• Journal of Genetic Medicine
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• 제10권2호
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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.

• Journal of Korean Neurosurgical Society
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• 제38권1호
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• pp.23-27
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• 2005

Objective : The aim of this study is to assess the significance of an apparent diffusion coefficient[ADC] study for diagnosis of ventriculitis. Methods : Seven patients with ventriculitis were enrolled in this study. Diffusion-weighted[DW] magnetic resonance images[MRI] and ADC maps in the dependent intraventricular collection, periventricular white matter and non-dependent cerebrospinal fluid[CSF] were obtained. The images and ADC data from the different lesions were compared. Results : The DW MRI showed marked hyperintensity in the purulent pus lesion, and the corresponding ADC maps revealed prominent hypointensity and restricted ADC values compared with the non-dependent CSF and normal white matter. Conclusion : The decreased ADC value and increased signal intensity of the dependent intraventricular fluid on the DW MRI show restricted water diffusion in the purulent fluid, which is indicative of a pyogenic ventriculitis diagnosis.

• Journal of Medicine and Life Science
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• 제15권2호
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• pp.89-94
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• 2018

Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is inherited microangiopathy caused by mutations in the Notch3 gene. Typical findings from brain magnetic resonance imaging (MRI) include subcortical lacunes, extensive white matter change and cerebral microbleeds(CMBs). CMBs are indicative of bleeding-prone microangiopathy. Despite some studies investigating the association between lacunes and cognitive dysfunction in CADASIL, few studies have examined the relationship between cognitive dysfunction and CMBs. We sought to assess whether CMBs are associated with cognitive dysfunction in CADASIL. This study enrolled 83 consecutive patients with CADASIL between April 2012 and January 2014. Their degree of cognitive dysfunction was assessed by the Korean version of the CERAD neuropsychological assessment battery, digit span test, and the Stroop test. A 3.0-T MRI was used to obtain T1-weighted, fluid-attenuated inversion recovery, and susceptibility weighted images. In multiple logistic regression analysis, the grade of CMBs influenced tests of memory dysfunction (p=0.003). Three or more lacunes correlated with dysfunction in the executive domain (p=0.013) and attention domain (p=0.005). White matter hyperintensity (WMH) was an independent predictor of executive dysfunction (p=0.001). These findings suggest that in addition to lacunes, CMBs and WMHs may be useful imaging markers to associated with cognitive dysfunction in CADASIL.

• The Journal of Korean Physical Therapy
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• 제33권6호
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• pp.304-306
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• 2021

Objectives: We report on a patient who showed mild bradykinesia due to injury of the corticofugal tract (CFT) from the secondary motor area following direct head trauma, which was demonstrated on diffusion tensor tractography (DTT). Case summary: A 58-year-old male patient underwent conservative management for subarachnoid hemorrhages caused by direct head trauma resulting from a fall from six-meter height at the department of neurosurgery of a local hospital. His Glasgow Coma Scale score was 3. He developed mildly slow movements following the head trauma and visited the rehabilitation department of a university hospital at ten weeks after the fall. The patient exhibited mild bradykinesia during walking and arm movements with mild weakness in all four extremities (G/G^-). Results: On ten-week DTT, narrowing of the right CFT from the supplementary motor area (SMA-CFT), and partial tearing of the left SMA-CFT, left CFTs from the dorsal premotor cortex (dPMC-CFT) and both corticospinal tracts (CSTs) at the subcortical white matter were observed. Conclusion: This case demonstrated abnormalities in both CSTs (partial tearing at the subcortical white matter and narrowing), both SMA-CFTs (narrowing and partial tearing) and left dPMC-CFT. We believe our findings suggest the necessity of assessment of the CFTs from the secondary motor area for patients with unexplained bradykinesia following direct head trauma.

• Korean Journal of Radiology
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• 제22권7호
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• pp.1152-1162
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• 2021

