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Serial Changes in Diffusion-Weighted Magnetic Resonance Images with Hypoperfusion on Brain SPECT in a Case of Hashimoto's Encephalopathy: Understanding Pathophysiology of Hashimoto's Encephalopathy.

Sung Jae KIM ; Eun Hwan JEUNG ; Mi Kyung PARK ; Sunseob CHOI ; Kyung Won PARK

Dementia and Neurocognitive Disorders.2013;12(1):29-32. doi:10.12779/dnd.2013.12.1.29

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

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A Semi-Automated Method for Measuring White Matter Hyperintensity Volume.

Yongsoo SHIM ; Bora YOON ; Yun Jeong HONG ; A Hyun CHO ; Dong Won YANG

Dementia and Neurocognitive Disorders.2013;12(1):21-28. doi:10.12779/dnd.2013.12.1.21

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

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Neurologists' Awareness and Preparedness on Prion Diseases in Korea.

Jae Won JANG ; Young Ho PARK ; Jae Sung LIM ; Soo Chul PARK ; Hae Kwan CHEONG ; Jung E KIM ; Sangyun KIM

Dementia and Neurocognitive Disorders.2013;12(1):9-20. doi:10.12779/dnd.2013.12.1.9

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

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Preliminary Study for a Multicenter Study of Alzheimer's Disease Cerebrospinal Fluid Biomarkers.

Sun Ah PARK ; Jung Hun KIM ; Hyeong Jun KIM ; Tae Eun KIM ; Yoon Jeong KIM ; Dong Hyun LEE ; Jeong Ho PARK ; Won Seok CHAE ; Soo Jae YIM ; Sang Won SEO ; Duk L NA ; Seong Hye CHOI

Dementia and Neurocognitive Disorders.2013;12(1):1-8. doi:10.12779/dnd.2013.12.1.1

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

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Can Silent Ischemic Cerebral Lesions Affect Cognition of Parkinson's Disease Dementia?.

Il Ung KANG ; In Uk SONG ; Soo Jin LEE ; Young Do KIM ; Hyun Ji CHO ; Sung Woo CHUNG ; Youngsoon YANG

Dementia and Neurocognitive Disorders.2013;12(3):72-77. doi:10.12779/dnd.2013.12.3.72

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

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Diffusion Tensor Imaging Changes Correlate with Clinical Progression in Vascular Mild Cognitive Impairment and Vascular Dementia of Subcortical Type.

Na Young RYOO ; Byung Nam YOON ; Cindy W YOON ; Jong Hyeon AHN ; Jun Yong CHOI ; Myung Kwan LIM ; Hunki KWON ; Jun Sung PARK ; Jong Min LEE ; Seong Hye CHOI

Dementia and Neurocognitive Disorders.2013;12(3):61-71. doi:10.12779/dnd.2013.12.3.61

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

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Transient Global Amnesia Caused by Bilateral Medial Temporal-Lobe Infarction.

UnKyu YUN ; Inha HWANG ; Sang Won HA

Dementia and Neurocognitive Disorders.2017;16(4):132-133. doi:10.12779/dnd.2017.16.4.132

No abstract available.
Amnesia, Transient Global* ; Infarction*

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Posterior Type of Alzheimer's Dementia Presenting with Homonymous Hemianopsia.

YoonAh PARK ; Kun Woo PARK ; Chan Nyeong LEE

Dementia and Neurocognitive Disorders.2017;16(4):128-131. doi:10.12779/dnd.2017.16.4.128

BACKGROUND: Alzheimer's disease is a chronic neurodegenerative condition, mostly affecting the medial temporal lobe and associated neocortical structures. In this report, we present a rare clinical manifestation of this disease. CASE REPORT: A 61-year-old female with word finding difficulty and memory disturbances was diagnosed with Alzheimer's disease. Two years later, she complained of right homonymous hemianopia without optic ataxia, ocular apraxia, and simultagnosia. No findings other than parenchymal disease were apparent in magnetic resonance imaging and laboratory tests. CONCLUSIONS: In this case, in a patient initially diagnosed with Alzheimer's dementia with progressive disease, we found only homonymous hemianopia, without signs of Balint's syndrome or Gerstmann's syndrome. After careful investigation showing that Alzheimer's dementia with visual symptom was not associated with parenchymal disease, we concluded a case of atypical variant of Alzheimer's disease.
Alzheimer Disease ; Apraxias ; Ataxia ; Dementia* ; Female ; Gerstmann Syndrome ; Hemianopsia* ; Humans ; Magnetic Resonance Imaging ; Memory ; Middle Aged ; Temporal Lobe

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Neural Stem Cell Death Mechanisms Induced by Amyloid Beta.

