Diffuse or focal white matter hyperintensity lesions on MRI have been reported in only a few patients with Hashimoto's encephalopathy (HE), and anti-TPO antibody level is high in most cases. We report a 59-year-old woman who presented with acute onset of disorientation with confusion. Anti-thyroglobulin antibody was detected in high titer, although anti-TPO antibody titer was not high. Thyroid sonography and biopsy revealed Hashimoto's thyroiditis. Initial fluid-attenuated inversion recovery (FLAIR) image and diffusion-weighted imaging (DWI) revealed ill-defined, diffuse, high-signal intensity lesions on the deep white matters and globus pallidus. Brain SPECT showed significant hypoperfusion in both basal ganglia (especially globus pallidus), frontal and temporal lobes. With the impression of HE, the patient was treated on a high-dose steroid. Over the next 15 weeks, her cognition improved to a nearly normal state and the MRI findings on DWI and FLAIR showed resolution paralleling her clinical improvement. Our case illustrates the peculiar changes in the MR findings, especially in DWI, with hypoperfusion on brain SPECT in patients with HE and allows for a greater understanding of the pathophysiology of HE.
Autoantibodies ; Basal Ganglia ; Biopsy ; Brain ; Brain Diseases ; Cognition ; Female ; Globus Pallidus ; Hashimoto Disease ; Humans ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy ; Magnetics ; Magnets ; Temporal Lobe ; Thyroid Gland ; Thyroiditis ; Tomography, Emission-Computed, Single-Photon

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. Inter- and 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, 0.9840-0.9968) 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.
Artifacts ; Consensus ; Glycosaminoglycans ; Humans ; Magnetic Resonance Imaging ; Reproducibility of Results

BACKGROUND: Creutzfeldt-Jakob disease (CJD) is very rare human prion disease. But, neurologists take a key role in diagnosis, surveillance and management of the cases because of its complexity and difficulty in diagnosis of the disease. The aim of this study is to investigate the level of awareness and preparedness of Korean neurologists on this rare disease. METHODS: Survey sheets of self-administered questionnaire were given to Korean neurologists who participated in the 31st Annual Meeting of the Koran Neurological Association. Data from 133 respondents were conducted by descriptive analysis. RESULTS: Their answers were as follows: About 62% of neurologists have experienced patients of CJD. Forty-four percent of the patients were confirmed by brain biopsy. Most of neurologists (44%) were not confident to diagnose CJD and the reason why they felt hard to diagnose was due to the variable initial clinical manifestations (45.1%) and the lack of clinical experience (51.9%). Heidenheim variant CJD, proteinase sensitive prionopathy, molecular subtypes of sporadic CJD, diagnostic criteria was not familiar term to Korean neurologists (76.7%, 53.4%, 58.6%, and 62.4% respectively). Opinion for the most useful diagnostic tool was brain MRI (45.1%), CSF 14-3-3 protein (30.1%), typical EEG finding (36.8%) and gene (PRNP) test (42.9%). And they consider none of them are specific for the diagnosis of CJD (89.5%, 73.7%, 83.5%, 91.7%, respectively). Most of the neurologist in this survey answered that the opportunity for education of CJD should be increased (67.7%). CONCLUSIONS: Most of neurologists have encountered CJD patients although it is very rare disease. Some of the important and fundamental concepts of CJD were not correctly recognized to Korean neurologists, necessitating a persistent support for updating knowledge and information.
14-3-3 Proteins ; Biopsy ; Brain ; Creutzfeldt-Jakob Syndrome ; Surveys and Questionnaires ; Electroencephalography ; Encephalopathy, Bovine Spongiform ; Humans ; Korea ; Prion Diseases ; Rare Diseases

