No abstract available.
Korea* ; Moyamoya Disease*

No abstract available.
Stevens-Johnson Syndrome*

No abstract available.
Kasabach-Merritt Syndrome*

Anti-N-methyl D-aspartate receptor (anti-NMDAR) encephalitis, recently recognized as a form of paraneoplastic encephalitis, is characterized by a prodromal phase of unspecific illness with fever that resembles a viral disease. The prodromal phase is followed by seizures, disturbed consciousness, psychiatric features, prominent abnormal movements, and autonomic imbalance. Here, we report a case of anti-NMDAR encephalitis with initial symptoms of epilepsia partialis continua in the absence of tumor. Briefly, a 3-year-old girl was admitted to the hospital due to right-sided, complex partial seizures without preceding febrile illness. The seizures evolved into epilepsia partialis continua and were accompanied by epileptiform discharges from the left frontal area. Three weeks after admission, the patient's seizures were reduced with antiepileptic drugs; however, she developed sleep disturbances, cognitive decline, noticeable oro-lingual-facial dyskinesia, and choreoathetoid movements. Anti-NMDAR encephalitis was confirmed by positive detection of NMDAR antibodies in the patient's serum and cerebrospinal fluid, and her condition slowly improved with immunoglobulin, methylprednisolone, and rituximab. At present, the patient is no longer taking multiple antiepileptic or antihypertensive drugs. Moreover, the patient showed gradual improvement of motor and cognitive function. This case serves as an example that a diagnosis of anti-NMDAR encephalitis should be considered when children with uncontrolled seizures develop dyskinesias without evidence of malignant tumor. In these cases, aggressive immunotherapies are needed to improve the outcome of anti-NMDAR encephalitis.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis* ; Antibodies ; Anticonvulsants ; Antihypertensive Agents ; Cerebrospinal Fluid ; Child* ; Child, Preschool ; Cognition ; Consciousness ; D-Aspartic Acid ; Diagnosis ; Dyskinesias ; Encephalitis ; Epilepsia Partialis Continua* ; Female ; Fever ; Humans ; Immunoglobulins ; Immunotherapy ; Korea* ; Methylprednisolone ; Rituximab ; Seizures ; Virus Diseases

Tibialis anterior muscle hernia is the most common hernia among lower extremity muscles. This condition can be diagnosed by physical examination and radiologic findings, especially by dynamic ultrasonography. There are surgical methods of treatment for muscle hernia, including direct repair, fasciotomy, fascial patch grafting using autologous fascia lata or synthetic mesh. We report a case of tibialis anterior muscle hernia treated with local periosteal rotational flap. Because there are several advantages to the local periosteal rotational flap, such as lack of donor site morbidity, lack of skin irritation, low cost, simplicity, and an easy approach, this technique could be an option for tibialis anterior muscle hernia.
Fascia Lata ; Hernia ; Humans ; Lower Extremity ; Muscle, Skeletal ; Muscles ; Physical Examination ; Skin ; Tissue Donors ; Transplants

Objective: The purpose of this study was to verify the validity and reliability of Korean elderly by translating the Hayling Test, a test that measures semantic inhibition ability among executive functions. Methods: Total 91 participants were recruited in accordance with inclusion criteria and exclusion criteria for each group. Within them, 34 were normal from Dongjak-gu, Seoul, 29 were mild cognitive impairment (MCI) from clinics located in Seoul, and 28were Alzheimer’s disease (AD) from clinics located in Seoul, respectively. The one-way analysis of covariance, the Bonferroni veri-fication, the receiver operating characteristic (ROC) curve analysis, and the Brain Imaging analysis of voxel-based morphometrywere conducted in order to compare and analyze performance of demographics characteristics of each group, Hayling Test results, and The Continuous Performance Test-X (CPTX) results. Results: The comparison analysis showed the Hayling Test is more valid methodology than the CPTX test in discriminating nor-mal, MCI, and AD. Furthermore, ROC curves between normal and AD groups also demonstrated higher sensitivity (88%) and specificity (73%) of Hayling Test, and confirmed its diagnostic validity. In line with the above, brain imaging analysis displays sig-nificant positive correlation between temporal & frontal brain regions through the Hayling Test scores, however, this correlationwas not found for CPTX. Conclusion It is expected that the Haling Test, which measures language inhibition function, can be used simply and usefully in diagnosing dementia.

