Alexander disease (AxD) is a progressive neurodegenerative disorder caused by mutations in the gene encoding the glial fibrillary acidic protein (GFAP). Three subtypes of AxD have been identified based on the age of onset: infantile (under age 2), juvenile (age 2 to 12) and adult (over age 12). The adult form is rare and presents with unique clinical features different from those of the infantile forms. Here, we present the first Korean case of adult-onset cerebellar ataxia with typical tadpole-like brainstem atrophy on the magnetic resonance imaging (MRI). Molecular genetic analysis revealed a heterozygous missense mutation (c.1246C>T, p.R416W) in the GFAP gene.

The authors reviewed the clinical findings and treatment results of 12 cases of spinal meningeal cysts which were detected in MRI of low back patients. In these lesions, large cysts without CSF communication can compressed the nerve roots within spinal canal and it is difficult to confirm the cause of symptom whether it is originated from cysts or from associated spinal disorders. The terms and classifications of spinal meningeal cysts were very confusing. Among 12 cases, we excised 3 cases of large cysts with gluteal and perianal pains that were caused by compressed sacral nerve roots. All three cases were type 2 cyst (classified by Nabors) and located in sacral canal. In one case associated with isthmic spondylolisthesis, posterolateral fusion and pedicle screw fixations were combined with cyst excision. In other two cases, there were not any spinal pathologic findings that compressed sacral nerve roots except mild degenerative changes and intervertebral disc herniation in lower lumbar and sacral levels All 3 excised cases showed good prognosis in more than one year follow up. The other cases were treated conservatively for the associated spinal disorders.
Classification ; Follow-Up Studies ; Humans ; Intervertebral Disc ; Magnetic Resonance Imaging ; Prognosis ; Radiculopathy ; Spinal Canal ; Spondylolisthesis

Purpose: This descriptive study investigated the influence of married nurses’ self-efficacy, parenting stress, and organizational culture on their health-promoting behaviors. Methods: Data were collected from 173 married nurses in G and B metropolitan city; the study was conducted from August to September 2019. Data were analyzed using the independent t-test, one-way ANOVA, Pearson correlation coefficient, and multiple regression with SPSS 18.0. Results: The most significant influencing factors on the health-promoting behavior of the subject were self-efficacy (β=.38, p<.001), followed by parenting stress (β=-.21, p=.002), education (β=-.20, p=.001), perceived health status (β=.19, p=.003) and the overall explanatory power was 42.0%. Conclusion Based on the results, an intervention program that can increase their self-efficacy and reduce parenting stress is needed, considering education level of married nurses and their perceived health status to be developed.

OBJECTIVES: The present study aims to investigate the effects of physical illness on depression in North Korean Defectors. METHODS: One hundred forty-four North Korean Defectors(20 males, 124 females) and 376 South Koreans 133 males, 243 females) in Incheon Metropolitan areas participated the present study. Face-to-face interview was conducted for demographic information including presence of physical illnesses. To investigate depressive symptoms, all participants were required to complete the Center for Epidemiologic Studies-Depression scale (CES-D). RESULTS: After controlling gender, age, marital status, educational year, employment status and physical illness, North Korean Defectors showed higher CES-D score than South Koreans(beta=0.449, p<0.001). Within North Korean Defectors, the presence of physical illnesses independently was related to higher CES-D score(beta=0.243, p<0.001). However, within South Koreans, the presence of physical illnesses did not significantly predict CES-D score. North Korean Defectors with physical illness have higher CES-D score than North Korean Defectors without physical illness(26.8+/-13.8 versus 19.7+/-12.7). However, there was no significant differences of CES-D score between South Koreans with physical illness and South Koreans without physical illness(10.3+/-9.8 versus 9.3+/-8.8). CONCLUSION: Compared to South Koreans, North Korean Defector showed higher depressive symptoms independently from gender, age, education, employment, marriage. In addition, only North Korean Defectors showed the relationship between depression and physical illness. Our study suggests that depression should be assessed when North Korean Defectors have physical illness.
Depression ; Employment ; Humans ; Male ; Marital Status ; Marriage

Purpose: This study was conducted to develop a predictive model for self-esteem of adolescents in multicultural families. The study was based on the theory of family stress model and literature review. Methods: This study used some of the data from the 1st year (2011) to the 6th year (2016) of the ‘Multicultural Adolescents Panel Study (MAPS)'.Data analysis was conducted for 1,208 children and 1,208 mothers from the data of the 4th MAPS (2014), which was the first year of the middle school students. The collected data were analyzed using SPSS-WIN Version 20.0 and AMOS Version 20.0. Results: The factors that have influence on the self-esteem of multicultural adolescents are mother’s parenting attitude and mother’s self-esteem. The explanatory power of these variables was 14%. Conclusion To increase the self-esteem of multicultural adolescents, a program that can positively change the self-esteem and parenting attitudes of multicultural mothers should be developed, and various social supports should be provided for multicultural mothers.

Purpose: To establish a foundation for raising awareness and disseminating accurate information about enuresis—one of the most challenging conditions to discuss openly—this paper examines public perceptions of enuresis. Methods: This paper collected title and text data from posts related to enuresis on the top popular online platforms such Naver Cafe in South Korea and Reddit in the United States (US). The data along with the thematic subcommunities where the posts were uploaded, was analyzed and visualized using word cloud, Latent Dirichlet Allocation (LDA) topic modeling, and pyLDAvis. Results: The findings reveal both similarities and differences in how the patients from the 2 countries addressed enuresis online. In both countries, enuresis symptoms were a daily concern, and individuals used online platforms as a space to talk about their experiences. However, South Koreans were more inclined to describe symptoms within region-based communities or mothers’ forums, where they exchanged information and shared experiences before consulting a doctor. In contrast, US patients with medical experience or knowledge frequently discussed treatment processes, lifestyle adjustments, and medication options. Conclusions South Koreans tend to be cautious when selecting and visiting hospitals, often relying on others for advice and preparation before seeking medical attention. Compared to online communities in the US, Korean users are more likely to seek preliminary diagnoses based on nonprofessional opinions. Consequently, it is important to lower the barriers for patients to access professional medical advice to mitigate the potential harm of relying on nonprofessional opinions. Additionally, there is a need to raise awareness so that adults can recognize and address their symptoms in a timely manner.