BACKGROUND: The usefulness of cerebrospinal fluid (CSF) concentrations of amyloid beta protein 1-42 (Abeta42), phosphorylated tau (pTau) and total tau (tTau) have been increasing in Alzheimer's disease (AD). However, the direct adoption of previously reported standard values is not appropriate due to interlaboratory variability. We started this study to set up an accessible system to measure CSF biomarkers in our country with high reproducibility and validity. METHODS: Including CSFs from four different institutes the levels of Abeta42, pTau181 and tTau were measured in one lab. The intertest variability and difference in the levels of biomarkers depending on diseases were assessed. Through analysis of receiver operating characteristic cut points and binary logistic regression the cut-off values of Abeta42, pTau and tTau level were obtained, and their validity was evaluated. RESULTS: The intertest consistency was high in measuring CSF biomarkers. The value of Abeta42 was markedly decreased in AD (n= 17) and other dementia (n= 9) compared to normal control (n= 12). The levels of pTau181 and tTau were high in AD, but not in other dementia and normal control. The threshold values of Abeta42, pTau181 and tTau were 290.3 pg/mL, 54.3 pg/mL, and 320.7 pg/mL in differentiating AD from normal control showing high sensitivity and specificity. Especially, the ratios of pTau181/Abeta42 (> 0.16) and tTau/Abeta42 (> 0.76) showed the prime validity. CONCLUSIONS: Our data of CSF Abeta42, pTau181, and tTau levels were highly reproducible. PTau181/Abeta42 and tTau/Abeta42 ratios were the greatly helpful in differentiating AD from normal control.
Academies and Institutes ; Adoption ; Alzheimer Disease ; Amyloid beta-Peptides ; Biomarkers ; Dementia ; Enzyme-Linked Immunosorbent Assay ; Logistic Models ; Pyridines ; ROC Curve ; Sensitivity and Specificity ; Thiazoles

BACKGROUND: Several studies have shown that the presence of cerebrovascular lesions may play an important role for determining the severity of the clinical symptoms of dementia. But no study to date has explored the clinical effect of cerebrovascular disease in Parkinson's disease with dementia (PDD), although cerebrovascular disease is common causes of dementia in elderly population. Therefore we conducted this study to evaluate the relationship between silent cerebrovascular lesions and cognitive decline in PDD. METHODS: Only 27 patients with PDD were chosen; 17 patients had PDD with silent cerebral ischemic lesions (PDDI) and 10 patients had PDD without silent cerebral ischemic lesions (pure PDD). These subjects received the global cognitive function testing and were all evaluated with detailed neuropsychological tests including attention, memory, language, and also the visuospatial and frontal function. RESULTS: There were no significant differences between pure PDD and PDDI group on general cognitive functions tests. Regard to mean time duration of suffering from Parkinson motor symptoms and motor function scale, pure PDD group showed more long duration than PDDI group but there was no significant difference between two groups. Furthermore, there were not any significant differences between the two groups on detailed neuropsychological tests. CONCLUSIONS: We concluded that silent cerebrovascular lesions do not contribute to neuropsychological severity of PDD, although vascular disease is a common cause of cognitive impairment in the elderly. Thus the results of the present study suggest that factors other than cerebrovascular disease contribute to severity of PDD.
Aged ; Cognition ; Dementia ; Humans ; Memory ; Neuropsychological Tests ; Parkinson Disease ; Stress, Psychological ; Vascular Diseases