OBJECTIVES: Klinefelter syndrome is the most common genetic cause of male infertility and presents with 47, XXY mainly or 46, XX/47, XXY mosaicism. It is characterized by hypogonadism and azoospermia due to testicular failure, however, sporadic cases of natural pregnancies have been reported. With the development of testicular sperm extraction (TESE) and intracytoplasmic sperm injection (ICSI), sperm can be retrieved successfully and ART is applied in these patients for pregnancy. It has been suggested that the risk of chromosome aneuploidy for both sex chromosome and autosome is increased in the sperms from 47, XXY germ cells. Considering the risk for chromosomal aneuploidy in the offspring, preimplantation genetic diagnosis (PGD) could be applied as a safe and more effective treatment option in Klinefelter syndrome. The aim of this study is to assess the outcome of PGD cycles by using FISH for sex chromosome and autosome in patients with Klinefelter syndrome. MATERIALS AND METHODS: From Jan. 2001 to Dec. 2003, PGD was attempted in 8 cases of Klinefelter syndrome but TESE was failed to retrieve sperm in the 3 cases, therefore PGD was performed in 8 cycles of 5 cases (four 47, XXY and one 46, XY/47, XXY mosaicism). In one case, ejaculated sperm was used and in 4 cases, TESE sperm was used for ICSI. After fertilization, blastomere biopsy was performed in 6~10 cell stage embryo and the chromosome aneuploidy was diagnosed by using FISH with CEP probes for chromosome X, Y and 17 or 18. RESULTS: A total of 127 oocytes were retrieved and ICSI was performed in 113 mature oocytes. The fertilization rate was 65.3+/-6.0% (mean+/-SEM) and 76 embryos were obtained. Blastomere biopsy was performed in 61 developing embryos and FISH analysis was successful in 95.1% of the biopsied blastomeres (58/61). The rate of balanced embryos for chromosome X, Y and 17 or 18 was 39.7+/-6.9%. The rate of aneuploidy for sex chromosome (X and Y) was 45.9+/-5.3% and 43.2+/-5.8% for chromosome 17 or 18, respectively. Embryo transfer was performed in all 8 cycles and mean number of transferred embryos was 2.5+/-0.5. In 2 cases, clinical pregnancies were obtained and normal 46, XX and 46, XY karyotypes were confirmed by amniocentesis, respectively. Healthy male and female babies were delivered uneventfully at term. CONCLUSION: The patients with Klinefelter syndrome can benefit from ART with TESE and ICSI. Considering the risk of aneuploidy for both sex chromosome and autosome in the sperms and embryos of Klinefelter syndrome, PGD could be offered as safe and more effective treatment option.
Amniocentesis ; Aneuploidy ; Azoospermia ; Biopsy ; Blastomeres ; Chromosomes, Human, Pair 17 ; Embryo Transfer ; Embryonic Structures ; Female ; Fertilization ; Germ Cells ; Humans ; Hypogonadism ; Infertility, Male ; Karyotype ; Klinefelter Syndrome* ; Male ; Mosaicism ; Oocytes ; Pregnancy ; Preimplantation Diagnosis* ; Prostaglandins D ; Sex Chromosomes ; Sperm Injections, Intracytoplasmic ; Spermatozoa

OBJECTIVES: To compare the quality improvement of the abstracts of original articles, according to the revised manuscript format, of the Korean Journal of Preventive Medicine (Korean J Pre Med) was adopted in 1999. METHODS: A total 63 abstracts for 1997, and 49 for 2001, were selected as the original articles from the Kor J Pre Med. This study was carried out by the separate-sample pretest-posttest design. The quality of the abstracts was measured by a checklist of Narine' evaluation criteria, and the other information related to the articles were also surveyed by e-mail and fax or telephone using a self-made questionnaire. From the response rate, a total of 62 abstracts for 1997 and 49 for 2001 were finally analyzed. RESULTS: The mean number of words in an abstract decreased from 285 in 1997, to 250 by 2001. The mean number of key words per abstract decreased from 3.9 in 1997, to 3.6 by 2001. The mean number of inappropriate usage of key words per abstract, by the MeSH standard, decreased from 1.9 in 1997, to 0.4 by 2001. Also, the overall mean score of abstract quality increased from 0.54 in 1997 to 0.61 by 2001. The range of scores for the abstract quality was better in 2001 (0.40~0.77) than in 1997 (0.20~0.81). From the multiple regression analyses of the 1997 and 2001 databases, the intervention of the manuscript format's revision, and the number of English words to the quality score of the abstract, were the only statistically significant factors. CONCLUSIONS: In conclusion, the quality of abstracts in the Kor J Pre Med has improved since the revised manuscript format was adopted in 1999. The Korean Society for Preventive Medicine will continuously recommend proposals for more informative abstracts in their journal, and will evaluate the abstracts' content with quality criteria. Future studies should address these issues, and compare the quality of abstracts between different international and domestic journals.
Checklist ; Electronic Mail ; Preventive Medicine* ; Quality Improvement* ; Telephone

