• The Journal of the Society of Stroke on Korean Medicine
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• v.13 no.1
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• pp.77-85
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• 2012

Amyotrophic lateral sclerosis(ALS) is a progressive disorder that causes degeneration of motor neurons of the brain and spinal cord. It is characterized by loss of motor neurons leading to muscle weakness, and sensation and mental function stay intact during the course of the disease. Diagnostic tests include magnetic response imaging(MRI), electromyogram(EMG), muscle biopsy, and blood tests. In order to a definitive diagnosis of ALS, damage must be evident in both upper and lower motor neurons. When three limbs are sufficiently affected, the diagnosis is ALS. There is no cure for ALS. We experienced one case of ALS, the patients was diagnosed as ALS by EMG and symptoms. We diagnosed her as ShinEumHur(SEH) and treated by Choakwiyeum(左歸飮). We report the change of her symptoms through oriental medical treatment.

• Radiation Oncology Journal
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• v.27 no.1
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• pp.23-28
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• 2009

Purpose: This study was designed to evaluate the effectiveness of postoperative radiotherapy for patients with low-grade astrocytomas and to define an optimal radiotherapeutic regimen and prognostic factors. Materials and Methods: A total of 69 patients with low-grade astrocytomas underwent surgery and postoperative radiotherapy immediately following surgery at our institution between October 1989 and September 2006. The median patient age was 36 years. Forty-one patients were 40 years or younger and 28 patients were 41 years or older. Fourteen patients underwent a biopsy alone and the remaining 55 patients underwent a subtotal resection. Thirty-nine patients had a Karnofsky performance status of less than 80% and 30 patients had a Karnofsky performance status greater than 80%. Two patients were treated with whole brain irradiation followed by a coned down boost field to the localized area. The remaining 67 patients were treated with a localized field with an appropriate margin. Most of the patients received a dose of $50\sim55$ Gy and majority of the patients were treated with a dose of 54 Gy. Results: The overall 5-year and 7-year survival rates for all of the 69 patients were 49% and 44%, respectively. Corresponding disease free survival rates were 45% and 40%, respectively. Patients who underwent a subtotal resection showed better survival than patients who underwent a biopsy alone. The overall 5-year survival rates for patients who underwent a subtotal resection and patients who underwent a biopsy alone were 57% and 38%, respectively (p<0.05). Forty-one patients who were 40 years or younger showed a better overall 5-year survival rate as compared with 28 patients who were 41 years or older (56% versus 40%, p<0.05). The overall 5-year survival rates for 30 patients with a Karnofsky performance status greater than 80% and 39 patients with a Karnofsky performance status less than 80% were 51% and 47%, respectively. This finding was not statistically significant. Although one patient was not able to complete the treatment because of neurological deterioration, there were no significant treatment related toxicities. Conclusion: Postoperative radiotherapy following surgery is a safe and effective treatment for patients with low-grade astrocytomas. The extent of surgery and age were noted as significant prognostic factors in this study. However, further effective treatment might be necessary in the future to improve long-term survival rates.

• Journal of Korean Neurosurgical Society
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• v.30 no.12
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• pp.1399-1405
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• 2001

Objectives : Gliomatosis cerebri is an uncommon primary brain tumor characterized by diffuse neoplastic proliferation of glial cells, with the preservation of the underlying cytoarchitecture. The aim of this study is to evaluate clinical features, outcome of surgical treatment and adjuvant therapy of gliomatosis cerebri. Methods : Between Jan. 1990 and Dec. 2000, 12 patients were diagnosed with gliomatosis cerebri based on characteristic radiological and histological findings. The patients' age ranged from 18 to 77(mean 44) years and the male to female ratio was 7 : 5. Nine patients underwent decompressive surgery and three, biopsy only. Postoperative radiation therapy was given in all cases except three. In addition to radiation therapy, four patients received chemotherapy. The mean duration of follow-up period was 18.8 months. Results : The most common presenting symptom were seizure and motor weakness. The mean duration of symptom was 5.9 months. There was 5 bilateral lesions and tumor involved corpus callosum in 5, basal ganglia-thalamus in 4, and brain stem in 2. There was no operative mortality but four patients died during the follow-up. The mean survival period for 11 patients was 20.5 months from the time of diagnosis. In univariate analysis, the lesion involving corpus callosum, basal ganglia-thalamus and brain stem correlated significantly with the short length of survival(p<0.05). Also, postoperative radiation as a adjuvant therapy prolonged the patient's survival(p<0.05). Conclusions : In the management of gliomatosis cerebri patients, early detection by MR imaging, active management of increased intracranial pressure, decompressive surgical removal and postoperative adjuvant therapy such as radiation is thought to be a good treatment modality.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.33 no.5
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• pp.535-542
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• 2007