Objective: This study aimed to determine whether there are regional differences in the blood-brain barrier (BBB) permeability of cognitively normal elderly participants and to identify factors influencing BBB permeability with a clinically feasible, 10-minute dynamic contrast-enhanced (DCE) MRI protocol. Materials and Methods: This IRB-approved prospective study recruited 35 cognitively normal adults (26 women; mean age, 64.5 ± 5.6 years) who underwent DCE T1-weighted imaging. Permeability maps (K_trans) were coregistered with masks to calculate the mean regional values. The paired t test and Friedman test were used to compare Ktrans between different regions. The relationships between K_trans and the factors of age, sex, education, cognition score, vascular risk burden, vascular factors on imaging, and medial temporal lobar atrophy were assessed using Pearson correlation and the Spearman rank test. Results: The mean permeability rates of the right and left hippocampi, as assessed with automatic segmentation, were 0.529 ± 0.472 and 0.585 ± 0.515 (K_trans, x 10^-3 min^-1), respectively. Concerning the deep gray matter, the K_trans of the thalamus was significantly greater than those of the putamen and hippocampus (p = 0.007, p = 0.041). Regarding the white matter, the K_trans value of the occipital white matter was significantly greater than those of the frontal, cingulate, and temporal white matter (p < 0.0001, p = 0.0007, p = 0.0002). The variations in K_trans across brain regions were not related to age, cognitive score, vascular risk burden, vascular risk factors on imaging, or medial temporal lobar atrophy in the study group. Conclusion: Our study demonstrated regional differences in BBB permeability (K_trans) in cognitively normal elderly adults using a clinically acceptable 10-minutes DCE imaging protocol. The regional differences suggest that the integrity of the BBB varies across the brains of cognitively normal elderly adults. We recommend considering regional differences in K_trans values when evaluating BBB permeability in patients with neurodegenerative diseases.

• Investigative Magnetic Resonance Imaging
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• 제16권2호
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• pp.115-123
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• 2012

목적: Tract-based spatial statistics와 화소기반 형태분석 방법을 이용하여 경도의 알츠하이머병 환자와 경도인 지장애 환자에서 확산텐서영상을 이용하여 뇌 백질의 이상을 평가하고자 하였다. 대상과 방법: 21명의 경도의 알츠하이머병 환자와 13명의 경도인지장애 환자, 그리고 16명의 건강한 노인군을 대상으로 확산텐서영상을 시행하였다. 각 참가자마다 분할 비등방도를 구하여 Tract-based spatial statistics를 이용하여 세 그룹간의 비교를 하였다. Tract-based spatial statistics 방법과의 비교를 위하여, 화소기반 형태분석 방법을 이용한 분석도 함께 시행하였다. 결과: Tract-based spatial statistics 분석결과, 경도의 알츠하이머 환자에서 건강한 노인군보다 방사관의 양측 전각과 우측 후각, 후시상방사, 우측 상세로다발, 뇌량체부, 우측 쐐기전소엽이랑에서 분할 비등방도가 유의하게 감소하였다. 화소기반 형태분석에서는 양측 갈고리다발, 좌측 부해마회의 백질, 우측 대상다발에서 추가적으로 분할 비등 방도가 감소되어 있었다. 경도의 알츠하이머 환자군과 경도인지장애 환자군의 비교, 경도인지장애 환자군과 건강한 노인군의 비교연구에서는 분할 비등방도의 유의한 차이는 없었다. 결론: Tract-based spatial statistics 분석결과, 경도의 알츠하이머 환자군에서 건강한 노인군에 비해 뇌 백질의 분할 비등방도가 여러 곳에서 감소되어 있었다. 화소기반 형태분석방법은 tract-based spatial statistics보다 더 많은 곳에서 뇌 백질의 이상을 보였다. 그러나, 인공물 또한 더 많이 관찰되었다.

• 대한영상의학회지
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• 제81권6호
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• pp.1412-1423
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• 2020

목적 본 연구는 신생아 경련 환자에서 구조적 이상이나 대사로 인한 원인이 없으면서, 확산강조영상에서 미만성 대뇌 백질의 이상을 보이는 경우의 임상적 소견과 영상 소견의 특징을 기술하고자 한다. 대상과 방법 2008년 11월에서 2017년 2월 사이에 경련에 대한 평가를 위해 56명의 생후 1주 미만의 신생아가 뇌 자기공명영상을 시행하였다. 이중 33명을 제외한 23명중, 13명에서 확산강조영상에서 미만성 대뇌 백질 이상을 보였다. 내원 시 임상 소견, 자기공명영상 소견, 추적검사 결과에 대해 후향적으로 분석하였다. 결과 확산제한을 보인 13명의 환아는 모두 만기 분만 환아였다. 대변 바이러스 검사를 시행한 10명 중 6명은 로타바이러스, 한 명은 아스트로 바이러스가 검출되었다. 확산 제한은 대뇌 백질, 시상, 중뇌에 걸쳐 다양하게 분포하였다. 결론 신생아 경련 환자에서 구조적 이상이나 대사로 인한 원인이 없으면서, 확산강조영상에서 미만성 대뇌 백질의 이상을 보이는 경우, 로타바이러스가 검출되는 경우가 흔히 있었다. 따라서 이러한 임상적, 영상의학적 소견을 보일 때, 바이러스 감염과 관련된 뇌증의 가능성을 고려할 필요가 있을 것으로 판단된다.

