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

OBJECTIVES: This study researched the clinical effects of hippotherapy and medication in attention deficit hyperactivity disorder (ADHD) children. METHODS: The study employed an open label randomized controlled trial. Participants were 10 children of ages between 6 and 12 years. These subjects were placed in 2 groups : the hippotherapy group and the medication group. Evaluation tools used in this study include Affective Disorders and Schizophrenia-Present and Lifetime Version- Korean Version, the Wechsler Intelligence Scale for Children-IV, Korean Child Behavior Check List (K-CBCL), ADHD rating scale (ARS), Clinical Global Impression (CGI), and Quantitative electroencephalography (QEEG). RESULTS: Grossly scores of investigator-administered ADHD rating scale (ARS-I), K-CBCL, and CGI-Severity of Illness scale were improved in both the hippotherapy and the medication group. The ARS-I scores of the Hippotherapy group were not significantly different from baseline after 12 weeks. In the QEEG, theta/beta ratio of Pz area in the hippotherapy group was significantly different from baseline. Children treated with medication showed significant difference from baseline in ARS-I, K-CBCL subscales, and QEEG. CONCLUSION: The use of hippotherapy could be a viable treatment strategy as a part of a multimodal therapy for children with ADHD. This pilot study provides good prospects for future studies to document these positive trends on larger samples.
Attention Deficit Disorder with Hyperactivity* ; Child Behavior ; Child* ; Electroencephalography ; Equine-Assisted Therapy* ; Humans ; Intelligence ; Mood Disorders ; Pilot Projects*

BACKGROUND: A good acne grading system should reflect epidemiological and clinical features of a particular patient group. Current Korean acne grading systems are mostly based on the skin colors, epidemiology, and clinical features of Western countries, which make them less than being ideal for Korean acne patients. OBJECTIVE: We tried to devise an acne grading system based on clinical and epidemiological characteristics of Korean acne patients. This system is intended for use in primary care setting as well as in research purposes. METHODS: In the formulation of a new acne grading system, a panel of 5 dermatologists from 5 referral centers took into account the degree of complexity of the system, criteria for severity as well as skin colors through the review of clinical photographs of 250 acne patients. The new grading system then underwent inter-rater and intra-rater discrepancy assessment and simulation with 149 patient photos to find out an optimal epidemiological representation. RESULTS: We developed 6-scale grading system with both standard pictures and ranges of number of lesions as criteria. Intra-rater and inter-rater reproducibility turned out to be moderate to strong, suggesting clear instruction and easy performance. Simulation with 4 different models yielded best parameter ranges for the optimal criteria. CONCLUSIONS: With the feedback from the large-scale field applications, this new acne grading system will serve as a multipurpose acne grading system for Korean patients.
Acne Vulgaris* ; Epidemiology ; Humans ; Primary Health Care ; Referral and Consultation ; Skin

Objective: Adult attention deficit hyperactivity disorder (ADHD) has a heterogeneous clinical presentation with patients showing very frequent emotional problems. In the present study, patients with adult ADHD were subtyped based on their psychopathology using a person-centered approach. Methods: In the present chart review study, detailed findings of psychological evaluation conducted as part of routine care were utilized. A total of 77 subjects with adult ADHD were included in the analysis. Detailed ADHD symptoms, psychiatric comorbid Diagnostic and Statistical Manual of Mental Disorders (DSM) diagnoses, and severity of mood and anxiety symptoms were evaluated in the person-centered analysis. Results: Three clusters were generated using clustering analysis. DSM comorbid conditions did not significantly impact the clustering. Cluster 1 consisted of ADHD combined presentation (ADHD-C) with less mood symptoms, cluster 2 of ADHD predominantly inattentive presentation and cluster 3 of ADHD-C with significant mood symptoms. Patients in cluster 3 had adulthood functional impairment more frequently compared with patients in cluster 1. Patients in cluster 3 showed recurrent thoughts of death and suicidal ideation more frequently compared with patients in cluster 1. Conclusion Further studies are needed to confirm the relationships observed in the present study.

