) in AD and apathy/indifference (93%) in SVaD. Patients with SVaD had significantly greater total K-NPI scores than patients with AD and exhibited apathy/indifference, agitation/aggression and anxiety more frequently. Composite score of apathy/indifference over 4.7 point discriminates between AD and SVaD with accuracy of 75.8%. CONCLUSIONS: The K-NPI provides behavioral profiles that differentiate patients with SVaD from patients with AD. Patients with SVaD are more behaviorally disturbed. Clinicians need to pay more attention to the behavioral disturbances when managing the patients with SVaD.
Alzheimer Disease* ; Anxiety ; Caregivers ; Dementia ; Dementia, Vascular* ; Education ; Humans ; Prevalence

BACKGROUND: The Clinical Dementia Rating (CDR) scale is broadly accepted by clinicians as a staging measure for dementia. The aim of this study was to test inter-rater reliability and concurrent validity of the Korean version of the CDR. METHODS: Participants included 34 persons without dementia and 41 patients with Alzheimer's disease, and 37 patients with vascular dementia. The participants and their informed collateral sources were interviewed by a rater using a semistructured clinical interview. To obtain concurrent validity, all the participants received K-MMSE, K-SKT (Syndrom Kurz Test), and a 15-items abbreviated K-BNT on the same day. We also tested inter-rater reliability among four raters using a videotape design. RESULTS: Agreements of the overall CDR ratings (kappa, 0.86-1.0) and Sum of Boxes of CDR (CDR-SB) (gamma, 0.89-1.0) by the four raters were high. Both global CDR and CDR-SB correlated significantly with K-MMSE, K-SKT and the abbreviated K-BNT. The Orientation and Memory box score correlated significantly with orientation and memory scores of K-MMSE. CONCLUSIONS: The Korean version of CDR appears to be a reliable and valid tool as a staging measure for Korean dementia patients.
Alzheimer Disease ; Dementia* ; Dementia, Vascular ; Humans ; Memory ; Videotape Recording