Ameloblastoma, a benign tumor of odontogenic type, represents 10% of all tumors of the jaw. It is localized in the mandible(80%) and in the maxilla(20%). In every case, the selection of the surgical treatment must consider some fundamental elements, including the age and general state of health the clinicopathological variant, and the localization and extent of the tumor. This study was invested the clinicopathological findings of 23 patients with ameloblastoma which had been diagnosed by biopsy during the period of 1987 to 2005 at Chonnam National University Hospital. And it contained the statistical analysis according to the treatment methods and the clinicopathological findings such as sex, age, location, chief complaints, duration, radiographic findings, histologic findings, treatment methods. The results obtained are were follows. The age of patient ranged from 10 to 91 years(means, 35.9 years) at biopsy. Thirteen(57%) of the 23 subjects were males, and 10(43%) were females. Twenty(87%) of the 23 ameloblastomas were located in the mandible. Swelling was the most common symptom and was experienced by 20(87%) patients. Radiographically, 11(48%) of the 23 tumors were unilocular with a well-demarcated border and 12(52%) were multilocular. The most common histologic pattern was plexiform and acanthomatous rather then follicular. Conservative treatment was performed 7 cases(30%), radical treatment 11 cases(48%), and combined treatment 5 cases(22%). Follow-up period ranged from 2.1 years to 22 years(mean 5.1 years). Based on the above results, surgical excision after marsupialization was found to be useful as a preliminary treatment of the large cystic ameloblastoma in children and adolescents. On the contrary, the lesion with a soap bubble appearance, the one with ineffective marsupialization was subjected to extensive excision of the tumor with a wide margin of normal bone.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.2
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• pp.48-54
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• 2017

Purpose: Fukuyama congenital muscular dystrophy (FCMD) is a rare, autosomal-recessive disorder characterized by early-onset hypotonia associated with brain malformations in dystroglycanopathy. Although the wide spectrum of congenital muscular dystrophies causes difficulty in diagnosis, correlating the genotype with the clinical phenotype can help diagnose FCMD. Here, we evaluated the correlation of targeted molecular genetic analysis of FKTN gene mutation with the FCMD phenotype. Methods: This study was conducted retrospectively with 9 subjects. Inclusion criteria included clinical symptoms characterized by early-onset hypotonia with magnetic resonance imaging (MRI) featuring brain malformations. FKTN gene-alteration analysis was performed using various FKTN gene-analysis methods, including sequencing. Results: Among the 9 subjects studied, 4 (44.4%) were male and 5 (55.6%) were female. The median age of onset of the first symptom was 3.1 months. The first symptom was a delayed milestone in 6 cases (66.7%). All 9 subjects (100%) presented with early-onset hypotonia and global delayed development. All subjects presented with cortical malformation in their brain MRIs. Of the 9 subjects, 6 subjects had previously undergone muscle biopsy and 4 cases (4/6; 66.7%) showed dystrophic or myopathic features. Pathogenic mutations causing FCMD were identified in 3 cases. Conclusions: In this study, all 3 subjects with FKTN mutations showed important MRI findings (pachygyria and cerebellar dysplasia). These data suggest that patients with characteristic phenotypes who show pachygyria and cerebellar abnormalities in brain MRIs may have a high probability of being diagnosed with FCMD.

