• Journal of The Korean Society of Inherited Metabolic disease
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• v.20 no.1
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• pp.29-35
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• 2020

Joubert syndrome (JS) is a rare genetic disorder that is characterized by ataxia, hypotonia, developmental delay, respiratory abnormalities such as apnea-hyperpnea, and abnormal eye movements. The pathognomonic diagnostic finding is the "molar tooth sign" (MTS) on brain magnetic resonance imaging (MRI), described as cerebellar vermis hypoplasia or dysplasia, thick and horizontally oriented superior cerebellar peduncles, and an abnormally deep interpeduncular fossa. JS is characterized by genetic heterogeneity: pathogenic variants in over 30 genes have been identified to date. The CEP290 protein, which is on chromosome 12q21.3, is most frequently mutated in patients with JS, especially with renal involvement. Here, we report a case of JS in a 14-year-old male patient with end-stage renal disease. To the best of our knowledge, this is the first Korean report of a patient with JS due to CEP290 mutation (c.6012-12T> A) whose diagnosis was confirmed after repetitive MRI. We suggest consultation with an experienced neuro-radiologist and follow-up MRI studies to detect a "hidden" MTS if clinical findings suggest a diagnosis of JS. Furthermore, even in the absence of an MTS, whole exome sequencing should be considered.

• Science of Emotion and Sensibility
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• v.12 no.4
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• pp.425-432
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• 2009

The purposes of the study were to examine cerebellar areas and lateralization responsible for visuospatial and verbal tasks using functional Magnetic Resonance Imaging(fMRI). Eight healthy male college students($21.5\;{\pm}\;2.3$ years) and eight male college students($23.3\;{\pm}\;0.5$ years) participated in this fMRI study of visuospatial and verbal tasks, respectively. Functional brain images were taken from 3T MRI using the single-shot EPI method. All functional images were aligned with anatomical images using affine transformation routines built into SPM99. The experiment consisted of four blocks. Each block included a control task(1 minute) and a cognitive task(1 minute). A run was 8 minutes long. Using the subtraction procedure, activated areas in the cerebellum during the visuospatial and verbal tasks were color-coded by t-score. A cerebellar lateralization index was calculated for both cognition tasks using number of activated voxels. The activated cerebellar regions during the both cognition tasks of this study agree with previous results. Since the number of activated voxels of the left and right cerebellar hemisphere was almost same, there was no cerebellar lateralization for both cognition tasks.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.74-78
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• 2019

Atypical teratoid rhabdoidal tumor(AT/RT) is a rare and fast-growing tumor usually diagnosed in childhood. Routine magnetic resonance imaging(MRI) assessment of AT/RT patients is done to detect metastatic tumors and recurrent lesions. The purpose of this case report is to present a case about caries treatment of a 3-year-8-month old female patient with AT/RT under general anesthesia. A 3-year-8-month old patient with AT/RT visited Seoul National University Dental Hospital for caries treatment. At the first dental visit, she was receiving chemotherapy. Multiple caries were observed from clinical and radiographic examination. The dental procedure was successfully performed under general anesthesia. Patients with brain tumor may take routine MRI to detect metastatic tumors and recurrent lesions. Dentists should take into consideration of possible dislodgement of restoration by ferromagnetic properties and artifact formation on MRI image. Ready-made zirconia crowns can be a good restoration option for such patients.

• The Korean Journal of Nuclear Medicine
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• v.34 no.6
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• pp.445-455
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• 2000

Purpose: We evaluated the distribution of hypoperfusion in patients with traumatic brain injury (TBI) and the relationship of thalamic hypoperfusion to severity of cognitive and behavioral sequelae. Materials and Methods: Tc-99m ECD SPECT and MRI were performed in 103 patients (M/F=81/22, mean age $34.7{\pm}15.4$ yrs) from 0.5 to 55 months (mean 10.3 months) after TBI. The patients were divided into three groups showing no abnormalities (G1), focal (G2) and diffuse injury (G3) on MRI. Psychometric tests assessed 11 cognitive or behavioral items. In all patients, we evaluated the distribution of hypoperfused areas in SPECT, and in 57/103 patients, neuropsychological (NP) abnormalities in patients with thalamic hypoperfusion were compared with those of patients without thalamic hypoperfusion. Results: The perfusion deficits were most frequently located in the frontal lobe (G1, 42.3%: G2 34.5%: G3 33.3%), temporal lobe ($24{\sim}26%$) thalami ($21{\sim}22.4%$), parietal and occipital lobe (${\leq}10%$). Numbers of NP abnormalities in the cases of cortical hypoperfusion with or without concomitant thalamic hypoperfusion were following: the former $4.7{\pm}1.5$ and the latter $3.2{\pm}1.4\;in\;G1,\;5.0{\pm}1.1\;and\;4.8{\pm}1.2\;in\;G2,\;6.8{\pm}1.8\;and\;6.3{\pm}1.1\;in\;G3$, respectively. This difference according to thalamic hypoperfusion was significant in G1 (p=0.002), but was not significant in G2 or G3. Conclusion: SPECT in patients with TBI had demonstrated hypoperfusion mostly involving the frontal, temporal and thalami. In normal group on MRI, frontal hypoperfusion was more prominent than that of any other group, Furthermore in this group, SPECT could predict severity of NP outcome by concomitant thalamic hypoperfusion with cerebral cortical abnormalities.

