• Clinical and Experimental Pediatrics
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• v.53 no.9
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• pp.859-862
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• 2010

Transient magnetic resonance (MR) signal changes in the splenium of the corpus callosum (SCC) arise from many different conditions, including encephalopathy or encephalitis caused by infection, seizures, metabolic derangements, and asphyxia. Few case reports exist on reversible SCC lesions associated with rotavirus infection. A benign convulsion with mild gastroenteritis (CwG) is frequently associated with rotaviral infections. This entity is characterized by normal laboratory findings, electroencephalogram, neuroimaging, and good prognosis. We report a case of a 2.5-year-old Korean girl with rotavirus-associated CwG demonstrating a reversible SCC lesion on diffusion-weighted MR images. She developed 2 episodes of brief generalized tonic-clonic seizure with mild acute gastroenteritis without any other neurologic abnormality. Stool test for rotavirus antigen was positive. Brain MRI done on the day of admission showed a linear high signal intensity and decreased apparent diffusion coefficient values on the SCC. The lesion completely disappeared on follow-up MRI 6 days later. The patient fully recovered without any sequelae.

• Korean Journal of Biological Psychiatry
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• v.13 no.1
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• pp.38-42
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• 2006

The case of a 66- year-old woman with coexisting idiopathic basal ganglia calcification(IBGC) and dementia was presented. The calcification was detected in bilateral basal ganglia, dentate nucleus, and thalamus by brain imaging. Serum calcium and phosphorus levels were normal. The underlying diseases of calcification of basal ganglia such as parathyroid dysfunction and other infectious, toxic, or metabolic illness were excluded. The patient had memory impairment and frontal executive dysfunction without aphasia, agnosia, apraxia, and visuospatial impairment in neuropsychological test. It suggested that the cognitive impairment might be due to the dysfunction of frontal-subcortical circuit.

• The Korean Journal of Nuclear Medicine
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• v.37 no.1
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• pp.1-12
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• 2003

Single photon emission computed tomography(SPECT) and positron emission tomography(PET) are modern imaging techniques that allow for both qualitative and quantitative assessment of hemodynamic changes in cerebrovascular diseases. SPECT has been becoming an indispensable method to investigate regional cerebral blood flow because equipment and isotope are easily available in most general hospitals. Acetazolamide stress SPECT has also been proved to be useful to evaluate the cerebrovascular reserve of occlusive cerebrovascular diseases and to select surgical candidate. PET has gained wide spread clinical use in the evaluation of the hemodynamic and metabolic consequences of extracranial or intracranial arterial obstructive disease despite its complexity and limited availability. PET has been established as an invaluable tool in the pathophysilogy investigation of acute ischemic stroke. The potentials, limitations, and clinical applications of SPECT and PET in various cerebrovascular diseases will be discussed in this article with reviews of literatures.

• Journal of Genetic Medicine
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• v.18 no.1
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• pp.55-59
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• 2021

West syndrome (WS) presenting with infantile spasms, developmental delay, and hypsarrhythmia has genetic etiology in some patients. Movement disorders or visual impairment that share genetic underpinnings with infantile spasms can provide diagnostic clues for specific genetic mutations. Mutations of the GRIN1 gene encoding the glutamate receptor inotropic N-methyl-D-aspartate subunit can result in WS with hyperkinetic movements, cortical visual impairment, autistic features, and bilateral polymicrogyria. An 11-month-old boy with WS showed hyperkinetic movements and visual impairment. Brain magnetic resonance imaging and metabolic investigations revealed no abnormalities. Whole-exome sequencing revealed a novel likely pathogenic variant (c.1561_1563del; p.Asn521del) of GRIN1 (NM_007327.3). The proband was treated with vigabatrin and became seizure-free within one week. Notably, the cortical blindness improved within 3 months and the hyperkinetic movements resolved one year after the proband became seizure-free. To the best of our knowledge, this is the first report of GRIN1 encephalopathy in Koreans.

