The confirmatory diagnosis of Moyamoya disease has been obtained by invasive angiographic examination. We report the results of MRI and transcranial doppler sonography of three cases ol Moyamoya disease, which ws disgnosed by clinical and angiography. We think that the diagnosis of Moyamoya disease can be made by noninvasive MRI and transcranial doppler sonography without conventional invasive angiography.
Angiography ; Diagnosis ; Magnetic Resonance Imaging* ; Moyamoya Disease* ; Ultrasonography, Doppler, Transcranial*

BACKGROUND & OBJECTIVE: Huntington disease(HD) is clinically diagnosed by the triad of autosomal dominant inheritance, involuntary movements mainly chorea and dementia. The phenotype of HD is variable and other diseases can have same phenocopy. Therefore gene diagnosis of HD becomes essential for confirmatory diagnosis. Recent discovery of an expanded CAG trinucleotide repeat at the telomeric position of chromosome 4p made the gene diagnosis possible even in sporadic cases. We examined the length of CAG repeat in Huntington gene locus by PCR method in clinically diagnosed HB patients to make a confirmatory diagnosis. METHODS: Three patients with chorea, dementia and family history were tested. All laboratory tests including MRI had been normal so far. Genomic DNA was extracted from their WBC, and PCR was done on Huntington gene locus using primers modified from HD Collaboratory Group. Agarose gel electrophoresis to examine the rough degree of expansion, polyacrylamide gel electrophoresis to determine repeat length, and sequencing of the expanded allele were done. As a second step, three choreic patients without family history, one patient with tardive dyskinesia and one whole HD family were tested. RESULTS: Three choreic patients with family history showed expansion of CAG repeats in the amplified site. Two sporadic choreic patients, and one asymptomatic member in a HD family had increased CAG repeats. CONCLUSION: We confirmed expansion of CAG repeats in Huntington gene locus in clinically diagnosed HD. None of the patients had caudate atrophy, which has been considered an early finding. Sporadic choreic patients could be diagnosed as HD by gene study. Presymptomatic case was found in a family screening, and will need to be followed. Gene analysis offers a critical tool to make a confirmatory diagnosis of HD, and will be a powerful tool in genetic counseling.
Alleles ; Atrophy ; Chorea ; Dementia ; Diagnosis ; DNA ; Dyskinesias ; Electrophoresis, Agar Gel ; Electrophoresis, Polyacrylamide Gel ; Genetic Counseling ; Humans ; Huntington Disease* ; Magnetic Resonance Imaging ; Mass Screening ; Movement Disorders ; Phenotype ; Polymerase Chain Reaction ; Trinucleotide Repeats ; Wills

Although orbital abscess is seen in all age, it is found predominantly in children 16 years of age or less. It may result from orbital trauma, foreign body, inflammation and direct spillage or hematogenous extension of bacteria from sinuses to orbit. The authors experienced a 8-year-old male patient who presented with proptosis, decreased visual acuity, and limitation of eyeball movement on left eye after injury by soccer ball. Orbital subperiosteal abscess or orbital rhabdomyosarcoma were suspected, and the patient was operated for biopsy and removal of mass. Subperiosteal abscess was found below frontal bone during exploration, and incisional drainage and massive irrigation was done. At postoperative seventh day, visual acuity of the patient was 20/20, proptosis was markedly decreased and eyeball movement was normal. The authors experienced a case of orbital subperiosteal abscess associated with acute paranasal sinusitis after trauma, and a good result was obtained following surgical draining.
Abscess* ; Bacteria ; Biopsy ; Child ; Drainage ; Exophthalmos ; Foreign Bodies ; Frontal Bone ; Humans ; Inflammation ; Male ; Orbit* ; Rhabdomyosarcoma ; Sinusitis* ; Soccer ; Visual Acuity

PURPOSE: To compare high-sensitivity C-reactive protein (hsCRP) levels and lipid profiles between Korean normal tension glaucoma (NTG) patients and healthy controls. METHODS: This cross-sectional study included 38 Korean patients with NTG and 38 age- and sex-matched healthy control subjects. We excluded the patients with cardiovascular risk factors and other systemic diseases that might affect CRP levels and lipid profiles. Each patient underwent a Humphrey visual field examination and blood sampling for hsCRP and lipid profile analyses. Subsequently, the NTG patients were classified into two groups based on their untreated intraocular pressure (IOP) level: low NTG (LNTG) with IOP< or =13 mmHg (13 subjects) and high NTG (HNTG) with relatively high IOP (>13 and < or =21 mmHg, 25 subjects). The hsCRP levels and lipid profiles were compared between NTG patients and healthy controls, and between LNTG, HNTG, and healthy controls. RESULTS: There were no significant differences in hsCRP and lipid profiles between either the NTG patients and healthy controls, or between the LNTG, HNTG, and controls (p>0.05) after exclusion of Korean patients with cardiovascular risk factors. There was no significant association between hsCRP and visual field indices (p>0.05). CONCLUSIONS: High-sensitivity C-reactive protein-related vascular inflammatory conditions may not be directly associated with the development of NTG, regardless of the untreated IOP level.
Adolescent ; Adult ; Aged ; *Asian Continental Ancestry Group ; C-Reactive Protein/*metabolism ; Cross-Sectional Studies ; Female ; Humans ; Lipids/*blood ; Low Tension Glaucoma/*blood ; Male ; Middle Aged ; Young Adult