• Journal of radiological science and technology
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• v.32 no.3
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• pp.325-334
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• 2009

Purpose : This study was conducted to assess how effective the permeability ratio and relative cerebral blood volume ratio are to tumor through perfusion MRI by measuring and reflecting the grade assessment and differential diagnosis and the permeability and relative cerebral blood volume of contrast media plunged from blood vessel into organ due to breakdown of blood-brain barrier in cerebral. Subject and Method : Subject of study was 29 patients whose diagnosis were confirmed by biopsy after surgery and 550 (11 slice$\times$50 image) perfusion MRI were used to make image of relative cerebral blood volume with the program furnished on instrument. The other method was to transmit to private computer and the image analysis was made additionally by making image of relative cerebral blood volume-reformulated singular value decomposition, rCBV-rSVD and permeability using IDL.6.2. In addition, Kruskal-wallis test tonggyein non numerical average by a comparative analysis of brain tumors Results : The rCBV ratio (Functool PF; GE Medical Systems and IDL 6.2 program by analysis) and permeability ratio of tumors were as follows; high grade glioma(n=4), (14.75, 19.25) 13.13. low grade astrocytoma(n=5) (14.80, 15.90) 11.60, glioblastoma(n=5) (10.90, 18.60), 22.00, metastasis(n=6) (11.00, 15.08). 22.33. meningioma(n=6) (18.58, 7.67), 5.58. oliogodendroglioma(n=3) (23.33, 16.33, 15.67. Conclusion : It was not easy to classify the grade with the relative cerebral blood volume ratio measured by using the relative cerebral blood image by type of tumors, however, permeability ratio measured by permeability image revealed that the higher the grade of tumor, the higher the measured permeability ratio, showing the assessment of tumor grade is more effective to differential diagnosis.

• Radiation Oncology Journal
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• v.6 no.2
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• pp.177-182
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• 1988

A retrospective analysis was peformed on 23 patients with pineal region tumors treated with radiation from 1979 through 1985 at the Department of Therapeutic Radiology, Seoul National University Hospital. Histologic confirmation was done in only one case by surgical removal, and in the remaining 22 patients, the diagnosis was based on clinical and radiological findings. The radiation volume was the primary tumor site in 1 case, whole brain in 14 cases, and the whole craniospinal axis En 8 cases. The overall 5 year survival was $71.5\%$. The 5 year survival was $69.3\%$ for whole brain treated group and $73.3\%$ for craniospinal axis treated group. The survival for the two groups did not differ significantly. In two cases sites of recurrence were detected, one in supratentorial area, and the other in the lung. The results from this retrospective analysis and the review of other reports indicate that routine use of prophylactic spinal irradiation is not warranted in pineal region tumor, and the craniospinal irradiation is recommended in cases with high risk for subarachnoid seeding such as positive CSF cytology, surgical removal or biopsy.

• Neonatal Medicine
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• v.17 no.2
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• pp.254-261
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• 2010

Little is known about neonatal mitochondrial disease, though mitochondrial metabolic disorders may often present in the neonatal period because of the high energy requirement of neonate. In newborn period, common presentations are not specific and the disease course may be rapid and fatal. In this study, we report three cases of neonatal mitochondrial disease. The first case was strongly suspected because of sudden seizure and mental change with severe lactic acidosis, and multiorgan failure. Plasma lactate/pyruvate (L/P) ratio was increased to 55.6 with marked lactic aciduria and increased plasma alanin up to 2,237 nmol/mL. In the second patient, a peritoneal dialysis was performed for acute adrenal and renal failure, but metabolic acidosis persisted. Plasma L/P ratio was increased to 23.9, and MRC I (mitochondrial respiratory chain defect) was diagnosed through the enzymatic analysis of the muscles. The third case showed repetitive episode of lactic acidosis during the first two months of life, hypotonia, failure to thrive and feeding difficulties. We found markedly increased cerebrospinal fluid L/P ratio up to 57 though plasma L/P ratio(19.4) was borderline with increased plasma lactate. The lactate peak was prominent in brain magnetic resonance spectroscopy (MRS). MRC II was confirmed through muscle biopsy. Plasma lactate level and lactate peak of brain MRS were normalized after conservative treatment.

