Spinocerebellar ataxia type-2 (SCA2) is an autosomal dominant cerebellar ataxia that occurs due to expanded CAG trinucleotide repeats in the ATXN2 gene. Clinical features of parkinsonism in SCA2 vary from phenotypes of levodopa-responsive parkinsonism to multiple system atrophy. We described a patient with SCA2 presenting typical clinical manifestations of multiple system atrophy-c type with levodopa responsive parkinsonism whose dopamine transporter (DAT) image showed atypically reduced DAT uptake in in the striatum.

PURPOSE: This study was conducted to investigate the prognostic factors of postpsychotic depressive symptoms in patients with schizophrenia. MATERIALS AND METHODS: Eighty patients were selected based on the diagnostic criteria from the DSM-IV, PANSS and ESRS. For all patients information was collected on demographic and clinical characteristics. The subjective depressive symptoms and the objective depressive symptoms, as well as patients insight regarding psychosis were evaluated. The subjective depressive symptoms were evaluated by BDI and ZDS; the objective depressive symptoms were evaluated by HDRS and CDSS, and patient insight into the psychosis was evaluated by KISP. RESULTS: The comparisons using demographic and clinical characteristics showed that HDRS and CDSS had significant difference with regard to gender and suicide attempts; the BDI was associated with difference in education level and age of onset. The patients with scores above cuff-off score for each scale were 20 (25.0%) for the BDI, 16 (20.0%) for the ZDS, 18 (22.5%) for the CDSS and 6 (7.5%) for the HDRS. The results of the stepwise multiple regression analysis showed that the scores for the KISP, education levels, gender and suicide attempts were the main prognostic factors in patients with the psychotic depressive disorder of schizophrenia. CONCLUSION: The main prognostic factors in psychotic depressive disorder of schizophrenia included: insight into psychosis, suicidal attempts. Insight into the psychosis was the most reliable prognostic factor but this characteristic had a negative relationship to the with depressive symptoms.
Age of Onset ; Depression ; Depressive Disorder* ; Diagnostic and Statistical Manual of Mental Disorders ; Education ; Humans ; Prognosis ; Psychotic Disorders ; Schizophrenia* ; Suicide

BACKGROUND: Hepatitis B virus (HBV) is major source of chronic liver disease in Korea. However this virus might have different nucleotide sequence according to races, different region, etc. Recently the novel method that allows sensitive amplification with dramatically decreased mis-incorporation has developed. We determined to get the major form of HBV nucleotide sequence from whole sequencing data of 26 Korean HBV carriers. METHODS: HBV DNA were collected from 26 Korean chronic HBV carriers. We used the novel PCR with pfu for the amplification of HBV DNA, and specific primers were made with combination sequence bases of non-HBV part and HBV parts which were located head and tail in the virion. Then whole length of HBV were directly sequenced and analysed. RESULT: HBV DNA was consisted of 3215 bases in 20 cases of 26 Korean chronic HBV carriers. And the remainder had smaller or larger number due to deletion, insertion or both in pre-S2 and S gene. They were 99.03% homology of their nucleotide sequence and belong to genotype C. The variability of nucleotide sequence was significantly higher in the singly coding region (SCR) than doubly coding region (DCR), and also high in pre-S1 and pre-S2 gene among the DCR. Hot-spots were more frequently found in the SCR, pre-S1 and pre-S2 gene. CONCLUSION: In Korean chronic HBV carriers, HBV is consisted of 3215 nucleotides, and belongs to genotype C. And it might exist one genotype with the variability in Korea.
Base Sequence* ; Clinical Coding ; Continental Population Groups ; DNA ; Genome* ; Genotype* ; Head ; Hepatitis B virus* ; Hepatitis B* ; Hepatitis* ; Humans ; Korea ; Liver Diseases ; Nucleotides ; Polymerase Chain Reaction ; Polymerization* ; Polymers* ; Virion

Henoch-Scholein Purpura(HSP) is a syndrome characterized by nonthrombocytopenic purpura accompanied by a arthralgia, gastrointestinal symptom, or renal involvement. This report describes a 13-year-old boy with hemorrhaic bullous HSP followed by cellulitis as a complication. This unusual cutaneous manifestation of HSP may be a source of diagnostic confusion, but it does not appear to have prognostic implication.
Adolescent ; Arthralgia ; Cellulitis* ; Humans ; Male ; Purpura* ; Purpura, Schoenlein-Henoch