PURPOSE: To assess the applicability of quantitative MR microscopy for the detection of glycosaminoglycan (GAG) depletion as an early sign of degeneration in the articular cartilage of humans treated by trypsin. MATERIALS AND METHODS: Four cartilage-bone blocks were obtained from the patient who had suffered from osteoarthritis of the knee and underwent a total knee replacement arthroscopy. Each articular cartilage segment was resected as to a round disk shape (8 mm in diameter) with a remnant of subchondral bone 1 mm in thickness. Four different culture solutions were prepared, and these solutions were 0.2 mg/ml of trypsin solution (group 1), 1 mM of Gd (DTPA) 2-mixed trypsin solution (group 2), phosphate buffered saline (PBS) (group 3), and 1 mM of Gd (DTPA) 2-mixed PBS (group 4). The cartilages were cultured and then MR imagings were performed every hour for 5 hrs, and we continued the additional cultures of 24 hrs, 36 hrs and 48 hrs. Three imaging sequences were used: T1-weighted spin echo (TR/TE, 450/22), proton density turbo spin echo with fat suppression (TR/TE, 3000/25), and CPMG (Carr-Purcell-Meiboom-Gill) (TR/TE/TI, 760/21-168, 360). MR imaging data were analyzed with pixel-by-pixel comparisons in all groups. RESULTS:The GAG loss in the articular cartilage was increased proportionately to the culture duration. Mean changes of T1 relaxation time were 1.2% for group 1, -1.9% for group 3, -54.7% for group 2 and -64.2% for group 4 (p<0.05). When comparing by linear profile on the T1-weighted images, SNR increased and T1 relaxation time decreased for group 2 and 4, as the culture duration increased (p<0.05). On the correlation analysis, there is significant correlation between GAG loss and Gd (DTPA) 2-enhancement for group 2 (p=0.0431), but there was no significant difference for group 4 (p=0.0918). More enhancement with Gd (DTPA) 2-was noted for group 2 than for group 4. Group 2 showed a diffuse enhancement in all the layers of cartilage, but for group 4, prominent enhancement was noted only in the superficial layer. CONCLUSION: Early degenerative changes of the articular cartilage were not noted on the T1-weighted images, turbo spin echo with fat suppresison and T1, T2, or the rho relaxometry by a 1.5 T machine. Only Gd (DTPA) 2- enhancement was useful in the detection of early degeneration of the articular cartilage.
Arthroplasty, Replacement, Knee ; Arthroscopy ; Cartilage ; Cartilage, Articular* ; Humans* ; Knee ; Magnetic Resonance Imaging* ; Microscopy ; Osteoarthritis ; Protons ; Relaxation ; Trypsin

A previously healthy 52-year-old man presented to the emergency room with acute onset left hemiparesis and dysarthria. Brain computed tomography and magnetic resonance examinations revealed acute cerebral infarction in the right middle cerebral artery territory and a sphenoid ridge meningioma encasing the right carotid artery terminus. Cerebral angiography demonstrated complete occlusion of the right proximal M1 portion. A computed tomography perfusion study showed a wide area of perfusion-diffusion mismatch. Over the ensuing 48 hours, left sided weakness deteriorated despite medical treatment. Emergency extracranial-intracranial bypass was performed using a double-barrel technique, leaving the tumor as it was, and subsequently his neurological function was improved dramatically. We present a rare case of sphenoid ridge meningioma causing acute cerebral infarction as a result of middle cerebral artery compression.
Brain ; Carotid Arteries ; Cerebral Angiography ; Cerebral Infarction* ; Dysarthria ; Emergencies ; Emergency Service, Hospital ; Humans ; Meningioma* ; Middle Aged ; Middle Cerebral Artery ; Paresis ; Perfusion