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Background Non-fluent agrammatic primary progressive aphasia (naPPA) is characterized by progressive non-fluent speech disorder and might be associated with taupathy such as corticobasal degeneration (CBD) and progressive supranuclear palsy. We report a case of overlap syndrome presented with language impairment, and diagnosed as naPPA with possible CBD. Case Report A 58-year-old woman visited a memory and dementia clinic, with a 10-month history of progressive language disturbance. She was diagnosed as naPPA and overlapping CBD, based on the clinical features and neuroimaging findings including florbetaben PET. Conclusions naPPA is pathologically caused by taupathy, and might progress to asymmetrical parkinsonism and apraxia, suggestive of CBD. Overlapping clinical features in our case represent various phenotypes of taupathy.

The rapid rise in obesity-related diseases has increased interest in oral and dietary agents that disrupt fat metabolism, resulting in the excretion of dietary lipids in the feces. In this study, a rapid and convenient liquid chromatography method to comprehensively analyze fecal lipids in a single injection was developed. An evaporative light-scattering detector (ELSD) for routine analysis or atmosphere pressure chemical ionization tandem mass spectrometry [(+)APCI-MS/MS] for structural confirmation and peak purity was used. The method was applied to characterize lipid components of feces from hamsters fed high-fat diets with either 5% microcrystalline cellulose or 5% hydroxypropyl methylcellulose (HPMC) fibers, to test the effect of HPMC on lipid metabolism. HPMC is a nonfermentable, soluble cellulose fiber. The fecal lipid components identified using this method includes two secondary bile acids, deoxycholic acid, lithocholic acid, and neutral sterols including cholesterol, coprostanol, stigmastanol, and sitosterol. The profile of fecal lipid components was compared between two groups. It was found that the bile acid excretion was increased 2-fold in HPMC-fed hamsters. More interestingly, diacylglycerides and triacylglycerides were detected in feces from hamsters on HPMC-included high-fat diets. We believe that this is the first report of excretion of acylglycerides following neutral soluble fiber feeding.

Understanding how environment, crop management, and other factors, particularly soil fertility, influence the composition and quality of food crops is necessary for the production of high-quality nutritious foods. The flavonoid aglycones quercetin and kaempferol were measured in dried tomato samples (Lycopersicon esculentum L. cv. Halley 3155) that had been archived over the period from 1994 to 2004 from the Long-Term Research on Agricultural Systems project (LTRAS) at the University of California-Davis, which began in 1993. Conventional and organic processing tomato production systems are part of the set of systems compared at LTRAS. Comparisons of analyses of archived samples from conventional and organic production systems demonstrated statistically higher levels (P0.05) of quercetin and kaempferol aglycones in organic tomatoes. Ten-year mean levels of quercetin and kaempferol in organic tomatoes [115.5 and 63.3 mg g(-1) of dry matter (DM)] were 79 and 97% higher than those in conventional tomatoes (64.6 and 32.06 mg g(-1) of DM), respectively. The levels of flavonoids increased over time in samples from organic treatments, whereas the levels of flavonoids did not vary significantly in conventional treatments. This increase corresponds not only with increasing amounts of soil organic matter accumulating in organic plots but also with reduced manure application rates once soils in the organic systems had reached equilibrium levels of organic matter. Well-quantified changes in tomato nutrients over years in organic farming systems have not been reported previously.

Ocular complications of HIV-related cryptococcal meningitis are reasonably common, but complete binocular blindness as the first manifestation of HIV is extremely rare. A 58-year-old man presented with binocular blindness. He experienced blurred vision for 3 days before the blindness. Mild pleocytosis was present in the cerebrospinal fluid, from which Cryptococcus neoformans was cultured. Serology revealed positivity for HIV antibody. He was treated with antifungal and antiretroviral therapy. This case indicates that HIV-related cryptococcal meningitis should be taken into consideration when determining the cause of unexpected sudden binocular blindness. J Clin Neurol 3(4):212-214, 2007

7 pages, 7 figures, 1 table., The fruits of the date palm (Phoenix dactylifera) are consumed throughout the world and are an important part of the diet in the Middle East. Dates at the rutab and tamar maturity and ripening stages contain a wide array of phenolic antioxidants, but little is known about the composition of phenolic compounds in dates at the khalal stage of ripening. In the current study, the flavonoid glycoside and procyanidin compositions of dates of the cultivar Deglet Noor harvested at the khalal stage of maturity were characterized using liquid chromatography−electrospray ionization−tandem mass spectrometry (LC-ESI/MS/MS). Procyanidin oligomers through decamers were identified in extracts of these dates. Higher molecular weight polymers, undecamers through heptadecamers, were also apparent from mass spectra. Thirteen flavonoid glycosides of luteolin, quercetin, and apigenin, 19 when considering isomeric forms, were also identified. Mass spectra indicate that both methylated and sulfated forms of luteolin and quercetin are present as mono-, di-, and triglycosylated conjugates whereas apigenin is present as only the diglycoside. LC-ESI/MS/MS spectra indicate that quercetin and luteolin formed primarily O-glycosidic linkages whereas apigenin is present as the C-glycoside.

