Alzheimer's disease(AD) is associated with a characteristic neuropathology. The major hallmarks of AD are senile plaques(SPs) and neurofibrillary tangles(NFTs). beta-amyloid protein(Abeta) is derived from the proteolysis of amyloid precursor protein(APP) and then converted to SPs. Mature SPs produce cytotoxicity through direct toxic effects and activation of microglia and complement. NFTs are composed of paired helical filaments(PHFs) including abnormally phosphorylated form of the microtubule-associated protein(MAP) tau and increased tau level in cerebrospinal fluid may be observed in most AD. The aggregation of Abeta and tau formation are thought to be a final common pathway of AD. Acetycholine, dopamine, serotonin, GABA and their receptors are associated with AD. Especially, decreased nicotinic acetylcholine receptors(nAChRs) in AD are reported. Genetic lesions associated with AD are mutations in the structural genes for the APP located on chromosome 21, presenilin(PSN)1 located on chromosome 14 and PSN2 located on chromosome 1. Also, trisomy 21, Apo-E gene located on chromosome 19, PMF locus, low density lipoprotein receptor-related protein and alpha-macroglobulin increase risk of AD. In this article, we will review about the neurobioloby of AD and some newly developed research areas.
Acetylcholine ; Alzheimer Disease* ; Amyloid ; Amyloid beta-Peptides ; Apolipoproteins E ; Cerebrospinal Fluid ; Chromosomes, Human, Pair 1 ; Chromosomes, Human, Pair 14 ; Chromosomes, Human, Pair 19 ; Chromosomes, Human, Pair 21 ; Complement System Proteins ; Dopamine ; Down Syndrome ; gamma-Aminobutyric Acid ; Genetics ; Lipoproteins ; Microglia ; Neurobiology* ; Proteolysis ; Serotonin

PURPOSE: To evaluate the long-term therapeutic effects of intravitreal bevacizumab on myopic choroidal neovascularization (CNV). METHODS: Medical records of 6 patients who underwent intravitreal bevacizumab injection for myopic CNV and were followed for more than 2 years, were retrospectively investigated. The best corrected visual acuity was compared at 1,3,12, and 24 months after injection. Two years after the injection, a fluorescein angiography and optical coherence tomography (OCT) were performed to evaluate the central macular thickness and leakage of CNV. RESULTS: The mean best corrected visual acuity was 1.16 +/- 0.43 (logMAR), 0.45 +/- 0.21 (logMAR), 0.29 +/- 0.23 (logMAR), 0.14 +/- 0.11 (logMAR), and 0.11 +/- 0.06 (logMAR) at baseline, 1, 3, 12, and 24 months after injection, respectively. CONCLUSIONS: Intravitreal bevacizumab injection for the treatment of myopic CNV was effective in maintaining postoperative visual acuity for 2 years.
Antibodies, Monoclonal, Humanized ; Choroid ; Choroidal Neovascularization ; Fluorescein Angiography ; Humans ; Intravitreal Injections ; Medical Records ; Retrospective Studies ; Tomography, Optical Coherence ; Visual Acuity ; Bevacizumab

No abstract available.
Escherichia coli* ; Escherichia* ; Humans*

No abstract available.
Methotrexate* ; Skin*

PURPOSE: The Pediatric Risk of Mortality(PRISM) III score was developed from the Physiologic Stability Index(PSI) to assess pediatric ICU mortality and Provide an objective data as a severity index. Although the PRISM score has been applied to many comparisions and analyses in previous studies, there are few reports applied to pediatric intensive care patients in Korea. To evaluate the effectiveness of the PRISM III score as a severity index for expecting mortality and find important variables influencing mortality, we applied this scoring scale to pediatric neurologic patients admitted to the ICU and analyzed the data statistically. METHODS: Data collection was done by careful review of medical records and scored each clinical variable. The outcome at discharge was determined as non-survival, survival, and hopeless discharge. Determination of mortality in the hopeless discharge group was done within 48 hours after discharge by telephone interview. The study populations were classified into four groups; CNS infection(26 patients), acute encephalopathy(31 patients), status epilepticus(35 patients) and cerebrovascular disorder(4 patients). The difference of the PRISM III score between the survival group and non-survival group was compared by using the nonparametric Mann~Whitney test in the entire study population and for each diagnostic group. To confirm the degree of fitness between the actual mortality and Predicted mortality, the Hosmer-Lemeshow goodness-of-fit test, a multiple logistic regression model was used. All clinical variables used for scoring were compared for survivals and non-survivals by the Chi-square test. f values <0.05 were considered significant. RESULTS: The PRISM III score was significantly higher in the non-survival groups than in the survival group. Predicted mortality from the PRISM III score has fitted to actual mortality According to the results of analyses in each diagnostic groups, the PRISM III score was higher in non-survivals of the acute encephalopathy and CNS infection groups, but statistically insignificant in the cerebrovascular disorders and status epilepticus groups. The important variables of the PRISM III score associated with mortality were mental state, Pupil reflex, systolic blood pressure, acidosis, blood sodium level blood creatinine level, blood glucose level, and PT/PTT. , CONCLUSION: The PRISM III score is helpful in predicting mortality in pediatric intensive care neurologic patients, especially those in the acute encephalopathy or the CNS infection groups. However, this score was not useful in the status epilepticus group, and insignificant in cerebrovascular group. Due to the smallness of the study group, more massive and comprehensive studies are needed as a follow up to this study.
Acidosis ; Blood Glucose ; Blood Pressure ; Cerebrovascular Disorders ; Creatinine ; Data Collection ; Follow-Up Studies ; Humans ; Critical Care* ; Interviews as Topic ; Korea ; Logistic Models ; Medical Records ; Mortality* ; Pupil ; Reflex ; Sodium ; Status Epilepticus