Lymphoepithelioma-like carcinonma of the skin(LELCS) is a rare tumor with a microscopic resemblance to lymphoepitheliomatous tumors of the nasopharynx. Less than 100 examples of LELCS are having been reported since its initial description in 1988. Elderly patient are preferentially affected, and the skin of the head and neck is the principal location for this neoplasm. We report a 70 year-old male patient who had brownish colored, moderately firm nodule on the left malar area of the face. Histopathologically, the nodule showed features of LELCS and he was treated with excision and local flap coverage.
Aged ; Head ; Humans ; Male ; Nasopharynx ; Neck ; Skin*

BACKGROUND: Bronchial asthma is characterized by a reversible airway obstruction, airway hyperresponsiveness, and eosinophilic airway inflammation. The bronchodilator response(BDR) after short acting beta agonist inhalation and PC20 with methacholine inhalation are frequently used for diagnosing bronchial asthma, However, the relationship between the presence of a bronchodilator response and the degree of airway hyperresponsiveness is uncertain. Therefore, the availability of a eosinophil cationic protein(ECP) and a correlation ECP with a bronchodilator response and airway hyperresposiveness was investigated. METHOD: A total 71 patients with a moderate to severe degree of bronchial asthma were enrolled and divided into two groups. 31 patients with a positive bronchodilator response and 38 patients with a negative bronchodilator response were evaluated. In both groups, the serum ECP, peripheral blood eosinophil counts, and total IgE level were measured and the methacholine bronchial provocation test was examined. RESULTS: There were no differences observed in age, sex, atopy, and baseline spirometry in both groups. The peripheral eosinophil counts showed no difference in both groups, but the ECP level in group 1 (bronchodilator responder group) was higher than in group 2(non-bronchodilator responder group) (22.4±20.7 vs 14.2±10.4, mean±SD). The PC20 in group 1 was significantly lower than in group 2 (1.14±1.68 vs 66±2.98). There was a significant positive correlation between the BDR and ECP, and a negative correlation between the bronchial hyperresponsiveness and ECP. CONCLUSION: The bronchodilator response significantly correlated with the bronchial hyperresponsiveness and serum ECP in the moderate to severe asthma patients. Hence, the positive bronchodilator response is probably related with active bronchial inflammation and may be used as a valuable index in treatment, course and prognosis of bronchial asthma.
Airway Obstruction ; Asthma ; Bronchial Provocation Tests ; Eosinophil Cationic Protein* ; Eosinophils ; Humans ; Immunoglobulin E ; Inflammation ; Inhalation ; Methacholine Chloride ; Prognosis ; Spirometry

Anticonvulsant hypersensitivity syndrome(AHS) is an uncommon, but potentially fatal, multi-systemic disorder that occurs after exposure to phenytoin, carbamazepine, phenobarbital. Clinical features and laboratory data are diverse and variable. The multi-systemic reaction presents as fever, skin eruptions, lymphadenopathy, hematologic abnormality, and hepatitis. It is postulated that this mechanism can cause deficient enzymatic reduction by epoxide hydrolase. The diagnosis of AHS is made by reviewing the history of drug exposure and clinical course. It is important to discontinue use of the offending drug suspected for AHS and to closely observe patients with anticonvulsant therapy. We experienced a case of anticonvulsant hypersensitivity syndrome developed by carbama-zepine, presented with pseudolymphoma in lymph node biopsy and improved by discontinuing the drug and implementing steroid treatment. We report this case with pathologic findings and a brief review.
Biopsy ; Carbamazepine* ; Diagnosis ; Fever ; Hepatitis ; Humans ; Hypersensitivity* ; Lymph Nodes ; Lymphatic Diseases ; Phenobarbital ; Phenytoin ; Pseudolymphoma* ; Skin