• Investigative Magnetic Resonance Imaging
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• 제19권1호
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• pp.10-18
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• 2015

Background and Purpose: The association between the low emotional regulation and the brain structural change of major depressive disorder (MDD) has been proposed, but the voxel-based morphometry (VBM) studies on female MDD are rare. The purpose of the present study was to show the regional volume changes of gray matter (GM) in female patients with MDD by optimized VBM. Methods: To control subjects homogeneity, twenty female MDD patients and age, sex matched 21 normal controls were included for the VBM analysis. To identify the change of regional gray matter volume (GMV), the optimized VBM was performed with T1 MRIs. The amounts of gray/white matter and intracranial cavity volumes (ICV) were measured. The analysis of covariance (ANCOVA) and partial correlation analyses covariate with age and ICV were applied for VBM. Results: The age and ICV distributions were similar between the two groups. In the ANCOVA, the total GMV of MDD was smaller than that of normal controls. In the VBM, regional GMV was relatively decreased in the limbic system (amygdalae, ambient gyri, hippocampi heads, subiculum, posterior parahippocampal gyri, pulvinar nuclei, dorsal posterior cingulate gyri, and left pregenual cingulate gyrus). The lingual gyri, short insular gyri, right fusiform gyrus, and right inferior frontal gyrus were also showed decreased regional GMV. Conclusion: The results of this study indicate that the female MDD is mainly associated with the structural deficits of the limbic system and limbic system related cortices, which were known to the center of emotions.

• Asian-Australasian Journal of Animal Sciences
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• 제21권8호
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• pp.1171-1177
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• 2008

The performance of growing rabbits fed Ruzi grass (Brachiaria ruziziensis), head lettuce (Lactuca sativa) residue, Mimosa pigra and water spinach (Ipomoea aquatica) was studied in an experiment using 64 rabbits (4 males and 4 females per treatment) of 2 breeds, New Zealand White and a crossbred between New Zealand White and native breed. The rabbits had an average initial weight of 668 g, were about 6 weeks old and were housed in individual pens. The foliages were fed ad libitum and a commercial concentrate was fed at a restricted level of 2% of body weight on a dry matter (DM) basis. In the digestibility experiment, the rabbits, 4 per foliage and males only, were fed the same foliages as in the growth experiment but without concentrate. Daily weight gain was lower in the group fed Ruzi grass, 14.8 g/d (p<0.001) compared to 17.6, 18.5 and 18.4 g/d for head lettuce, Mimosa pigra and water spinach, respectively. Feed intake and feed conversion ratio were lowest for the rabbits fed water spinach, 66 g DM/d and 3.6 kg DM/kg live weight, respectively. The New Zealand White breed had a higher daily gain than the crossbred rabbits (p<0.05), 18.0 and 16.7 g/d, respectively. There were no significant differences in feed intake, growth or feed conversion ratio due to sex. The digestibility coefficients of DM, organic matter, crude protein, crude fiber, neutral detergent fiber and acid detergent fiber were significantly lower (p<0.001) in the rabbits fed Ruzi grass. Breed and sex had no effect on digestibility. In conclusion, feeding head lettuce residue, Mimosa pigra and water spinach resulted in higher growth rate and digestibility than feeding Ruzi grass and can be recommended as alternative feeds.

• 대한의용생체공학회:의공학회지
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• 제28권3호
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• pp.317-324
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• 2007

The application of diffusion tensor imaging (DTI) and fiber tractography to Wallerian degeneration (WD) is important because this technique is a very potent tools for quantitatively evaluating fiber tracts in vivo brain. We analyzed a case and control using tracts of interest (TOI) analysis to quantify WD. We scanned a case of old infarction and an age-matched healthy volunteer. T1 magnetization prepared rapid acquisition gradient echo (MPRAGE), fluid attenuated inversion recovery (FLAIR) and 12-direction diffusion tensor imaging (DTI) were obtained and analyzed using TOI analysis. The value of mean diffusity ($D_{av}$) and fracional anisotrophy (FA) were analyzed statistically by MWU test. A p-value of less than 0.05 was considered to indicate statistical significance. A comparison of the global fiber diffusion characteristics shows WD of both the corpus callosum and the ipsilateral superior longitudinal fasciculus. The corpus callosum in particular showed trans-hemispherical degeneration. Local fiber characteristics along the geodesic paths show WD in the corpus callosum, ipsilateral superior longitudinal fasciculus, ipsilateral corticospinal tract, and ipsilateral corticothalamic tract. We have demonstrated changes in $D_{av}$ and FA values and a clear correspondence with the WD in various tracts. TOI analysis successfully revealed radial WD in white matter tracts from a region of encephalomalacia and primary gliosis, although they were only slightly affected.