• Molecules and Cells
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• v.27 no.6
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• pp.621-627
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• 2009

Spinocerebellar ataxia type 1 (SCA1) is an autosomal-dominant neurodegenerative disorder characterized by ataxia and progressive motor deterioration. SCA1 is associated with an elongated polyglutamine tract in ataxin-1, the SCA1 gene product. As summarized in this review, recent studies have clarified the molecular mechanisms of SCA1 pathogenesis and provided direction for future therapeutic approaches. The nucleus is the subcellular site where misfolded mutant ataxin-1 acts to cause SCA1 disease in the cerebellum. The role of these nuclear aggregates is the subject of intensive study. Additional proteins have been identified, whose conformational alterations occurring through interactions with the polyglutamine tract itself or non-polyglutamine regions in ataxin-1 are the cause of SCA-1 cytotoxicity. Therapeutic hope comes from the observations concerning the reduction of nuclear aggregation and alleviation of the pathogenic phenotype by the application of potent inhibitors and RNA interference.

• Korean Journal of Biological Psychiatry
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• v.10 no.2
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• pp.97-106
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• 2003

Criticisms about amyloid cascade hypothesis of Alzheimer's disease(AD) are based on the findings, first, that the degree of dementia does not correlate with the number of plaques, and second, that the neurofibrillary tangle formation seems to predate plaque formation. In addition, neurofibrillary tangle counts correlate well with the degree of cognitive impairment. These findings suggest the independent importance of tau abnormality in AD research which is involved in the neurofibrillary tangle formation. Recently, tau pathology without amyloid deposits and mutations in tau protein gene were reported to be the major pathogenic mechanism in Pick's disease, progressive supranuclear palsy, corticobasal degeneration and FTDP-17(frontotemporal dementia and parkinsonism linked with chromosome 17). These data suggest that understanding the causes and consequences of tau dysfunction might give new clinical and therapeutic solutions to many known tauopathies.

• Journal of Chest Surgery
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• v.12 no.1
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• pp.23-29
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• 1979

Hyuk An, M.D.Atrial myxoma constitutes the most significant of all cardiac neoplasm. About 75% occur in the left atrium and 25% in the right. They may cause severe and progressive disease mimicking valvular heart disease. But recently attention to differential point in the clinical features along with various diagnostic techniques have allowed pre-operative diagnosis in the most cases. From April, `77 to Aug., `78, three cases of left atrial myxoma and one case of right atrial myxoma were operated in Seoul National University Hospital. Pre-operative diagnosis was established with echocardiography and cardiac angiography. In all cases, tumors were resected with cardiopulmonary bypass successfully, and discharged with good results.

• Journal of Korean Neurosurgical Society
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• v.49 no.4
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• pp.234-236
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• 2011

Lumbar spinal stenosis (LSS) is a common spinal disease in the elderly. The cardinal symptom of LSS is neurogenic claudication, but not all patients present with such typical symptom. The clinical symptoms are often confused with symptoms of peripheral neuropathy, musculo-skeletal disease and other medical conditions in elderly patients. In particular, LSS presenting with rapid progression of leg weakness must be distinguished from other combined diseases. We report a case of rapid progressive leg weakness in a patient with LSS and iatrogenic adrenal insufficiency that was induced by obscure health supplement.

• Journal of Chest Surgery
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• v.15 no.1
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• pp.98-106
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• 1982

Cardiac Myxoma is a most frequent benign tumor in primary cardiac tumors. About 75% occur in the left atrium & 20% in the right atrium, ventricular Myxoma is a very rare one among the cardiac myxoma. They may cause severe and progressive disease resembling valvular heart disease. With the advent of various diagnostic modalities, especially real time bidimensional echocardiography enabled us more accurate diagnosis of cardiac myxoma noninvasively and preoperatively. From April 1977 to Sept. 1981, 16 cases of cardiac myxomas were operated in Seoul National University Hospital using cardiopulmonary bypass. There were 13 cases of left atrial myxomas and each of a case was right atrial and right ventricular and left ventricular one. In all cases tumors were resected successfully & were discharged in healthy state. Follow up results of each patient was excellent.