BACKGROUND: Cerebral small vessel disease (SVD) induces vascular cognitive impairment (VCI) such as subcortical vascular dementia (SVaD) and subcortical vascular mild cognitive impairment (svMCI). We compared MRI parameters between SVaD and svMCI and determined which MRI parameters best correlated with cognitive function and disability on cross-sectional and longitudinal analyses within them. METHODS: Twenty-four patients with SVaD and twelve with svMCI were recruited. They underwent multimodal MRIs including fluid-attenuated inversion recovery lesion load, lacunar infarct number, and fractional anisotropy (FA) and mean diffusivity (MD) from diffusion tensor imaging (DTI), neuropsychological testing, Sum of Boxes of Clinical Dementia Rating Scale (CDR-SB), Barthel Index, and the Korean version of a Geriatric Depression Scale (GDS-K). Seventeen patients were retested after 20 months for a brain MRI and clinical evaluation. RESULTS: There were significant differences in average MD and peak height of MD histograms within normal-appearing brain tissue (NABT) between SVaD and svMCI patients. In the cross-sectional analysis, average MD within NABT significantly correlated with the composite neuropsychology score (r=-0.80, p<0.001), the composite executive function score (r=-0.67, p< 0.001), and the CDR-SB (r=0.54, p=0.001), and the Barthel Index correlated with peak heights of the MD histograms (r=0.37, p=0.03) in NABT. Changes of CDR-SB was associated with changes of average MD within WMH (r=0.57, p=0.02), and changes of GDS-K was associated with changes of WMH volume (r=0.51, p=0.04) on a longitudinal scale. CONCLUSIONS: DTI parameters in NABT correlated with cognitive impairment and disability in VCI associated with SVD. Clinical progression of SVD was associated with some increment of WML volume and ultrastructural changes in WMH.
Anisotropy ; Brain ; Cerebral Small Vessel Diseases ; Cross-Sectional Studies ; Dementia ; Dementia, Vascular ; Depression ; Diffusion ; Diffusion Tensor Imaging ; Executive Function ; Glycosaminoglycans ; Humans ; Mild Cognitive Impairment ; Neuropsychological Tests ; Neuropsychology ; Stroke, Lacunar

BACKGROUND AND PURPOSE: Although acetyl-L-carnitine (ALC) treatment may have beneficial effects on Alzheimer's disease (AD), its underlying neural correlates remain unclear. The purpose of this study was to investigate cerebral perfusion changes after ALC treatment in AD patients using technetium-99m hexamethylpropylene amine oxime single photon emission computed tomography (SPECT). METHODS: A total of 18 patients with early AD were prospectively recruited and treated with ALC at 1.5 g/day for 1.4±0.3 years. At baseline and follow-up, brain SPECT, Mini-Mental State Examination (MMSE), Clinical Dementia Rating (CDR), Global Deterioration Scale (GDS), and Neuropsychiatric Inventory (NPI) were used to assess participants. After ALC administration, changes in brain perfusion, severity of dementia, cognitive performance, and neuropsychiatric disturbances were examined. RESULTS: After ALC administration, changes in scores of MMSE, CDR, GDS, and NPI were not statistically significant (p>0.05). Voxel-wise whole-brain image analysis revealed that perfusion was significantly (p<0.001) increased in the right precuneus whereas perfusion was reduced in the left inferior temporal gyrus (p<0.001), the right middle frontal gyrus (p<0.001), and the right insular cortex (p=0.001) at follow-up. CONCLUSIONS: Although previous studies have suggested that AD patients generally demonstrate progressive deterioration in brain perfusion and clinical symptoms, this study reveals that the perfusion of the precuneus is increased in AD patients after ALC administration and their cognitive and neuropsychiatric symptoms are not aggravated. Further studies are warranted to determine the potential association between perfusion increase in the precuneus and clinical symptoms after ALC treatment in AD patients.
Acetylcarnitine* ; Alzheimer Disease* ; Brain ; Cerebral Cortex ; Cognition ; Dementia ; Follow-Up Studies ; Humans ; Parietal Lobe ; Perfusion* ; Prospective Studies ; Temporal Lobe ; Tomography, Emission-Computed, Single-Photon*

