A Two-Year Observational Study to Evaluate Conversion Rates from High- and Low-Risk Patients with Amnestic Mild Cognitive Impairment to Probable Alzheimer's Disease in a Real-World Setting.

Background: Predicting conversion to probable Alzheimer&s disease (AD) from amnestic mild cognitive impairment (aMCI) is difficult but important. A nomogram was developed previously for determining the risk of 3-year probable AD conversion in aMCI.

Objective: To compare the probable AD conversion rates with cognitive and neurodegenerative changes for 2 years from high- and low risk aMCI groups classified using the nomogram.

Genetic Screening in Korean Patients with Frontotemporal Dementia Syndrome.

Background: Frontotemporal dementia (FTD) syndrome is a genetically heterogeneous group of diseases. Pathogenic variants in the chromosome 9 open reading frame 72 (), microtubule-associated protein tau (), and progranulin () genes are mainly associated with genetic FTD in Caucasian populations.

Objective: To understand the genetic background of Korean patients with FTD syndrome.

Survival in Korean Patients with Frontotemporal Dementia Syndrome: Association with Behavioral Features and Parkinsonism.

We investigated the survival time of each clinical syndrome of frontotemporal dementia (FTD) and the impacts of behavioral and motor features on survival of FTD. A total of 216 patients with FTD [82 behavioral variant FTD (bvFTD), 78 semantic variant primary progressive aphasia (svPPA), 43 non-fluent/agrammatic variant PPA (nfvPPA), 13 FTD-motor neuron disease (MND)] were enrolled from 16 centers across Korea. Behaviors and parkinsonism were assessed using the Frontal Behavioral Inventory and Unified Parkinson's Disease Rating Scale Part III, respectively.

Increased telomere length in patients with frontotemporal dementia syndrome.

Background: Telomeres are repetitive DNA sequences of TTAGGG at the ends of chromosomes. Many studies have shown that telomere shortening is associated with aging-related diseases, such as cardiovascular diseases, hypertension, diabetes, cancer, and various neurodegenerative diseases, including Alzheimer's disease, vascular dementia, Parkinson's disease, and dementia with Lewy bodies. However, changes in telomere length (TL) in patients with frontotemporal dementia (FTD) syndrome are unclear.

White matter hyperintensities in vascular contributions to cognitive impairment and dementia (VCID): Knowledge gaps and opportunities.

White matter hyperintensities (WMHs) are frequently seen on brain magnetic resonance imaging scans of older people. Usually interpreted clinically as a surrogate for cerebral small vessel disease, WMHs are associated with increased likelihood of cognitive impairment and dementia (including Alzheimer's disease [AD]). WMHs are also seen in cognitively healthy people.

Analysis of frontotemporal dementia, amyotrophic lateral sclerosis, and other dementia-related genes in 107 Korean patients with frontotemporal dementia.

To identify pathogenic variants in 107 Korean patients with sporadic frontotemporal dementia (FTD), 46 genes related to FTD, amyotrophic lateral sclerosis, and other dementias were screened by next-generation sequencing. Hexanucleotide repeats in C9orf72 gene were also tested by repeat-primed polymerase chain reaction. Next-generation sequencing revealed one known pathogenic variant (c.

Effect of rivastigmine or memantine add-on therapy is affected by butyrylcholinesterase genotype in patients with probable Alzheimer's disease.

Background: The K variant of butyrylcholinesterase (BCHE-K) exhibits a reduced acetylcholine-hydrolyzing capacity; so the clinical response to rivastigmine may differ in Alzheimer's disease (AD) patients with the BCHE-K gene.

Objective: To investigate the clinical response to rivastigmine transdermal patch monotherapy or memantine plus rivastigmine transdermal patch therapy in AD patients based on the BCHE-K gene.

Methods: A total of 146 probable AD patients consented to genetic testing for butyrylcholinesterase and underwent the final efficacy evaluations.

Clinical and Neuropsychological Characteristics of a Nationwide Hospital-Based Registry of Frontotemporal Dementia Patients in Korea: A CREDOS-FTD Study.

Background: We investigated the demographic, clinical, and neuropsychological characteristics of frontotemporal dementia (FTD) from the Clinical Research Center for Dementia of South Korea (CREDOS)-FTD registry.

Methods: A total of 200 consecutive patients with FTD recruited from 16 neurological clinics in Korea were evaluated by cognitive and functional assessments, a screening test for aphasia, behavioral questionnaires, motor assessments, and brain MRI or PET.

Results: In our registry, 78 patients were classified as having been diagnosed with behavioral-variant FTD (bvFTD), 70 with semantic dementia (SD), 33 with progressive nonfluent aphasia (PNFA), and 8 with motor neuron disease plus syndrome (MND-plus).

Clinical and genetic analysis of MAPT, GRN, and C9orf72 genes in Korean patients with frontotemporal dementia.