Between July 1988 and December 1992, we treated 45 patients who had deep seated inoperable or residual and/or recurrent intracranial tumors using LINAC based stereotactic radiosurgery at the Department of Therapeutic Radiology, Kangnam St. Mary's Hospital, Catholic University Medical College. Treated intracranial tumors included pituitary tumors(n=15), acoustic neurinomas(n=8), meningiomas(n=7), gliomas(n=6), craniopharyngiomas(n=4), pinealomas(n=3), hemangioblastomas(n=2), and solitary metastatic tumor from lung cancer (n=1). The dimension of treatment field varied from 0.23 to 42.88 cm3(mean ; 7.26 cm3). The maximum tumor doses ranging from 5 to 35.5 Gy (mean; 29.9 Gy) were given, and depended on patients' age, target volume, location of lesion and previous history of irradiation. There were 22 male and 23 female patients. The age was varied from 5 to 74 years of age(a median age; 43 years). The mean duration of follow-up was 35 months (2~55 months). To date, 18(35.1%) of 46 intracranial tumors treated with SRS showed absent or decrease of the tumor by serial follow-up CT and/or MRI and 16(34.8%) were stationary, e.g. growth arrest. From the view point of the clinical aspects, 34(73.9%) of 46 tumors were considered improved status, that is, alive with no evidence of active tumor and 8(17.4%) of them were stable, alive with disease but no deterioration as compared with before SRS. Although there showed slight increase of the tumor in size according to follow-up imagings of 4 cases(pituitary tumor 1, acoustic neurinomas 2, pinealoma 1), they still represented clinically stable status. Clinically, two(4.4%) patients who were anaplastic astrocytoma(n=1) and metastatic brain tumor(n=1) were worsened following SRS treatment. So far, no serious complications were found after treatment. The minor degree headache which could be relieved by steroid or analgesics and transient focal hair loss were observed in a few cases. There should be meticulous long term follow-up in all cases.
Acoustics ; Analgesics ; Brain ; Craniopharyngioma ; Female ; Follow-Up Studies ; Glioma ; Hair ; Headache ; Hemangioblastoma ; Humans ; Lung Neoplasms ; Magnetic Resonance Imaging ; Male ; Meningioma ; Neuroma, Acoustic ; Pinealoma ; Pituitary Neoplasms ; Radiation Oncology ; Radiosurgery*

This study demonstrates that the nonnarcotic analgesic Zomepirac sodium administered in repeated doses is well tolerated and effective in 42 patients with mild to severe chronic pain. When the relative incidence of side effects such as gastrointestinal up set and central nervous system disturbances are taken into account, Zomepirac is clearly the preferable therapeutic agent. We therefore, suggest that Zomepirac is an acceptable nonnarcotic agent for the management of chronic pain and that its efficacy and safety profile are consistent with current postsurgical managements including early ambulation.
Central Nervous System ; Chronic Pain* ; Early Ambulation ; Humans ; Incidence ; Sodium

PURPOSE: To investigate the relationship between blood pressure (BP) parameters in the habitual position and glaucomatous damage at initial presentation in patients with untreated normal tension glaucoma (NTG). METHODS: Fifty-four eyes from 54 subjects diagnosed with NTG were consecutively enrolled. BP was measured with an automated ambulatory monitoring device in the habitual position during 24-hour in-hospitalization. Patients were classified into three groups: non-dippers, dippers, and over-dippers. corresponded to the degree of reduction in their nocturnal mean arterial pressure (MAP) compared with their diurnal MAP. Regression models were used to evaluate potential risk factors, including: age, pre-admission office intraocular pressure (IOP), central corneal thickness (CCT), and BP parameters. Functional outcome variables for glaucomatous damage included mean deviation (MD) and pattern standard deviation (PSD) on a Humphrey field analyzer (HFA). Anatomic outcome variables were TSNIT score (temporal, superior, nasal, inferior, and temporal) average, superior average, inferior average, and nerve fiber indicator (NFI) score on scanning laser polarimetry with variable corneal compensation (SLP-VCC; GDx-VCC). RESULTS: Marked systolic blood pressure (SBP), diastolic blood pressure (DBP), and MAP fluctuation were noted in the over-dipper group (p<0.05). A linear regression analysis model revealed that nocturnal trough DBP and MAP, average nocturnal SBP, and MAP were all significantly associated with a decreased average TSNIT score and an increased NFI score. CONCLUSIONS: Nocturnal BP reduction estimated in the habitual position was associated with structural damage in eyes with NTG. This finding may suggest systemic vascular etiology of NTG development associated with nocturnal BP reduction.
Adult ; Aged ; Aged, 80 and over ; Blood Pressure/*physiology ; Blood Pressure Monitoring, Ambulatory/*methods ; Circadian Rhythm/*physiology ; Disease Progression ; Female ; Follow-Up Studies ; Glaucoma, Open-Angle/diagnosis/*physiopathology ; Humans ; Intraocular Pressure/physiology ; Male ; Middle Aged ; Nerve Fibers/pathology ; Posture/*physiology ; Prognosis ; Prospective Studies ; Retina/pathology ; Risk Factors ; Visual Fields