• Investigative Magnetic Resonance Imaging
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• v.15 no.2
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• pp.130-138
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• 2011

Purpose : To compare fMRIs of visual and auditory word generation tasks, and to evaluate the difference of its activated areas and lateralization according to the mode of stimuli. Materials and Methods : Eight male normal volunteers were included and all were right handed. Functional maps were obtained during auditory and visual word generation tasks in all. Normalized group analysis were performed in each task and the threshold for significance was set at p<0.05. Activated areas in each task were compared visually and statistically. Results : In both tasks, left dominant activations were demonstrated and were more lateralized in visual task. Both frontal lobes (Broca's area, premotor area, and SMA) and left posterior middle temporal gyrus were activated in both tasks. Extensive bilateral temporal activations were noted in auditory task. Both occipital and parietal activations were demonstrated in visual task. Conclusion : Modality independent areas could be interpreted as a core area of language function. Modality specific areas may be associated with processing of stimuli. Visual task induced more lateralized activation and could be a more useful in language study than auditory task.

• Journal of the Korean Society of Radiology
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• v.82 no.5
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• pp.1124-1139
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• 2021

The objective assessment of atrophy and the measurement of brain volume is important in the early diagnosis of dementia and neurodegenerative diseases. Recently, several MR-based volumetry software have been developed. For their clinical application, several issues arise, including the standardization of image acquisition and their validation of software. Additionally, it is important to highlight the diagnostic performance of the volumetry software based on expert opinions. We instituted a task force within the Korean Society of Neuroradiology to develop guidelines for the clinical use of MR-based brain volumetry software. In this review, we introduce the commercially available software and compare their diagnostic performances. We suggest the need for a standard protocol for image acquisition, the validation of the software, and evaluations of the limitations of the software related to clinical practice. We present recommendations for the clinical applications of commercially available software for volumetry based on the expert opinions of the Korean Society of Neuroradiology.

• Korean Journal of Radiology
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• v.2 no.2
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• pp.63-67
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• 2001

Objective: Atrophy and a high T2 signal of the hippocampus are known to be the principal MR imaging findings of hippocampal sclerosis. The purpose of this study was to determine whether or not individual MRI findings correlate with surgical outcome in patients with this condition. Materials and Methods: Preoperative MR imaging findings in 57 consecutive patients with pathologically-proven hippocampal sclerosis who underwent anterior temporal lobectomy and were followed-up for 24 months or more were retrospectively reviewed, and the results were compared with the postsurgical outcome (Engel classification). The MR images included routine sagittal T1-weighted and axial T2-weighted spin-echo images, and oblique coronal T1-weighted 3D gradient-echo and T2-weighted 2D fast spin-echo images obtained on either a 1.5 T or 1.0 T unit. The images were visually evaluated by two neuroradiologists blinded to the outcome; their focus was the presence or absence of atrophy and a high T2 hippocampal signal. Results: Hippocampal atrophy was seen in 96% of cases (55/57) [100% (53/53) of the good outcome group (Engel class I and II), and 50% (2/4) of the poor outcome group (class III and IV)]. A high T2 hippocampal signal was seen in 61% of cases (35/57) [62% (33/53) of the good outcome group and 50% (2/4) of the poor outcome group]. All 35 patients with a high T2 signal had hippocampal atrophy. 'Normal' hippocampus, as revealed by MR imaging, occurred in 4% of patients (2/57), both of whom showed a poor outcome (Engel class III). The presence or absence of hippocampal atrophy correlated well with surgical outcome (p<0.01). High T2 signal intensity did not, however, significantly correlate with surgical outcome (p>0.05). Conclusion: Compared with a high T2 hippocampal signal, hippocampal atrophy is more common and correlates better with surgical outcome. For the prediction of this, it thus appears to be the more useful indicator.