The cytosolic GSTs were measured directly in tissue homogenates using HPLC interfaced via ESI to a mass spectrometer (LC/MS). Total ion chromatograms were generated and filtered for ion currents corresponding to m/z ratio characteristic of individual GST isozymes. Direct LC/MS has a high degree of precision (8%) and low instrument detection limits (50-100 ng) and offers the advantage of monitoring GST expression at the protein level. In this study we describe the sub-chronic effect of feeding flavone (2500 mg/kg diet) on the expression of mGSTA3, mGSTP1, mGSTM1, and mGSTM2 in male and female mice. Additionally, we tentatively identify mGSTO and its up-regulation by flavone; a result that will facilitate the study of this novel enzyme. Flavone induced mGSTM1 and mGSTP1 in a gender and isozyme specific manner yet had no appreciable effect on the expression of mGSTA3. Male animals (day 5) displayed a 8-fold increase in mGSTM1 and a 2.6-fold increase in mGSTP1 whereas female animals displayed a 5- and 3-fold increase in mGSTM1 and mGSTP1, respectively. The mGSTM2 was detected only in flavone-fed animals, indicating an up-regulation of this isozyme by flavone. Results obtained using direct LC/MS compare favorably to the specific activity of individual isozymes (P = 0.19), and are comparable to GST levels determined using affinity chromatography followed by LC/MS (P = 0.79).

Secondary phenolic metabolites play an important role in plant defense mechanisms, and increasing evidence indicates that many are important in human health. To date, few studies have investigated the impact of various agricultural practices on levels of secondary plant metabolites. To address this issue, the total phenolic (TP) content of marionberries, strawberries, and corn grown by sustainable, organic, or conventional cultural practices were measured. Additionally, the effects of three common postharvest processing treatments (freezing, freeze-drying, and air-drying) on the TP content of these agricultural products were also investigated. Statistically higher levels of TPs were consistently found in organically and sustainably grown foods as compared to those produced by conventional agricultural practices. In all samples, freeze-drying preserved higher levels of TPs in comparison with air-drying.

The “closing-in” phenomenon is defined as a tendency to draw objects as closely as possible or overlap the figures in severe cases.1 It was first described by Mayer-Gross.2 Since then, this phenomenon has been reported in patients with various brain lesions and in normal children.3-5 It frequently occurs in patients with Alzheimer’s disease (AD) and is considered a specific neurocognitive indicator of AD.1,6 Little is known about the mechanisms by which the closingin phenomenon occurs. However, several hypotheses have been proposed to explain the pathophysiology of the closingin phenomenon. Mayer-Gross2 described the phenomenon as an aspect of constructional apraxia that reflects “the fear of empty space”. In addition, de Ajuriaguerra et al.4 described the closAnatomical Correlates of the “Closing-In” Phenomenon

Background and Purpose The positive effects of galantamine on cognition and activities of daily living (ADL) in Alzheimer's disease (AD) are thought to be mediated via improvements in attention. The purpose of this study was to determine the effect of galantamine on attention in AD patients using a computerized attention test and to elucidate the relationship between improvements in attention and change in cognition and ADL. Methods In this multicenter, open-label, prospective study, patients with mild to moderate AD received galantamine and then submitted to computerized attention tests, the Alzheimer's Disease Assessment Scale-cognitive subscale, and instrumental ADL (IADL) at baseline, 4 weeks, and 12 weeks. The differences in reaction time on computerized tests were explored relative to the changes in cognition and IADL. Results After 12 weeks of taking the trial medication there was a significant reduction from baseline levels in the choice reaction time (baseline, 5,216±3,650 sec; 12 weeks, 4,139±2,920 sec; p

Subjective memory impairment (SMI) is now increasingly recognized as a risk factor of progression to dementia. This study investigated gray and white matter changes in the brains of SMI patients compared with normal controls and mild cognitive impairment (MCI) patients. We recruited 28 normal controls, 28 subjects with SMI, and 29 patients with MCI aged 60 or older. We analyzed gray and white matter changes using a voxel-based morphometry (VBM), hippocampal volumetry and regions of interest in diffusion tensor imaging (DTI). DTI parameters of corpus callosum and cingulum in SMI showed more white matter changes compared with those in normal controls, they were similar to those in MCI except in the hippocampus, which showed more degenerations in MCI. In VBM, SMI showed atrophy in the frontal, temporal, and parietal lobes compared with normal controls although it was not as extensive as that in MCI. Patients with SMI showed gray and white matter degenerations, the changes were distinct in white matter structures. SMI might be the first presenting symptom within the Alzheimer's disease continuum when combined with additional risk factors and neurodegenerative changes.