• Imaging Science in Dentistry
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• v.52 no.2
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• pp.231-238
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• 2022

Intraosseous xanthoma of the mandible is a rare benign disorder. A 17-year-old male patient presented with a suspected abscess in the right mandibular third molar, detected on a panoramic radiograph. The patient had no history of systemic or lipid-related metabolic diseases and complained of no specific symptoms or pain. A radiographic examination revealed a heterogeneous radiolucency extending from the apical to the distal aspect of the right mandibular third molar tooth germ. The lesion measured 9 × 16 × 24 mm (antero-posterior × mediolateral × supero-inferior) and showed a relatively well-defined, multilocular, foamy appearance with hyperostotic borders spreading to the inferior alveolar nerve canal. After excisional biopsy, a diagnosis of central xanthoma was made. The lesion recurred, and intraoral vertical ramus osteotomy was done near the lesion. For the treatment of xanthoma of the mandible, extensive and delicate surgical treatment under general anesthesia should be considered.

• Korean Journal of Radiology
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• v.22 no.11
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• pp.1886-1893
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• 2021

Objective: To assess the feasibility of quantitatively assessing pancreatic steatosis using magnetic resonance imaging (MRI) and its correlation with obesity and metabolic risk factors in pediatric patients. Materials and Methods: Pediatric patients (≤ 18 years) who underwent liver fat quantification MRI between January 2016 and June 2019 were retrospectively included and divided into the obesity and control groups. Pancreatic proton density fat fraction (P-PDFF) was measured as the average value for three circular regions of interest (ROIs) drawn in the pancreatic head, body, and tail. Age, weight, laboratory results, and mean liver MRI values including liver PDFF (L-PDFF), stiffness on MR elastography, and T2^* values were assessed for their correlation with P-PDFF using linear regression analysis. The associations between P-PDFF and metabolic risk factors, including obesity, hypertension, diabetes mellitus (DM), and dyslipidemia, were assessed using logistic regression analysis. Results: A total of 172 patients (male:female = 125:47; mean ± standard deviation [SD], 13.2 ± 3.1 years) were included. The mean P-PDFF was significantly higher in the obesity group than in the control group (mean ± SD, 4.2 ± 2.5% vs. 3.4 ± 2.4%; p = 0.037). L-PDFF and liver stiffness values showed no significant correlation with P-PDFF (p = 0.235 and p = 0.567, respectively). P-PDFF was significantly associated with obesity (odds ratio 1.146, 95% confidence interval 1.006-1.307, p = 0.041), but there was no significant association with hypertension, DM, and dyslipidemia. Conclusion: MRI can be used to quantitatively measure pancreatic steatosis in children. P-PDFF is significantly associated with obesity in pediatric patients.

• Nuclear Medicine and Molecular Imaging
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• v.41 no.3
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• pp.252-254
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• 2007

Image fusion is fast catching attention as Wagner pointed out in his 2006 version of the recent progress and development presented at the annual meeting of Society of Nuclear Medicine. Prototypical fusion of bone scan and radiograph was already attempted at in 1961 when Fleming et al. published an article on strontium-85 bone scan. They simply superimposed dot scan on radiograph enabling simultaneous assessment of altered bone metabolism and local bone anatomy. Indeed the parallel reading of images of bone scan and radiography, CT, MRI or ultrasonography has been practiced in nuclear medicine long since. It is fortunate that recent development of computer science and technology along with the availability of refined CT and SPECT machines has permitted us to open a new avenue to digitally produce precise fusion image so that they can readily be read, exchanged and disseminated using internet. Ten years ago fusion was performed using Bresstrahlung SPECT/CT and it is now achievable by PET/CT and SPECT/CT software and SPECT/CT hardware. The merit of image fusion is its feasibility of reliable assessment of morphological and metabolic change. It is now applicable not only to stationary organs such as brain and skeleton but also to moving organs such as the heart, lung and stomach. Recently, we could create useful fusion image of cardiac SPECT and 64-channel CT angiograph. The former provided myocardial metabolic profile and the latter vascular narrowing in two patients with coronary artery stenosis and myocardial ischemia. Arterial stenosis was severe in Case 1 and mild in Case 2.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.11 no.1
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• pp.88-98
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• 2011