• Proceedings of the KSMRM Conference
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• 2001.11a
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• pp.137-137
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• 2001

Purpose： The purpose of this study was to identify cerebral cortices related with sexual arousal fro visual sexual stimulation in healthy males using BOLD-based functional MR imaging Method： Sixteen male volunteers with sexually potent(mean age：24) were examined for thi study. Functional MRI was performed on a 1.5T MR scanner(GE Signa Horizon) with birdcage-type head coil. In this study, blood oxygenation level dependent(BOLD) technique was utilized to create fMR image reflecting local brain activities. The BOLD-based fMRI d were obtained from 7 oblique planes using gradient-echo EPI with $90^{\circ}$flip angle, 50ms TE 6000ms TR, $26cm{\times}26$ cm FOV, $128{\times}128$ matrix, and 10mm slice thickness. The sexual stimulation paradigm consisted of two alternating periods of rest and activati and it began with a 1 minute rest, followed by a 2 minute stimulation by a documentary a erotic video film. Brain activation maps were generated by cross-correlation of imag acquired during rest and activation periods. The index of activation was used to compare t number of pixels activated by each task in each volunteer, where the significance of th differences was evaluated by using Students t-test.

• Investigative Magnetic Resonance Imaging
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• v.15 no.1
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• pp.67-71
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• 2011

Purpose : To compare T2 relaxation times (T2) in the cingulate cortex, amygdaloid body, hippocampal body, and insular cortex between 1.5T and 3.0T MR imagers. Materials and Methods : Twelve healthy volunteers underwent FLAIR and CPMG imaging perpendicular to the hippocampal body at both 3.0T and 1.5T. T2 was measured in the cingulate cortex, amygdaloid body, hippocampal body, and insular cortex. The T2 relaxation time ratios of the cingulate cortex, insular cortex, and amygdaloid body to the hippocampal body were compared between 1.5T and 3.0T. Results : The mean T2 of the cingulate cortex, amygdaloid body, hippocampal body, and insular cortex at 1.5T were $109.5{\pm}3.1$, $117.0{\pm}7.1$, $114.7{\pm}2.4$, and $111.3{\pm}2.4$, respectively; $99.7{\pm}3.8$, $100.7{\pm}4.3$, $97.9{\pm}3.4$, and $96.2{\pm}2.0$, respectively, at 3.0T. Percentage changes of T2 in the cingulate cortex, insular cortex, amygdaloid body, and hippocampal body at 3.0T with respect to those at 1.5T were -8.9%, -13.5%, -14.6%, and -13.5%, respectively. The mean T2 ratios of the cingulate gyrus, insular cortex, and amygdaloid body to the hippocampal body at 1.5T and 3.0T were 0.96 and 1.02 (p = 0.003); 1.02 and 1.03 (p>0.05); 0.97 and 0.98 (p>0.05), respectively. Conclusion : T2 decrease in the cingulate cortex was less than the amygdaloid body, insular cortex, and hippocampal body at 3.0T. The mean T2 ratio of the cingulate gyrus to the hippocampal body was significantly different between 1.5T and 3.0T.

• Journal of Life Science
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• v.15 no.4 s.71
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• pp.664-667
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• 2005

Hydrosyringomyelia is a dilation of the spinal cord central canal. In human it may be caused by congenital malformations such as Dandy-Walker syndrome and Chiari malformations or may be acquired as a result of infection, trauma or neoplasia. Hydrocephalus is an excessive accumulation of cerebrospinal fluid within the ventricles and occipital dysplasia is the dorsal extension of the foramen magnum. Hydrosyringomyelia and hydrocephalus can be confirmed by computed tomography or magnetic resonance imaging (MRI). A 3-year-old male maltese was presented with a history of long-term seizure. Blood examination was all unremarkable. On rostrodorsal-caudoventral oblique radiograph of the skull showed severe occipital dysplasia. On brain sonography through the persistent fontanelle, severe lateral ventriculomegaly was revealed. MRI examination revealed hydrocephalus and hydrosyringomyelia. Diuretic therapy didn't reduce clinical symptoms and surgical decompression was conducted. The dog responded well with ventriculo-peritoneal shunting. MRI is the most superior modality to diagnose hydrocephalus and hydrosyringomyelia, to plan therapy and to determine the prognosis.