Enantiomeric selective synthesis of (S)-2-methylbutanoic acid methyl ester, which is known as a major apple and strawberry flavor, was performed from racemic 2-methylbutanoic acid using lipases in organic solvent. Among 20 lipases, lipase IM 20 (immobilized lipase of Rhizomucor miehei), lipase AP (Aspergillus niger), and lipase FAP-15 (Aspergillus javanicus) exhibited higher enzymatic activities and enantioselectivities and were selected for the synthesis of (S)-2-methylbutanoic acid methyl ester. Using these enzymes, the reaction conditions such as temperature and lyophilizing pH were optimized, and kinetic parameters were determined. All of the reactions were performed in isooctane, which was identified as the best reaction media for nonaqueous systems. At 20 degrees C maximum enantiomeric excess was observed, while synthetic activity increased as the temperature increased. Only lipases lyophilized at pH 5.5, 6. 0, 6.5, and 7.0 showed synthetic activity. In this pH range, enantioselectivities were not influenced by the lyophilizing pH. The K(M,S) and K(M,R) values for ester synthetic activity of lipase were 1120 and 1240 mM, respectively. Enzyme activity was inhibited by (S)-2-methylbutanoic amide, and its K(i) was calculated as 84 mM. (S)-2-Methylbutanoic amide acted as a competitive inhibitor.

YongSoo Shim, M.D.*, Bora Yoon, M.D.†, Yun Jeong Hong, M.D.‡, A-Hyun Cho, M.D.*, Dong-Won Yang, M.D.* Department of Neurology*, The Catholic University of Korea, Seoul; Department of Neurology†, Konyang University College of Medicine, Daejeon; ‡Hyoja Geriatric Hospital, Yongin, Korea Background: White matter hyperintensities (WMHs) on magnetic resonance imaging (MRI) have been considered as a reliable biomarker of small vessel damages. To evaluate the severity of WMHs, it is vital to develop reliable methods to measure the volume of WMHs. We applied open source software to measure WMH volume in the semi-automated way, and tested the reliability and validity by comparing with the commonly used qualitative rating scale. Methods: Twenty five subjects with variable WMHs were recruited. ANALYZE 10.0 was used for the image processing and volumetric measurement of WMHs. The inhomogeneity and artifacts of signal were corrected with Insight Segmentation and Registration Toolkit in ANALYZE. For the gold standard of the WMH volumetric measurement, threshold method was applied with consensus of manual editing on each slice of the MRI images by two raters. Histogram of the all slices of the Fluid Attenuated Inversion Recovery (FLAIR) MRI was generated to calculate the optimal voxel intensity of threshold, and the lowest voxel threshold was decided as the mean+1.4 SD. The volumes of WMHs were generated by multiplying the area and the thickness of each slice. Interand intrarater reliability of the semi-automated volumetric and Scheltens’methods, and the association between the individual methods were analyzed. Results: The semi-automated WMH volume at the threshold of 1.4 SD as well as the gold standard volume was well correlated with the Scheltens’ visual scale (r= 0.75, p< 0.001). The semi-automated volumetry showed the excellent intra-rater (ICC= 0.9929; 95% CI, 0.98400.9968) and inter-rater reliability (ICC= 0.9830; 95% CI, 0.9620-0.9925), superior to the Scheltens’ visual rating scale. Conclusions: The semi-automated volume measurement of the WMHs with Analyze was a valid and a reliable method to quantify subcortical white matter damages of various etiologies. Received: November 15, 2012 Revision received: March 29, 2013 Accepted: March 29, 2013

Background: Cultural characteristics of taking care of patients with dementia in Korea are based on the influence of Confucianism on the lifestyle of Koreans. In addition, as the family size has been getting smaller, the number of old persons who live alone has been increasing. The purpose of this study is to examine the proportion of the elderly with dementia who live alone and demographics of caregivers when such patients live with their family. Methods: From the multiple hospital-based cohort, 973 elderly patients with very mild dementia (Clinical Dementia Rating [CDR]=0.5), 1,056 patients with mild dementia (CDR=1) and 359 patients with moderate dementia (CDR=2) were recruited. We analyzed the proportion of the patients with dementia who live alone and the demographics of the caregivers if such patients live with and are taken care of by such caregivers depending upon the severity of dementia. Results: The proportion of the patients with dementia who live alone accounts for about 40% of all elderly patients with dementia. Major age groups of caregivers are of 40s and 50s. Sons, daughters, and spouses were ranked in the said order in the proportion of caregivers according to their relationship to patients. Conclusions: Up to now, family members care for about 60% of elderly patients with dementia, which may suggest that from the objective viewpoint, direct burden borne by caregivers for old patients with dementia would be considerably high. On the other hand, about 40% of dementia patients live alone, implying that the social care system is needed for them.

한국과학기술원 의과학대학원 *효자병원, †건양대학교 의과대학 신경과학교실, ‡가톨릭대학교 의과대학 신경과학교실, §영상의학과학교실 Anatomical Correlates of Interlocking Pentagon Drawing Eek-Sung Lee, M.D., Yun Jeong Hong, M.D.*, Bora Yoon, M.D.†, Sung-Chul Lim, M.D.‡, Yong S. Shim, M.D.‡, Kook Jin Ahn, M.D.§, A-Hyun Cho, M.D.‡, Dong-Won Yang, M.D.‡ Graduate School of Medical Science and Engineering, KAIST, Daejeon; *Hyoja Geriatric Hospital, Yongin; †Department of Neurology, Konyang University College of Medicine, Daejeon; ‡Departments of Neurology, §Radiology, College of Medicine, The Catholic University of Korea, Seoul, Korea