Jongmin LEE ; Hyun Hee PARK ; Seong Ho KOH ; Hojin CHOI

Dementia and Neurocognitive Disorders.2017;16(4):121-127. doi:10.12779/dnd.2017.16.4.121

BACKGROUND AND PURPOSE: Amyloid beta (Aβ) is the main component of amyloid plaques, which are deposited in the brains of patients with Alzheimer's disease (AD). Biochemical and animal studies support the central role of Aβ in AD pathogenesis. Despite several investigations focused on the pathogenic mechanisms of Aβ, it is still unclear how Aβ accumulates in the central nervous system and subsequently initiates the disease at the cellular level. In this study, we investigated the pathogenic mechanisms of Aβ using proteomics and antibody microarrays. METHODS: To evaluate the effect of Aβ on neural stem cells (NSCs), we treated primary cultured cortical NSCs with several doses of Aβ for 48 h. A 3-[4,5-dimethylthiazol-2-yl]-2,5-diphenyltetrazolium bromide assay, trypan blue staining, and bromodeoxyuridine cell proliferation assay were performed. We detected several intracellular proteins that may be associated with Aβ by proteomics and Western blotting analysis. RESULTS: Various viability tests showed that Aβ decreased NSCs viability and cell proliferation in a concentration-dependent manner. Aβ treatment significantly decreased lactate dehydrogenase B, high-mobility group box 1, aldolase C, Ezrin, and survival signals including phosphorylated phosphoinositide 3-kinase, Akt, and glycogen synthase kinase-3β. CONCLUSIONS: These results suggest that several factors determined by proteomics and Western blot hold the clue to Aβ pathogenesis. Further studies are required to investigate the role of these factors.
Alzheimer Disease ; Amyloid* ; Animals ; Blotting, Western ; Brain ; Bromodeoxyuridine ; Cell Proliferation ; Central Nervous System ; Fructose-Bisphosphate Aldolase ; Glycogen Synthase ; Humans ; L-Lactate Dehydrogenase ; Neural Stem Cells* ; Plaque, Amyloid ; Proteomics ; Trypan Blue

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Cerebrospinal Biomarker Cut-off Methods Defined Only by Alzheimer's Disease Predict More Precisely Conversions of Mild Cognitive Impairment.

Jong Hun KIM ; Hyunsun LIM ; Jee un LEE ; Jeong Hee CHO ; Gyu Sik KIM ; Seong Hye CHOI ; Jun Hong LEE

Dementia and Neurocognitive Disorders.2017;16(4):114-120. doi:10.12779/dnd.2017.16.4.114

BACKGROUND AND PURPOSE: The cerebrospinal fluid (CSF) biomarkers play an important supportive role as diagnostic and predictive indicators of Alzheimer's disease (AD). About 30% of controls in old age show abnormal values of CSF biomarkers and display a higher risk for AD compared with those showing normal values. The cut-off values are determined by their diagnostic accuracy. However, the current cut-off values may be less accurate, because controls include high-risk groups of AD. We sought to develop models of patients with AD, who are homogenous for CSF biomarkers. METHODS: We included participants who had CSF biomarker data in the Alzheimer's Disease Neuroimaging Initiative database. We investigated the factors related to CSF biomarkers in patients with AD using linear mixed models. Using the factors, we developed models corresponding to CSF biomarkers to classify patients with mild cognitive impairment (MCI) into high risk and low risk and analyzed the conversion from MCI to AD using the Cox proportional hazards model. RESULTS: APOE ε4 status and age were significantly related to CSF Aβ1-42. CSF t-tau, APOE ε2 status and sex were significant factors. The CSF p-tau181 was associated with age and frequency of diagnosis. Accordingly, we modeled the three CSF biomarkers of AD. In MCI without APOE ε4, our models were better predictors of conversion. CONCLUSIONS: We can interpret CSF biomarkers based on the models derived from the data obtained from patients with AD.
Alzheimer Disease* ; Apolipoproteins E ; Biomarkers ; Cerebrospinal Fluid ; Diagnosis ; Humans ; Methods* ; Mild Cognitive Impairment* ; Neuroimaging ; Proportional Hazards Models ; Reference Values

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