This study was carried out to investigate the supplementary effects of Suwon 464, which has over two times of dietary fiber content compared with normal rice (Ilpum), on blood glucose in diabetic mice. We supplied 5 kinds of experimental diets (corn starch diet as a control (CO), Ilpum polished rice diet (IP), Ilpum brown rice diet (IB), polished rice diet (SP) and brown rice diet (SB) of Suwon 464) to diabetic mice for 8 weeks, after analyzing dietary fiber contents of 5 experimental diets. Diet intake, body weight and contents of blood glucose, hemoglobin A(1c) and insulin were measured. The dietary fiber contents in CO, IP, IB, SP, and SB diets were 1.0, 1.2, 1.4, 1.4, and 2.0% respectively. Body weight was lower in SB group than the other groups though there was no significant difference in diet intake among experimental groups. The concentration of blood glucose in diabetic mice was lower in SB group than the other groups during the supplementary period of experimental diets. The hemoglobin A(1c) and serum insulin levels were lower in SP and SB groups. These results suggested that the brown rice of Suwon 464 with high dietary fiber can control diabetes in diabetic mice by reducing the blood glucose and hemoglobin A(1c).
Animals ; Blood Glucose* ; Body Weight ; Diet ; Dietary Fiber* ; Gyeonggi-do ; Insulin ; Mice* ; Starch

PURPOSE: Histone deacetylase inhibitors (HDACIs) induce accumulation of acetylated histones in nucleosomes, which lead to reactivate gene expression and inhibit the growth and survival of tumor cells. This study evaluated the efficacy of HDACIs in breast cancer cells in comparison with other established drug regimens. METHODS: Drug responses of tumor samples from mastectomy specimens of 78 breast cancer patients were evaluated using the histoculture drug response assay (HDRA). Tumor inhibition rates (IRs) of established drug regimens such as doxorubicin, cyclophosphamide, doxorubicin with cyclophosphamide (AC), paclitaxel, docetaxel and doxorubicin with docetaxel (AT), as well as those of three HDACIs (SAHA, PXD101, and a novel compound CG-2) were evaluate. RESULTS: The percentages of chemosensitive tumors (chemoresponsiveness) were 26.9-60.3% with established regimens and 61.5-73.1% with HDACIs when the cutoff value for inhibition rate was set at 30%. Breast cancer cells appeared to be more chemoresponsive to HDACIs than to established drug regimens. Chemoresponsiveness to AT was the highest among the established drug regimens. A combination regimen offered higher activity than did a single drug (doxorubicin vs AT; p<0.001). HER2/Neu-overexpressing breast cancers were chemosensitive to SAHA and AT (p=0.031 and 0.04, respectively). CONCLUSION: Our findings show that breast cancer cells were sensitive to HDACIs, with therapeutic efficacies comparable to those of established drug regimens. Specific biological markers such as HER2/Neu could be assessed for effectiveness as HDACIs chemosensitivity markers in further clinical trials.
Biomarkers ; Breast ; Breast Neoplasms ; Cyclophosphamide ; Doxorubicin ; Gene Expression ; Histone Deacetylase Inhibitors ; Histone Deacetylases ; Histones ; Humans ; Hydroxamic Acids ; Mastectomy ; Nucleosomes ; Paclitaxel ; Sulfonamides ; Taxoids

Patients who have complex endocarditis with involvement of both the aortic and mitral valves and intervalvular fibrous skeleton are among the most difficult to treat and still have the highest surgical mortality and morbidity rates. We report one case of aortic and mitral valve replacement with reconstruction of the fibrous skeleton performed in a 55-year-old female patient who had an aortic annular abscess and both the aortic and mitral prosthetic valve endocarditis with destruction of the fibrous skeleton. Previously, she had undergone redo double valve replacement. Transesophageal echocardiogram showed the paravalvular defect at the noncoronary aortic sinus and abnormal sinus tract along the fibrous skeleton. Emergent operation was performed due to positive blood cultures of staphylococcus epidermidis and persistent sepsis despite appropriate antibiotic therapy. After aortotomy extended to the roof of left atrium, both prosthetic valves and destroyed fibrous skeleton were completely resected and the aortic annular abscess was debrided and closed with a bovine pericardial patch. Reconstructions of both aortic and mitral annuli and the fibrous skeleton were done by using two separate bovine pericardial patches in triangular shape and mechanical valves were implanted. Postoperatively, adequate antibiotic therapies were continued and the patient was discharged at the postoperative 72 days without evidence of recurrence of endocarditis. Transthoracic echocardiogram of the postoperative 8 months shows no paravalvular leakage or recurrence of endocarditis and the patient has been followed up with no symptom.
Abscess ; Endocarditis* ; Female ; Heart Atria ; Humans ; Middle Aged ; Mitral Valve* ; Mortality ; Recurrence ; Sepsis ; Sinus of Valsalva ; Skeleton* ; Staphylococcus epidermidis