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The"closing-in phenomenon"in figure copying tasks refers to a tendency to copy near the target, or to overlap the target to be copied. The mechanisms underlying the closing-in phenomenon have not been fully elucidated. We posit that closing-in may be related to the patients'compensatory strategies to overcome visuospatial dysfunction or visuospatial working(More)
Behavioral variant frontotemporal dementia (bvFTD) erodes complex social-emotional functions as the anterior cingulate cortex (ACC) and frontoinsula (FI) degenerate, but the early vulnerable neuron within these regions has remained uncertain. Previously, we demonstrated selective loss of ACC von Economo neurons (VENs) in bvFTD. Unlike ACC, FI contains a(More)
Patients with right hemisphere injury often omit or misread words on the left side of a page or the beginning letters of single words (neglect dyslexia). Our study involving a large sample of acute right hemisphere stroke investigated (1) the frequency of neglect dyslexia (ND), (2) the association between ND and other types of contralesional hemispatial(More)
The DNase I binding loop (residues 38-52), the hydrophobic plug (residues 262-274), and the C terminus region are among the structural elements of monomeric (G-) actin proposed to form the intermonomer interface in F-actin. To test the proximity and interactions of these elements and to provide constraints on models of F-actin structure, cysteine residues(More)
A family with paramyotonia congenita (PC) is presented. At least 10 family members were affected in an autosomal dominant inheritance pattern. The proband had cold-sensitive muscle stiffness, paradoxical myotonia, and intermittent muscle weakness since childhood. The serum level of creatine kinase was mildly elevated and short exercise test with cooling(More)
Frontotemporal lobar degeneration (FTLD) can be subdivided into frontotemporal dementia (FTD), FTD combined with motor neuron disease (FTD-MND), semantic dementia (SD), and progressive nonfluent aphasia (PNFA). FTLD has been considered a rare disorder, and its' demographic and survival data have rarely been studied in Asian population. A survival analysis(More)
BACKGROUND Highly educated participants with normal cognition show lower incidence of Alzheimer's disease (AD) than poorly educated participants, whereas longitudinal studies involving AD have reported that higher education is associated with more rapid cognitive decline. We aimed to evaluate whether highly educated amnestic mild cognitive impairment (aMCI)(More)
With rapid population aging, the socioeconomic burden caused by dementia care is snowballing. Although a few community-based studies of Alzheimer's disease (AD) have been performed in Korea, there has never been a nationwide hospital-based study thereof. We aimed to identify the demographics and clinical characteristics of mild-to-moderate AD patients from(More)
We describe detailed clinical, biochemical, neuroimaging and neuropathological features in adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP), encompassing hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS) and pigmentary orthochromatic leukodystrophy (POLD), linked to colony-stimulating factor 1 receptor(More)
The behavioral variant of frontotemporal dementia (bvFTD) slowly undermines emotion, social behavior, personal conduct, and decision making. These deficits occur in concert with focal neurodegeneration that can be quantified with modern structural and functional imaging and neuropathological methods. As a result, studies of bvFTD have helped to clarify(More)