PURPOSE:Recent studies proposed that polyunsaturated fatty acids (PUFA) of the omega6 series (linoleic acid, LA) compared to the omega 3 series (linolenic acid, LNA) may be a potent promoters of adipogenesis during the gestation/lactation period. Increased ratio of LA/LNA may stimulate adipose tissue development during gestation/lactation period and may subsequently lead to chilhood obesity. The purpose of this study is to evaluate fatty acids composition of the breast milk in Korean lactating women and to analyze the relationship between LA/LNA and the body weight gain of the infants. METHODS:Fifty eight healthy postpartum lactating women and their healthy 58 breast feeding infants were studied. At 11.3+/-3.9 days of postpartum, breast milk were taken and analyzed by gas-liquid chromatography with one-step methylation method. RESULTS:The total fat contents in breast milk were 3.2+/-1.3%. The total fat contents in breast milk were not related to maternal BMI or Kaup index of infants. The percentage of PUFA was 24.9+/-6.0%. Among these PUFA, the percentage of LA and LNA was 20.3+/-5.8% and 2.2+/-0.8%, respectively. LA/ LNA ratio was 10.2+/-2.5. There were no significant correlations between LA/LNA ratio and infant weight gain during the first 2 months of life. CONCLUSION: Short term weight gain of infant was not related to the LA/LNA ratio in the breast milk. Further studies on the long term effects on fat percent and body weight of infants would be needed.
Adipogenesis ; Adipose Tissue ; alpha-Linolenic Acid ; Body Weight* ; Breast Feeding ; Breast* ; Chromatography, Gas ; Fatty Acids* ; Fatty Acids, Unsaturated ; Female ; Humans ; Infant* ; Linoleic Acid ; Methylation ; Milk, Human* ; Obesity ; Postpartum Period ; Weight Gain

PURPOSE: We carried out this study to determine if there is any difference in the occurrence rate of the epileptiform discharge between awake EEG and sleep EEG and if there are any factors influencing on the occurrence rate of EEG. METHODS: This study included 178 epileptic children who had visited neurology clinic of the department of pediatrics, Pusan National University Hospital from July 2005 to July 2006. The medical and EEG records of these children who had had both awake EEG and sleep EEG were reviewed. We analysed the occurrence rate of the epileptiform discharge between awake EEG and sleep EEG. We investigated the related clinical factors which included sex, seizure types, underlying causes, age at first seizure, antiepileptic drug (AED) medication, age at recording, and background activity. RESULTS: Among 178 epileptic children, 91 patients (51.1%) showed epileptiform discharge in awake or sleep states, 10 patients (11.0%) abnormal only in awake, 40 patients (44.0%) abnormal only in sleep, 41 patients (45.0%) abnormal in both awake EEG and sleep EEG. The occurrence rate of sleep EEG was 81 of 178 patients (45.5%) which was more than that of the awake EEG (28.7%) (P<0.001). The occurrence rate of sleep EEG is more than that of the awake EEG regardless of sex and underlying causes. But there is no significant difference from awake EEG and sleep EEG in finding the epileptiform discharge in the patient with generalized seizure, younger than 5 years old at first seizure, younger than 10 years old at recording, no antiepileptic medication, and abnormal background activity. CONCLUSION: The sleep EEG is thought to be more helpful in the diagnosis of childhood epilepsy.
Child ; Electroencephalography ; Epilepsy ; Humans ; Neurology ; Pediatrics ; Seizures

Testicular torsion is rare in newborn infants. However, its frequency has increased, most of which are reported in full-term infants. We diagnosed and treated testicular torsion in an extremely low birth weight infant (ELBWI). A 2x2 cm red mass was palpable in the left groin of a 24-week-old, 745 g, male newborn at 23 days of age. Left testicular torsion was diagnosed, and emergent orchiopexy was performed. Careful physical examination is needed in cases suspicious of testicular torsion in ELBWIs with cryptorchidism. Moreover, early diagnosis and emergent exploration are necessary to prevent complications such as the risk of anorchia.
Cryptorchidism ; Early Diagnosis ; Gonadal Dysgenesis, 46,XY ; Groin ; Humans ; Infant ; Infant, Extremely Low Birth Weight ; Infant, Newborn ; Male ; Orchiopexy ; Physical Examination ; Spermatic Cord Torsion ; Testis

The authors report seven cases of carotid-ophthalmic artery aneurysms who were surgically treated using the combined extradural-intradural or intradural approach from March, 1991, to December, 1993. Carotid-ophthalmic artery aneurysms are of considerably surgical interest because some of these were considered to have technical difficulties of clipping and disastrous surgical results. Removal of the anterior clinoid process and unroofing of the optic canal are the key points to exposure the operative field adequately. On the basis of these experiences, authors concluded that this approach is very useful, because of adequate surgical field and better mobilization of the internal carotid artery and optic nerve for direct clipping of carotid-ophthalmic artery aneurysm and basilar tip aneurysm.
Aneurysm* ; Arteries* ; Carotid Artery, Internal ; Optic Nerve