OBJECTIVE: The objectives of this study were to evaluate the reliability of the Korean version of Spinal Cord Independence Measure (SCIM) and to compare the sensitivity of the SCIM to functional changes of spinal cord injury (SCI) patients with that of the Functional Independence Measure (FIM). METHOD: Seventeen subjects with SCI were studied. The SCIM was translated and modified to convert as SCIM Korean-version. All patients were evaluated with the SCIM and the FIM by two raters every other week. To determine inter-rater reliability, the relationship between the SCIM scores obtained by two raters was evaluated by Kappa coefficient and linear regression. To determine relative sensitivity of the test to functional changes, changes in the scores on the SCIM and FIM were compared by McNemar test. RESULTS: The Kappa coefficient of the various individual tasks in SCIM ranged between 0.63 and 1.00. High correlations were also found between the total SCIM scores for the paired raters (r=0.99, p<0.01). The SCIM detected all the functional changes detected by FIM total scoring, but in 3 (14%) of 22 sequential test batteries, the FIM missed changes detected by SCIM total scoring. CONCLUSION: These results demonstrated that the SCIM is reliable and more sensitive than the FIM in reflecting the functional changes of SCI patients.
Humans ; Linear Models ; Spinal Cord Injuries* ; Spinal Cord*

The association of varicose vein, soft tissue and bony hypertrophy and cutaneous hemangi-oma of the port-wine variety confined to one extremity was first reported in 1900 by Klippel and Trenaunay, and then Weber. A case of Klippel-Trenaunay-Weber Syndrome with macrodactylia in a 2 months old male infant(dizygotic) is presented who was asmitted to B.N.U.H. with chief complaints of nevus fla-mmeus, swelling of the left lower extremity.
Extremities ; Humans ; Hypertrophy ; Infant ; Klippel-Trenaunay-Weber Syndrome* ; Lower Extremity ; Male ; Nevus ; Port-Wine Stain ; Varicose Veins

This is a case report of the familial olivo-ponto-cerebellar atrophy (Menzel type OPCA, OPCA III with retinal degeneration). The patient is a 37 year-old male with 5 years history of slowly progressive cerebellar ataxia, dysarthria, visual change, horizontal nystagmus and signs of pyramidal dysfunction. The CT brain scan shows significant atrophy of brain stem and cerebellum with dilatation of cisterns. His younger sister, also, has similar clinical manifestations and radiological abnormalities, but mild. By history, his mother who died at 47 years of her age looked like to have same kind of chronic disease.
Adult ; Atrophy ; Brain ; Brain Stem ; Cerebellar Ataxia ; Cerebellum ; Chronic Disease ; Dilatation ; Dysarthria ; Humans ; Male ; Mothers ; Nystagmus, Pathologic ; Olivopontocerebellar Atrophies* ; Retinaldehyde* ; Siblings

PURPOSE: To report three consecutive cases of methazolamide-induced Stevens-Johnson syndrome. CASE SUMMARY: We describe three patients who were all prescribed methazolamide for treatment of ophthalmologic conditions. A 29-year-old man and a 47- year-old woman were prescribed methazolamide (100 mg/day) for the treatment of central serous chorioretinopathy (CSCR). A 66-year-old woman was prescribed methazolamide (100 mg/day) for acute glaucoma of the left eye for approximately two weeks. After taking the methazolamide, three patients were showed the pururitic maculopapular rashes on the whole body and the vesicular eruptions of the oral mucosa and conjunctiva. On the basis of medication histories, characteristic skin lesions and mucosal involvement, we diagnosed all three patients with methazolamide-induced Stevens-Johnson syndrome. All three patients were hospitalized and treated with intravenous steroids and antihistamines. Two of the three cases showed conjunctival pseudomembranes. In two cases, the skin lesions worsened during the first week of treatment, and then resolved without complications over the next two to three weeks. The condition of the 47-year-old female patient deteriorated rapidly to toxic epidermal necrolysis due to sensitivity to sulfa antibiotics. HLA- A24, B59 and Cw1 were detected in all three cases. CONCLUSIONS: In 2008, domestic production of acetazolamide was halted in Korea. Because of this, methazolamide is expected to be prescribed by ophthalmologists more commonly than in previous years. Complete medical histories should be taken before prescribing methazolamide to patients. HLA typing should be conducted whenever possible to screen patients before prescription of methazolamide.
Acetazolamide ; Adult ; Aged ; Anti-Bacterial Agents ; Central Serous Chorioretinopathy ; Conjunctiva ; Epidermal Necrolysis, Toxic ; Exanthema ; Eye ; Female ; Glaucoma ; Histamine Antagonists ; Histocompatibility Testing ; HLA-B Antigens ; Humans ; Korea ; Methazolamide ; Middle Aged ; Mouth Mucosa ; Prescriptions ; Skin ; Steroids ; Stevens-Johnson Syndrome

Spinal meningiomas located purely in the extradural space are rare, and they may easily be confused with malignant neoplasm. We report an unusual case of a purely extradural spinal meningioma mimcking metestatic neoplasm. A 38-year-old woman had neck pain and left side weakness. MRI scan revealed extradural spinal mass. Preoperative and intraoperative diagnosis was metastatic carcinoma, but permanent diagnosis was extradural meningioma.
Adult ; Diagnosis ; Female ; Humans ; Magnetic Resonance Imaging ; Meningioma* ; Neck Pain ; Spine*