Lesch-Nyhan syndrome is a X-linked recessive disorder caused by a deficiency of the enzyme hypoxanthine-guanidine phosphoribosyltransferase (HPRT), enzyme to recycle purines. Case history: born induced vaginal delivery at 40 weeks complicated by premature membrane ruputure, body weight 2.820 gm. He showed failure to thrive showing severe protein aversion like milk products and pink daper. Developmental delay revealing rolling over at 10.5 month, followed by regression. Seizure at 2 months, His poor oral feeding was lifelong problem. Weak crying, spastic, choreoathetoid movement. Self mutilating behavior noted and diagnosed at age 3 years. No family history of consanguinity and neurological disorders. Method: Laboratory test, physical exam, imaging study and molecular. Clinical follow up Treat ment with allopurinol. Result: uric acid 10.5 mg/dL (N 3.5-7.9), APRT 151.1uM/ min/ml pro(25.7-101), HPRT 7.6 (N 233.5-701) and c.151C>T hemizygote (p,Arg51X). Abdominal sonogram showed staghorn calculi in both kidneys, brain MRI brain atrophy. Clinical follow up showed, seizure at 2 mo, developmental delay (head control and, rolling over at at 11mo, pointing body part at 2 yr 7 mo, eye hand coordination at 2 y 11mo,creeping at 3 y 7 mo, speaking words at 6 y 6 mo ),and developmental regression at 3 yr of age. Sleeping problem including insomnia and severe constipation. Self mutilating behavior (lip bite) started at 2.5 yr, neurologic sx including intermittent upward gaze accompanied by swallowing difficulty at 3 y 7 mo grand mal seizure at 4.5 yr and spastic extremity and trunchal hypotonia and choleoathetoid movement and ataxia at 6.5 yr. Scoliosis with severe spasticity at 9 yr 9 mo. Acute life threatening episode with irregular breathing at 9 yr and 9 mo, Emaciation and nephrolithiasis and recurrent pneumonia. Died suddenly at 10 yr 3 mo. Conclusion: life long feeding problem, chronic gut motility dysfunction, sleeping difficulty and progressing neurologic deterioration and nephrolithiasis despite normal serum uric acid maintence by allopurinol treatment.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.1
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• pp.54-59
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• 2014

Adrenoleukodystrophy is an X-linked genetic disease resulting from mutations in the ABCD1 gene. Cerebral adrenoleukodystrophy is one of the phenotypes of adrenoleukodystrophy and shows progressive demyelination of brain white matter and adrenal insufficiency. We report a nine year old male who presented with rapidly progressive cognitive and neurologic deterioration. He had abnormal findings in brain imaging and elevated very long chain fatty acid level in serum. Mutation analysis of ABCD1 revealed a c. 1252C>T (p.Arg418Trp) mutation which was previously known but not reported in Korea.

• The Plant Pathology Journal
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• v.32 no.4
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• pp.300-310
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• 2016

Pathogen infection in plants induces complex responses ranging from gene expression to metabolic processes in infected plants. In spite of many studies on biotic stress-related changes in host plants, little is known about the metabolic and phenotypic responses of the host plants to Pseudomonas cichorii infection based on image-based analysis. To investigate alterations in tomato plants according to disease severity, we inoculated plants with different cell densities of P. cichorii using dipping and syringe infiltration methods. High-dose inocula (${\geq}10^6cfu/ml$) induced evident necrotic lesions within one day that corresponded to bacterial growth in the infected tissues. Among the chlorophyll fluorescence parameters analyzed, changes in quantum yield of PSII (${\Phi}PSII$) and non-photochemical quenching (NPQ) preceded the appearance of visible symptoms, but maximum quantum efficiency of PSII ($F_v/F_m$) was altered well after symptom development. Visible/near infrared and chlorophyll fluorescence hyperspectral images detected changes before symptom appearance at low-density inoculation. The results of this study indicate that the P. cichorii infection severity can be detected by chlorophyll fluorescence assay and hyperspectral images prior to the onset of visible symptoms, indicating the feasibility of early detection of diseases. However, to detect disease development by hyperspectral imaging, more detailed protocols and analyses are necessary. Taken together, change in chlorophyll fluorescence is a good parameter for early detection of P. cichorii infection in tomato plants. In addition, image-based visualization of infection severity before visual damage appearance will contribute to effective management of plant diseases.