BACKGROUND AND PURPOSE: Vocabulary knowledge is used as a representative index of general intelligence, and is regarded as a marker for cognitive reserve in elderly people. However, vocabulary knowledge mainly depends on formal education, hence, it may not fully represent cognitive functioning in elderly people with poor educational backgrounds. Herein, we investigated whether vocabulary knowledge is a measure of general cognitive ability among normal elderly people with few years of formal education. METHODS: The association between vocabulary knowledge and general cognitive functioning was compared between 35 elderly females with very low educational attainment and 68 elderly females with higher education. RESULTS: The vocabulary knowledge was a significant predictor of general cognitive functioning in elderly individuals with more than primary education, even after controlling the effects of age and years of education. However, it was not a significant predictor of general cognitive functioning in elderly individuals with very low educational attainment. CONCLUSIONS: Vocabulary assessment was effective in estimating general cognitive functioning in elderly individuals who received ≥6 years of education. Our findings suggested that vocabulary knowledge may not be an effective proxy of premorbid intelligence or cognitive reserve in people who have not completed elementary schooling.
Aged* ; Cognitive Aging ; Cognitive Reserve ; Education ; Female ; Humans ; Intelligence ; Proxy ; Vocabulary*

BACKGROUND AND PURPOSE: With the rapid increase in the number of elderly people in Korea, multiple socio-economic problems have emerged. In 2015, 6.4 million people accounting for about 13% of the total population in Korea were aged 65 years and over. As the elderly population continues to grow, the elderly who live alone are also increasing. They have potential risks in medical and neuropsychological aspects. The purpose of this study was to investigate the association between cognition and socio-environmental status in the elderly who live alone. METHODS: This study was conducted on 512 people who live alone (equivalent to 1% of the total elderly people) in Daejeon Metropolitan City between April and November 2015. Structured questionnaires were used to investigate the general characteristics, socio-economic status, physical status, and mood for participants. Simple tests using Mini-Mental Status Examination-Demetia Screening, Geriatric Depression Scale and Korean-instrumental activities of daily living were also performed. RESULTS: Among the 512 participants, 109 participants (21.3%) had cognitive impairment, and 128 participants (25.0%) had depression. The number of daily meals, frequency of meeting with family, and depression were independent risk factors for cognitive impairment. Factors including the duration of living alone, cognitive impairment, poor self-perceived health status, frequency of meeting with family and duration of education were considered an independent risk factor for depression. CONCLUSIONS: This study showed that the elderly who live alone are susceptible to cognitive impairment and depression, and factors including the number of daily meals, social contact, and self-perceived health status may affect cognition and depressive mood. Thus, physicians need to pay attention to management of major factors that may cause cognition impairment and depression in the elderly who live alone; in addition, they require ongoing community interest and support.
Activities of Daily Living ; Aged* ; Cognition ; Cognition Disorders* ; Depression* ; Education ; Humans ; Korea ; Mass Screening ; Meals ; Risk Factors

BACKGROUND AND PURPOSE: Cognitive training is known to be an effective tool in enhancing cognitive functioning. Research has also shown that playing video game improves certain aspects of visual attention and cognitive processing speed. The effect of computer-based cognitive training has not been demonstrated so far. This study therefore evaluated whether computer-based cognitive training improved the cognitive abilities in patients with early stage of Alzheimer's dementia. METHODS: Totally, 20 participants (early stage of Alzheimer's dementia) participated in this study. To test the effectiveness of computer-based cognitive training programs to cognition, all patients were randomly allocated to either an intervention group (n=10) or a control group (n=10). The intervention group regularly received 24 sessions of computer-based cognitive training, over a 12 week period. Neuropsychological examinations were conducted before and after this training period. RESULTS: After 12 weeks, the intervention group showed a significant change in language of Korean version of the Mini-Mental State Examination (K-MMSE), compared with the baseline cognitive examinations. Also, there was greater improvement in language, attention, calculation, verbal memory, and frontal function for the experimental group, as compared with controls. CONCLUSIONS: Computer-based cognitive training might have beneficial effects on the general cognitive functions in early stage of Alzheimer's dementia.
Cognition* ; Dementia* ; Education ; Humans ; Memory ; Video Games