• Journal of Korean Neurosurgical Society
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• v.42 no.3
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• pp.216-219
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• 2007

Lymphocytic hypophysitis is a clinically rare disease, and it has been known to be an autoimmune disease which mainly affects pregnant women at the end of gestation or right after delivery. The authors experienced a case of lymphocytic hypophysitis in a 29-year-old pregnant woman with rapid progressing visual disturbance. Sella MRI showed a mass-like lesion of hypophysis and hypertrophy of pituitary stalk with evidences of hypopituitarism. Cesarean section was done and then TSA was performed. The pathologic diagnosis was lymphocytic hypophysitis. After TSA, visual acuity was improved and visual field defect was recovered. She was given thyroid hormone replacement therapy because of transient partial hypopituitarism for 6 months after surgery. One must consider the probability of lymphocytic hypophysitis, if there are alteration of visual acuity and visual field defect which aggravate rapidly during pregnancy due to mass effect, decreased serum hormonal levels shown in hypopituitarism and sella MRI findings of hypertrophy of pituitary stalk and enlargement of pituitary gland.

• Journal of Korean Neurosurgical Society
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• v.57 no.6
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• pp.401-407
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• 2015

The nonprogressive unilateral intracranial arteriopathy known as transient (focal) cerebral arteriopathy is not a well-recognized arteriopathy among practitioners of Korea and Japan, although it cannot be easily differentiated from early moyamoya disease. This review summarizes the nomenclature, pathophysiology, diagnostic evaluation, clinico-radiological features, and management of nonprogressive (reversible or stable) unilateral arteriopathy based on the relevant literature and our own experiences. Nonprogressive unilateral arteriopathy should be strongly suspected in children presenting with basal ganglia infarction and arterial beading. The early identification of patients likely to have nonprogressive or progressive arteriopathy would ensure proper management and guide further research for secondary stroke prevention.

• Journal of Chest Surgery
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• v.11 no.1
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• pp.58-64
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• 1978

Left atrial myxoma, an unusual clinical entity, may cause severe and progressive cardiac disease mimicking mitral valvular disease. In recent years, increased clinical awareness and improved diagnostic techniques has led to a higher incidence of correct preoperative diagnosis. Recently we experienced 2 cases of left atrial myxoma, which were removed successfully under the cardiopulmonary by pass. The first case was 45 years old woman and the second was 23 years old female. Preoperative definite diagnosis was entertained by angiocardiography and echocardiography in both cases. In the first case, tumor was removed with left atriotomy and atrial septectomy was done with additional right atriotomy. In the second case, tumor and atrial septum were removed en bloc through the right atrium. Both patients were discharged with good results 2 weeks postoperatively.

• International Journal of Oral Biology
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• v.45 no.2
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• pp.33-41
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• 2020

Neuroinflammation is known as the main mechanism implicated in the advancement of neurodegenerative diseases such as Alzheimer's disease and Parkinson's disease. The main feature of neuroinflammation is associated with the activation of microglia. The activated microglia increase proinflammatory cytokine production and induce progressive neuronal cell death. Citrus flavonoids show neuroprotective effects that are associated with the anti-inflammatory action of flavonoids in neurodegenerative diseases. Among these citrus flavonoids, kaempferol, naringin, and nobiletin show inhibitory effects on nuclear factor-κB and mitogen-activated protein kinase signaling pathways that can modulate inflammatory conditions in microglial cells. In the present review, we present the anti-inflammatory activities of citrus flavonoids and therapeutic potential of flavonoids as neuroprotective agents.

• KSBB Journal
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• v.28 no.5
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• pp.269-274
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• 2013

Motor neuron disease (MND) is a fatal neurodegenerative disorder caused by progressive and selective degeneration of motor neurons (MNs). Because of the versatile nature, stem cells have the potential to repair or replace the degenerated cells. In this review, we discussed stem cell based therapies including the use of embryonic stem cells (ESCs), neural stem cells (NSCs), induced pluripotent stem cells (iPSCs) and genetically engineered cells to produce the neurotrophic factors for the treatment of MND. To achieve this goal, the knowledge of specificity of the cell target, homing and special markers are required.