• Investigative Magnetic Resonance Imaging
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• v.25 no.2
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• pp.101-108
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• 2021

Purpose: To identify characteristic magnetic resonance imaging (MRI) features to differentiate between Krabbe disease and metachromatic leukodystrophy (MLD) in young children. Materials and Methods: We collected all confirmed cases of Krabbe disease and MLD between October 2004 and September 2020 at Seoul National University Children's Hospital. Patients with initial MRI available were included. Their initial MRIs were retrospectively reviewed for the following: 1) presence of white matter signal abnormality involving the periventricular and deep white matter, subcortical white matter, internal capsule, brainstem, and cerebellum; 2) presence of volume decrease and signal alteration in the corpus callosum and thalamus; 3) presence of the tigroid sign; 4) presence of optic nerve hypertrophy; and 5) presence of enhancement or diffusion restriction. Results: Eleven children with Krabbe disease and 12 children with MLD were included in this study. There was no significant difference in age or symptoms at onset. Periventricular and deep white matter signal alterations sparing the subcortical white matter were present in almost all patients of the two groups. More patients with Krabbe disease had T2 hyperintensities in the internal capsule and brainstem than patients with MLDs. In contrast, more patients with MLD had T2 hyperintensities in the splenium and genu of the corpus callosum. No patient with Krabbe disease showed T2 hyperintensity in the corpus callosal genu. A decrease in volume in the corpus callosum and thalamus was more frequently observed in patients with Krabbe disease than in those with MLD. Other MRI findings including the tigroid sign and optic nerve hypertrophy were not significantly different between the two groups. Conclusion: Signal abnormalities in the internal capsule and brainstem, decreased thalamic volume, decreased splenial volume accompanied by signal changes, and absence of signal changes in the callosal genu portion were MRI findings suggestive of Krabbe disease rather than MLD based on initial MRI. Other MRI findings such as the tigroid sign could not help differentiate between these two diseases.

• The Korean Journal of Physiology and Pharmacology
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• v.12 no.3
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• pp.89-94
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• 2008

In order to reproduce chronic cerebral hypoperfusion as it occurs in human aging and Alzheimer's disease, we introduced permanent, bilateral occlusion of the common carotid arteries (BCCAO) in rats (Farkas et al, 2007). Here, we induced BCCAO in two different rat strains in order to determine whether there was a strain difference in the pathogenic response to BCCAO. Male Wistar and Sprague-Dawley (SD) rats (250-270 g) were subjected to BCCAO for three weeks. Kluver-Barrera and cresyl violet staining were used to evaluate white matter and gray matter damage, respectively. Wistar rats had a considerably higher mortality rate (four of 14 rats) as compared to SD rats (one of 15 rats) following BCCAO. Complete loss of pupillary light reflex occurred in all Wistar rats that survived, but loss of pupillary light reflex did not occur at all in SD rats. Moreover, BCCAO induced marked vacuolation in the optic tract of Wistar rats as compared to SD rats. In contrast, SD rats showed fewer CA1 hippocampal neurons than Wistar rats following BCCAO. These results suggest that the neuropathological process induced by BCCAO takes place in a region-specific pattern that varies according to the strain of rat involved.

• Korean Journal of Biological Psychiatry
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• v.3 no.2
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• pp.203-210
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• 1996

Objects : To investigate the relationship between the age of onset with the atrophy and the white matter hyperintensities observed in the brain MRI of Alzheimer patients. Methods : The authors measured volumetrically cortical and ventricular brain atrophy and rated semiquantitatively white matter signal hyperintensities in nine presenile and 18 senile Alzheimer patients, who were matched for dementia severity, according to NINCDS-ADRDA criteria and in age-matched 10 presenile and 11 senile control subjects. Results : Presenile Alzheimer patients showed significantly greater cortical and ventricular atrophy indices(p<0.05) but no difference in white matter hyperintensity scores compared to the age-matched control group. On the contrary, senile Alzheimer patients showed significantly greater white matter hyperintensity scores(p<0.05) but no difference in cortical and ventricular atrophy indices compared to the age-matched control group. Conclusion : An earlier onset was related to marked brain atrophy with less white matter lesions and a later onset is related to marked white matter lesions with less brain atrophy in Alzheimer's disease. Our results suggested the passible difference in the pathophysiology between the presenile and the senile-onset Alzheimer's disease.