The hexanucleotide repeat expansion (GGGGCC) in chromosome 9 open-reading frame 72 (C9orf72) and mutations in the microtubule-associated protein tau (MAPT) and progranulin (GRN) genes are known to be associated with the main causes of familial or sporadic amyotrophic lateral sclerosis and frontotemporal dementia (FTD) in Western populations. These genetic abnormalities have rarely been studied in Asian FTD populations. We investigated the frequencies of mutations in MAPT and GRN and the C9orf72 abnormal expansion in 75 Korean FTD patients.

Bimanual-vertical hand movements.

Patients often demonstrate attentional and action-intentional biases in both the transverse and coronal planes. In addition, when making forelimb movements in the transverse plane, normal participants also have spatial and magnitude asymmetries, but forelimb spatial asymmetries have not been studied in coronal space. Thus, to learn if when normal people make vertical movements they have right-left spatial and magnitude biases, seventeen healthy, blindfolded volunteers had their hands (holding pens) placed vertically in their midsagittal plane, 10 inches apart, on pieces of paper positioned above, below, and at eye-level.

Ipsilesional 'where' with contralesional 'what' neglect.

Whereas contralesional spatial neglect is usually caused by right temporo-parietal lesions, ipsilesional spatial neglect is induced primarily by right frontal lesions. This report describes a 73-year-old woman with a right inferior parietal lesion who on 'where' tasks (line bisection and midline pointing) demonstrated ipsilesional neglect, but on 'what' tests (gap vs. no-gap detection cancellation and clothing tape removal) demonstrated contralesional neglect.

Neurologic signs in relation to cognitive function in subcortical ischemic vascular dementia: a CREDOS (Clinical Research Center for Dementia of South Korea) study.

The objective of this study was to investigate the relationship between neurologic signs and cognitive dysfunction in subcortical ischemic vascular dementia (SIVD). 121 patients with SIVD were recruited from multiple nationwide hospitals. The patients' neurologic signs were evaluated using the Focal Neurologic Sign Score (FNSS).

Line quadrisection errors in patients with hemispatial neglect.

Patients with left unilateral neglect misbisect lines toward the right. To discriminate between contralesional unawareness and ipsilesional hyperattention hypotheses for this ipsilesional bias, we performed the line quadrisection test on 18 patients with and 25 without neglect, and 24 normal controls. Overall the patients with neglect were unbiased when performing the left quadrisection task, but erred rightward on the right quadrisection task.

Action-intentional spatial bias in a patient with posterior cortical atrophy.

Patients without sensory neglect might demonstrate a spatial deviation when drawing. To better understand the mechanism of this deviation, we examined a 61-year-old woman with probable posterior cortical atrophy (PCA) who on imaging showed right hemisphere atrophy and dysfunction. When drawing a clock she correctly made a circle, but when writing the numbers deviated to the left.

A multicenter, open-label, 24-week follow-up study for efficacy on cognitive function of donepezil in Binswanger-type subcortical vascular dementia.

Objectives: To evaluate the efficacy and tolerability of donepezil in patients with Binswanger type subcortical vascular dementia.

Methods: Patients (n = 34, mean age = 71.8 + 7.

Effect of ApoE genotype on response to donepezil in patients with Alzheimer's disease.

Background/aims: The possible influence of apolipoprotein E (ApoE) genotype on the response to acetylcholinesterase inhibitor therapy in patients with Alzheimer's disease (AD) remains a matter of controversy. In order to address this issue, we investigated the effects of ApoE genotype on the clinical response to donepezil in patients with mild to moderate AD.

Methods: An open study was carried out in 51 patients with probable AD who were treated with 5-10 mg of donepezil per day for 48 weeks.

Length perception and production of normal subjects in proximal versus distal peripersonal space.

We investigated whether the perception or production of a given line length in normal subjects varies according to where in peripersonal space the line is perceived or produced. We also investigated the influence of the direction of movement used to make the line. In Experiment 1, blindfolded normal subjects were asked to estimate distances while the examiner moved the subject's hand in proximal (medial) or distal (lateral) space, moving centripetally or centrifugally.

Globus pallidus lesions associated with high mountain climbing.

Acute mountain sickness (AMS) occurs commonly in hikers who are rapidly exposed to high altitude environments. Despite the numerous reports of AMS, few studies have reported pallidal lesions associated with altitude sickness. A previously healthy 49-yr-old Korean patient, after ascent to 4,700 m, suffered symptoms consistent with AMS.

Manual approach during hand gesture imitation.

Background: Patients' tendency to draw near or into the target when copying figures, a phenomenon termed closing-in, has been previously described. That the closing-in could occur when copying hand gestures has also been noted.

Objectives: To study a patient with corticobasal degeneration to quantify his manual approach behavior and to test a possible working memory hypothesis.

Hanja alexia with agraphia after left posterior inferior temporal lobe infarction: a case study.

Korean written language is composed of ideogram (Hanja) and phonogram (Hangul), as Japanese consists of Kanji (ideogram) and Kana (phonogram). Dissociation between ideogram and phonogram impairment after brain injury has been reported in Japanese, but few in Korean. We report a 64-yr-old right-handed man who showed alexia with agraphia in Hanja but preserved Hangul reading and writing after a left posterior inferior temporal lobe infarction.