• The Journal of Internal Korean Medicine
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• v.33 no.1
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• pp.102-110
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• 2012

Multiple system atrophy (MSA) is a sporadic progressive neurodegenerative disorder characterized clinically by various combinations of parkinsonian, autonomic, cerebellar, or pyramidal signs and pathologically by cell loss, gliosis, and ${\alpha}$-synuclein-positive glial cytoplasmic inclusions in several brain and spinal cord structures. This is a clinical report about a 69-year-old female who had MSA treated by oriental medical treatment and evaluated by Unified Multiple System Atrophy Rating Scale (UMSARS). The patient was treated with herb medicine Chungsimyeonj-aeumgami(淸心蓮子飮加味), acupuncture, moxibustion and cupping. After treatment, the patient's symptoms improved meaningfully and the score decreased in UMSARS Part I, II. This suggests that oriental medical treatment could be effective to improve MSA patients' symptoms. It is necessary to have more observations and many cases of patients with MSA.

• 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.

• Clinical and Experimental Pediatrics
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• v.49 no.2
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• pp.208-211
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• 2006

Dyke-Davidoff-Masson Syndrome (DDMS) is a rare condition characterized by asymmetry of cerebral hemispheric growth with atrophy on one side, ipsilateral compensatory osseous hypertrophy, and contralateral hemiparesis. We experienced a 17 month-old male who presented with left focal clonic or tonic-clonic seizures accompanied by left hemiparesis and developmental delay. Brain MRIs demonstrated progressive atrophy of the right cerebral hemisphere with dilatation of the lateral ventricle, expansion of the ipsilateral frontal sinus with calvarial thickening, and elevation of the petrous pyramid and orbital roof. Brain SPECT showed a decreased volume of the right hemisphere with reduced blood flow. We therefore report a case of DDMS with a review of the literature.

• Korean Journal of Biological Psychiatry
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• v.8 no.1
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• pp.3-19
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• 2001

Recent basic and clinical studies demonstrate a major role for neural plasticity in the etiology and treatment of depression and stress-related illness. The neural plasticity is reflected both in the birth of new cell in the adult brain(neurogenesis) and the death of genetically healthy cells(apoptosis) in the response to the individual's interaction with the environment. The neural plasticity includes adaptations of intracellular signal transduction pathway and gene expression, as well as alterations in neuronal morphology and cell survival. At the cellular level, repeated stress causes shortening and debranching of dendrite in the CA3 region of hippocampus and suppress neurogenesis of dentate gyrus granule neurons. At the molecular level, both form of structural remodeling appear to be mediated by glucocorticoid hormone working in concert with glutamate and N-methyl-D-aspartate(NMDA) receptor, along with transmitters such as serotonin and GABA-benzodiazepine system. In addition, the decreased expression and reduced level of brain-derived neurotrophic factor(BDNF) could contribute the atrophy and decreased function of stress-vulnerable hippocampal neurons. It is also suggested that atrophy and death of neurons in the hippocampus, as well as prefrontal cortex and possibly other regions, could contribute to the pathophysiology of depression. Antidepressant treatment could oppose these adverse cellular effects, which may be regarded as a loss of neural plasticity, by blocking or reversing the atrophy of hippocampal neurons and by increasing cell survival and function via up-regulation of cyclic adenosine monophosphate response element-binding proteins(CREB) and BDNF. In this article, the molecular and cellular mechanisms that underlie stress, depression, and action of antidepressant are precisely discussed.

• 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 Korean Academy of Nursing
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• v.34 no.1
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• pp.150-159
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• 2004

Purpose: The purpose of this study was to determine the effect of DHEA on hindlimb muscles(soleus, plantaris and gastrocnemius) in a focal brain ischemia model rat. Method: Twenty-seven male Sprague-Dawley rats were randomly divided into three groups: CINS(cerebral ischemia + normal saline), CIDH(cerebral ischemia + DHEA), or SHNS(sham + normal saline). Both the CINS and CIDH groups underwent a transient right middle cerebral artery occlusion operation. In the SHNS group, a sham operation was done. 0.34mmol/kg DHEA was administered daily by an intraperitoneal injection for 7days. Results: The muscle weight, muscle fiber cross-sectional area of the Type I muscle fiber of soleus and Type II muscle fiber of plantaris and gastrocnemius, myofibrillar protein content of gastrocnemius, and muscle strength in the CINS group decreased compared with the SHNS group. The muscle weight, muscle fiber cross-sectional area of the Type II muscle fiber of plantaris and gastrocnemius, myofibrillar protein content of soleus, and muscle strength in the CIDH group increased compared with the CINS group. Conclusion: It was identified that muscle atrophy could be induced during 7 days after a cerebral infarction, and DHEA administration during the early stages of a cerebral infarction might attenuate muscle atrophy.