• Preventive Nutrition and Food Science
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• v.19 no.3
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• pp.145-155
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• 2014

Vascular dementia is characterized by white matter lesions involving the demyelination and activation of astrocytes and microglia. In a previous study, we showed that the supernatant of a laboratory-scale, hot water extract of ground whole wheat (TALE) attenuated white matter injury and astrocytic activation in a rat model of bilateral common carotid artery occlusion (BCCAO). In the present study, we made several modifications to the hot water extraction process to remove starch and enable large-scale production. We used wheat bran (WB), which contains less starch, instead of ground whole wheat. In addition, we removed starch granules with a decanter before hot water extraction. The final product, wheat bran extract (WBE), contained 2.42% arabinose, a surrogate marker of arabinoxylan, which is an active constituent of WBE. Supplementation of the rat model of BCCAO with WBE (400 mg/kg/day) for 33 days attenuated white matter injury, which was assessed by Luxol Fast Blue staining, in the corpus callosum (cc) and optic tract (opt) regions. Attenuation of white matter injury in the opt region was accompanied by improvement of the pupillary light reflex. Immunochemical staining revealed that supplementation with WBE reduced astrocytic activation in the cc and opt regions and reduced microglial activation in the opt region. These findings indicate that supplementation with WBE is effective at attenuating white matter injury accompanied by the inhibition of astrocytic and microglial activation. Therefore, extracts from WB, a cheap by-product of wheat milling, can be developed as a nutraceutical to prevent vascular dementia, a disease for which there is no approved pharmaceutical treatment.

• Clinical and Experimental Pediatrics
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• v.46 no.1
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• pp.95-99
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• 2003

Krabbe disease is a rare autosomal recessive disorder clinically characterized by retardation in motor development, prominent spasticity, seizures, and optic atrophy. Pathologically, there are many globoid cells in the white matter, in addition to the lack of myelin and the presence of severe gliosis. Hence Krabbe disease is known as globoid cell leukodystrophy. Biochemically, the primary enzymatic deficiency in Krabbe disease is galactocerebroside beta-galactosidase. Patients with Krabbe disease can be subdivided into the early-onset type and late-onset type, according to the onset of clinical manifestations. Most patients with early-onset type die before their second birthday. We describe a girl with Krabbe disease associated with uncontrolled seizures, which was confirmed with biochemical study and MRI. The clinical findings of this patient included hyperirritability, scissoring of the legs, flexion of arm, and clenching of the fists, and generalized tonic seizures. EEG showed hypsarrhythmia, and MRI demonstrated degenerative white matter changes in bilateral periventricular white matter, posterior rim of internal capsule, basal ganglia and brain stem on T2W1 and FLAIR image. The diagnosis was based on clinical features of progressive neurologic deterioration in conjunction with low galactocerebroside beta-galactosidase activity.

• Journal of Biomedical Engineering Research
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• v.26 no.6
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• pp.383-391
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• 2005

Age-related White Matter Changes (WMC) on Magnetic Resonance Imaging (MRI) are known to appear frequently in Multiple sclerosis (MS) and Alzheimer's disease and to be related to cognitive impairment. The characterization of these WMC is very important to the study of psychology and aging. These changes consist of periventricular and subcortical types, however it is difficult to detect and segment WMC using only intensity-based methods, because their intensity, level IS similar to th~t of the gray matter (GM). In this paper, we propose a new method of segmenting periventricular WMC using K-means clustering and morphological features.