• Investigative Magnetic Resonance Imaging
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• v.21 no.1
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• pp.9-19
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• 2017

Background: Normalized cerebral blood volume (nCBV) can be measured using manual or semiautomatic segmentation method. However, the difference in diagnostic performance on brain tumor differentiation between differently measured nCBV has not been evaluated. Purpose: To compare the diagnostic performance of manually obtained nCBV to that of semiautomatically obtained nCBV on glioblastoma (GBM) and primary central nervous system lymphoma (PCNSL) differentiation. Materials and Methods: Histopathologically confirmed forty GBM and eleven PCNSL patients underwent 3T MR imaging with dynamic susceptibility contrast-enhanced perfusion MR imaging before any treatment or biopsy. Based on the contrast-enhanced T1-weighted imaging, the mean nCBV (mCBV) was measured using the manual method (manual mCBV), random regions of interest (ROIs) placement by the observer, or the semiautomatic segmentation method (semiautomatic mCBV). The volume of enhancing portion of the tumor was also measured during semiautomatic segmentation process. T-test, ROC curve analysis, Fisher's exact test and multivariate regression analysis were performed to compare the value and evaluate the diagnostic performance of each parameter. Results: GBM showed a higher enhancing volume (P = 0.0307), a higher manual mCBV (P = 0.018) and a higher semiautomatic mCBV (P = 0.0111) than that of the PCNSL. Semiautomatic mCBV had the highest value (0.815) for the area under the curve (AUC), however, the AUCs of the three parameters were not significantly different from each other. The semiautomatic mCBV was the best independent predictor for the GBM and PCNSL differential diagnosis according to the stepwise multiple regression analysis. Conclusion: We found that the semiautomatic mCBV could be a better predictor than the manual mCBV for the GBM and PCNSL differentiation. We believe that the semiautomatic segmentation method can contribute to the advancement of perfusion based brain tumor evaluation.

• Brain Tumor Research and Treatment
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• v.6 no.2
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• pp.60-67
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• 2018

Background Recently, modern technology such as diffusion tensor imaging (DTI), neuro-navigation and intraoperative neurophysiological monitoring (IOM) have been actively adopted for the treatment of thalamic tumors. We evaluated surgical outcomes and efficacy of the aforementioned technologies for the treatment of pediatric thalamic tumors. Methods We retrospectively reviewed clinical data from 37 children with thalamic tumors between 2004 and 2017. There were 44 operations (27 tumor resections, 17 biopsies). DTI was employed in 17 cases, neuro-navigation in 23 cases and IOM in 14 cases. All diagnoses were revised according to the 2016 World Health Organization Classification of Tumors of the Central Nervous System. Progression-free survival (PFS) and overall survival (OS) rates were calculated, and relevant prognostic factors were analyzed. The median follow-up duration was 19 months. Results Fifteen cases were gross total resections (GTR), 6 subtotal resections (STR), and 6 partial resections (PR). Neurological status did not worsen after 22 tumor resections. There were statistically significant differences in terms of the extent of resection between the groups with DTI, neuro-navigation and IOM (n=12, GTR or STR=12) and the group without at least one of the three techniques (n= 15, GTR or STR=9, p=0.020). The mean PFS was $87.2{\pm}38.0$ months, and the mean OS $90.7{\pm}36.1$ months. The 5-year PFS was 37%, and the 5-year OS 47%. The histological grade ($p{\leq}0.001$) and adjuvant therapy (done vs. not done, p=0.016) were significantly related to longer PFS. The histological grade (p=0.002) and the extent of removal (GTR/STR vs. PR/biopsy, p=0.047) were significantly related to longer OS. Conclusion Maximal surgical resection was achieved with acceptable morbidity in children with thalamic tumors by employing DTI, neuro-navigation and IOM. Maximal tumor resection was a relevant clinical factor affecting OS; therefore, it should be considered the initial therapeutic option for pediatric thalamic tumors.