PURPOSE: To investigate the visual field (VF) and retinal nerve fiber layer (RNFL) status of the fellow eyes in patients with unilateral retinal vein occlusion (RVO). METHODS: Fifty patients with unilateral RVO and 35 normal control subjects wereconsecutively recruited. Humphrey VF parameters and RNFL status using scanning laser polarimetry with variable corneal compensation (GDx-VCC) were compared between the fellow eyes of the patients with unilateral RVO and control eyes. We also assessed the risk factors for the development of glaucomatous damage in the fellow eyes of unilateral RVO patients. RESULTS: Twelve fellow eyes out of 50 patients with unilateral RVO showed glaucomatous VF and RNFL changes assessed by GDx-VCC. VF indices and RNFL thickness parameters in the study group were significantly lower than those in the control group (p<0.05). Increased age and vertical cup-to-disc ratio were significantly associated with severity of VF and RNFL damage in the fellow eye of unilateral RVO patients (p<0.05). CONCLUSIONS: The fellow eyes in patients with unilateral RVO showed significantly worse VF indices and lower RNFL thickness than normal control eyes. The glaucomatous change should be carefully monitored in the fellow eyes of unilateral RVO patients.
Compensation and Redress ; Eye ; Glaucoma ; Humans ; Nerve Fibers ; Retinal Vein ; Retinal Vein Occlusion ; Retinaldehyde ; Risk Factors ; Scanning Laser Polarimetry ; Visual Fields

In order to elucidate the mechanism of neuronal cell death induced by retina ischemia and reperfusion, we investigated expression of p53, Bcl-2, and ICE(interleukin converting enzyme) in retinal neuronal cell death. Adult male Spague-Dawley rats were used and their intraocular pressures were maintained between 160 and 180mmHg for 90 min to induce retina ischemia. Retinal cell death was observed by light microscopy from 4 hours and peaked at 24 hours after retinal ischemia. By the 3rd day after retinal ischemia, the number of cells in GCL was decreased markedly and some cells in GCL and INL were spread out in inner plexiform layer(IPL). Finally, the boundary between GCL and INL became obscure and a few alive cells were found in GCL and INL on 7 days after retinal ischemia. The thickness of the retina was also decreased in a time-dependent manner. IN the study of gene expression in retinal ischemil, p53 expression was increased prominently at 24 hours and 3 days and decreased at 7 days, while Bcl02 expression was increased slightly at 24 hours by RT-PCR and in situ hybridization. ICE expression was not changed in this model. The expression of p53 was also observed at 24 hours after retinal ischemia by immunohistochemistry. With these results it was found that cell death was increased from 4 hours to 3 days after retina ischemia and the thickness of retina decreased markedly during the same period. Delayed neuronal cell death in retina seemed to be correlated with the expression of p53 in the retina ischemia model.
Adult ; Animals ; Cell Death* ; Gene Expression ; Humans ; Ice ; Immunohistochemistry ; In Situ Hybridization ; Intraocular Pressure ; Ischemia* ; Male ; Microscopy ; Neurons* ; Rats ; Reperfusion* ; Retina* ; Retinal Neurons ; Retinaldehyde

The loss of neuronal cells in the central nervous system may occur in many neurodegenerative diseases. Alzheimer's disease is a common senile disease in people over 65 years, and it causes impairment characterized by the decline of mental function, including memory loss and cognitive impairment, and affects the quality of life of patients. However, the current therapeutic strategies against AD are only to relieve symptoms, but not to cure it. Because there are only a few therapeutic strategies against Alzheimer's disease, we need to understand the pathogenesis of this disease. Cell therapy may be a powerful tool for the treatment of Alzheimer's disease. This review will discuss the characteristics of Alzheimer's disease and various available therapeutic strategies.
Alzheimer Disease* ; Cell- and Tissue-Based Therapy ; Central Nervous System ; Humans ; Memory Disorders ; Neurodegenerative Diseases ; Neurons ; Quality of Life ; Stem Cells* ; Transplantation