• Journal of Genetic Medicine
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• v.18 no.1
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• pp.44-47
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• 2021

Cerebral creatine deficiency syndrome (CCDS) is a disorder where a defect is present in transport of creatine in the brain. Creatine plays an essential role in the energy metabolism of the brain. This is a genetic disorder, autosomal recessive or X linked, characterized by intellectual disability, speech and language delay, epilepsy, hypotonia, etc. Until recently very few number of cases have been reported. Here we report a case of 1.5-year-old boy who had epilepsy (epileptic spasm and generalized tonic clonic seizure), intellectual disability, microcephaly, hypotonia and speech delay. His magnetic resonance imaging of brain showed cortical atrophy and electroencephalography showed burst suppression pattern. The diagnosis was confirmed by clinical exome sequencing which showed novel mutation of SLC6A8+ in exon 9, suggestive of X linked recessive CCDS. The patient was then treated with glycine, L-arginine and creatine monohydrate with multiple antiepileptic drugs.

• The Korean Journal of Nuclear Medicine
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• v.22 no.2
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• pp.163-170
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• 1988

8 patients of delayed CO sequelae were evaluated using Brain CT and $^{99m}Tc-HMPAO$ SPECT. The results were as follows; 1) CT findings of delayed CO sequleae were bilateral low density lesion in globus pallidus (l pt.), diffuse low density in white matter with bilateral low density in white matter (l pt.), diffuse low density in white matter with bilateral low density in globus pallidus (l pt.), diffuse low density in white matter with cortical atrophy (l pt.), bilateral low density in globus pallidus and diffuse low density in white matter with cortical atrophy (l pt.) and normal in 3 pts. 2) $^{99m}Tc-HMPAO$ Brain SPECT findings of delayed CO sequelae were decreased regional cerebral blood flow (rCBF) in frontal (1 among 8 pts.), frontal and basal ganglia (3 among 8 pts.), and diffuse patch decreased rCBF pattern (4 among 8 pts.) 3) $^{99m}Tc-HMPAO$ Brain SPECT study was well correlated with neurologic symptoms and signs in delayed CO sequelae. Our results may suggest that reduced cerebral blood flow contributes to the development of delayed CO sequelae.

• Journal of Medicine and Life Science
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• v.17 no.3
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• pp.65-73
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• 2020

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a single-gene disease of the cerebral small blood vessels caused by mutations in the NOTCH3 gene on chromosome 19. Although CADASIL was known as a rare disease, recent research has suggested that the NOTCH variants could be found frequently even in the general population. The main clinical features included recurrent stroke, migraine, psychiatric symptoms, and progressive cognitive decline. On brain magnetic resonance imaging, patients with CADASIL showed multifocal white matter hyperintensity lesions, lacunar infarcts, microbleeds, and brain atrophy. Among them, lacunar infarcts and brain atrophy are important in predicting the clinical outcomes of patients with CADASIL. In the Jeju National University Hospital, we have diagnosed 213 CADASIL patients from 2004 to 2020. Most NOTCH3 mutations were located in exon 11 (94.4%), and p.Arg544Cys was the most common mutation. The mean age at diagnosis was 61.0±12.8 years. The most common presenting symptoms were ischemic stroke (24.4%), followed by cognitive impairment(15.0%), headache (8.9%), and dizziness(8.0%). Although the exact prevalence of CADASIL in Jeju is still unknown, the disease prevalence could be as high as 1% of the population considering the prevalence reported in Taiwan. Therefore, it is necessary to discover efficient biomarkers and genetic tests that can accurately screen and diagnose patients suspected of having CADASIL in this region. Ultimately, it is urgent to explore the exact pathogenesis of the disease to identify leading substances of treatment potential, and for this, multi-disciplinary research through active support from the Jeju provincial government as well as the national government is essential.

• The Journal of Korean Medicine
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• v.21 no.4
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• pp.286-291
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• 2000

Behcet's disease is a systemic disease affecting multiple organs including the central nervous system. Neuro-Behcet's disease was regarded as relatively rare, but thanks to the development of diagnostic tools, more and more cases are being reported. We are reporting a case of neuro-Behcet's disease in which the patient displayed paraparesis, dysarthria and involuntary tremor as neurologic symptoms. The patient's brain MRI showed cerebellar atrophy, and a spinal cord MRI failed to reveal any significant lesions. The patient experienced a couple of fever attacks during hospitalization, which were managed adequately by herbal medicines. Her main neurological symptoms such as paraparesis were, however, grossly unchanged at discharge.