• Journal of Physiology & Pathology in Korean Medicine
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• v.18 no.2
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• pp.621-625
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• 2004

Bilateral striopallidodentate calcinosis, popularly referred to as Fahr's disease, is a disorder radiologically characterized by bilateral calcifications of the basal ganglia, thalami, dentate nuclei of the cerebellum, and the white matter of the cerebral hemisphere without serum calcium-phosphorus metabolism and related endocrinologic abnormalities. Intracranial calcifications are easily visible as high-density on CT. On magnetic resonance images, the calcifications exhibit different signal intensities. The differences in signal intensity are thought to be related to the stage of the disease, differences in calcium metabolism, and the volume of the calcium deposit. Based on literature review, I report the case of a 63 year man with bilateral symmetrical calcification in the basal ganglia, dentate nuclei of the cerebellum, and the white matter of the cerebral hemisphere who present a 5 year history of progressive dysarthria associated with left thalamic infarction.

• Dementia and Neurocognitive Disorders
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• v.17 no.3
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• pp.120-129
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• 2018

Background and Purpose: Prospective memory (PM) has a known relationship with frontal function, and PM decline has been observed in amnestic mild cognitive impairment (aMCI). Cerebral small vessel disease, as evidenced by white matter hyperintensities (WMHs), is linked to frontal dysfunction. This study was undertaken to evaluate the relationship between PM decline and WMHs in patients with aMCI. Methods: Of 74 enrollees with aMCI, 69 completed this prospective study. We compared total scores and sub-scores of the Prospective and Retrospective Memory Questionnaire (PRMQ) administered at baseline and 3 months later, stratifying patients by degree of WMHs. Results: A significant decline was seen in PRMQ total scores and PM scores at the 3-month mark in patients with moderate (vs. mild) degrees of WMHs ($-2.8{\pm}7.2$ vs. $0.2{\pm}7.1$; p=0.032). In addition, patients with moderate (vs. mild) degrees of deep WMHs (DWMHs) showed greater PM decline, whereas PM loss in patients with mild, moderate, or severe degrees of periventricular WMHs (PVWMHs) did not differ significantly. Conclusions: Findings of this study indicate that the burden of WMHs is consistently implicated in PM deterioration experienced by patients with aMCI, and signifies greater PM decline, especially in instances of extensive DWMHs. Greater attention to the change of PM is therefore needed in aMCI patients with WMHs.

• Proceedings of the IEEK Conference
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• 2000.07a
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• pp.441-445
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• 2000

This study is to segment white matter, gray matter, and cerebrospinal fluid(CSF) on a brain MR image of coronal section and to calculate the volume of each. First, we segmented the whole region of a brain from a black colored background, a skull and a fat layer. Then, we calculated the partial volume of each component, which was present in scanning finite thickness, with the arithmetical analysis of gray value from the internal region of a brain showing the blurring effects on the basis of the MR image forming principle. Calculated partial volumes of white matter, gray matter and CSF were used to determine the threshold for the segmentation of each component on a brain MR image showing the blurring effects. Finally, the volumes of segmented white matter, gray matter, and CSF were calculated. The result of this study can be used as the objective diagnostic method to determine the degree of brain atrophy of patients who have neurodegenertive diseases such as Alzheimer’s disease and cerebral palsy.

• Journal of Genetic Medicine
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• v.10 no.2
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• pp.88-93
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• 2013

Alexander disease (ALXD) is a rare demyelinating disease of the white matter of the brain that is caused by a mutation in the glial fibrillary acidic protein (GFAP) gene. The overexpression of GFAP in astrocytes induces a failure in the developmental growth of the myelin sheath. The neurodegenerative destruction of the myelin sheath of the white matter is accompanied by an accumulation of abnormal deposits of Rosenthal fibers in astrocytes, which is the hallmark of ALXD. The disease can be divided into four groups based on the onset age of the patients: neonatal, infantile, juvenile, or adult. Early-onset disease is more severe, progresses rapidly, and results in a shorter life span than late-onset cases. Magnetic resonance imaging and genetic tests are mostly used for diagnostic purposes. Pathological tests of brain tissue for Rosenthal fibers are definitive diagnostic methods. Therapeutic strategies are being investigated. Ceftriaxone, which is an enhancer of glial glutamate transporter (GLT-1) expression, is currently in clinical trials for the treatment of patients with ALXD. To date, there are no clinically available treatments. The cause, pathology, pathophysiology, inheritance, clinical features, diagnosis, and treatment of ALXD will be reviewed comprehensively.