Objective: Adult attention deficit hyperactivity disorder (ADHD) has a heterogeneous clinical presentation with patients showing very frequent emotional problems. In the present study, patients with adult ADHD were subtyped based on their psychopathology using a person-centered approach. Methods: In the present chart review study, detailed findings of psychological evaluation conducted as part of routine care were utilized. A total of 77 subjects with adult ADHD were included in the analysis. Detailed ADHD symptoms, psychiatric comorbid Diagnostic and Statistical Manual of Mental Disorders (DSM) diagnoses, and severity of mood and anxiety symptoms were evaluated in the person-centered analysis. Results: Three clusters were generated using clustering analysis. DSM comorbid conditions did not significantly impact the clustering. Cluster 1 consisted of ADHD combined presentation (ADHD-C) with less mood symptoms, cluster 2 of ADHD predominantly inattentive presentation and cluster 3 of ADHD-C with significant mood symptoms. Patients in cluster 3 had adulthood functional impairment more frequently compared with patients in cluster 1. Patients in cluster 3 showed recurrent thoughts of death and suicidal ideation more frequently compared with patients in cluster 1. Conclusion Further studies are needed to confirm the relationships observed in the present study.

BACKGROUND/AIMS: Noncardiac chest pain (NCCP) is substernal, squeezing chest pain, unrelated to the cardiac problem. Our study aimed to define the prevalence and clinical characteristics of reflux esophagitis in NCCP patients in Korea. MATERIALS AND METHODS: We reviewed medical records of patients who visited Department of Cardiology, St. Paul's Hospital due to chest pain and had normal coronary arteriography and who had received endoscopy within 6 months. Patients diagnosed with peptic ulcer or gastric cancer were excluded. The patients were classified into two groups according to their endoscopic results; the reflux esophagitis group and the control group. RESULTS: Two hundred seventeen NCCP patients were enrolled and 96 patients (44.2%) were diagnosed with reflux esophagitis: 68 patients (31.3%) with minimal change esophagitis; 26 patients (12.0%) with Los Angeles (LA) grade A; 2 patients (0.9%) with LA grade B. There were no patients with severe erosive reflux disease. There were no significantly different characteristics in the reflux esophagitis group and the control group. CONCLUSIONS: The prevalence of reflux esophagitis in NCCP patients in Korea was 44.2%. Most patients had mild reflux esophagitis.
Angiography ; Cardiology ; Chest Pain* ; Endoscopy ; Esophagitis ; Esophagitis, Peptic* ; Gastroesophageal Reflux ; Humans ; Korea* ; Medical Records ; Peptic Ulcer ; Prevalence* ; Stomach Neoplasms ; Thorax*

There have been few studies investigating the association between atopic dermatitis (AD) and prenatal exposure to heavy metals. We aimed to evaluate whether prenatal exposure to heavy metals is associated with the development or severity of AD in a birth cohort study. A total of 331 subjects were followed from birth for a median duration of 60.0 months. The presence and severity of AD were evaluated at ages 6 and 12 months, and regularly once a year thereafter. The concentrations of lead, mercury, chromium, and cadmium in umbilical cord blood were measured by inductively coupled plasma mass spectrometry. Cord blood mononuclear cells (CBMCs) were isolated and stimulated for analysis of cytokine production using ELISA. Heavy metal levels in cord blood were not associated with the development of AD until 24 months of age. However, a positive correlation was observed between the duration of AD and lead levels in cord blood (p=0.002). AD severity was also positively associated with chromium concentrations in cord blood (p=0.037), while cord blood levels of lead, mercury, and cadmium were not significantly associated with AD severity (p=0.562, p=0.054, and p=0.055, respectively). Interleukin-13 production in CBMCs was positively related with lead and chromium levels in cord blood (p=0.021 and p=0.015, respectively). Prenatal exposure to lead and chromium is associated with the persistence and severity of AD, and the immune reaction toward a Th2 polarization.
Cacao ; Cadmium ; Chromium ; Cohort Studies ; Dermatitis, Atopic ; Enzyme-Linked Immunosorbent Assay ; Fetal Blood ; Interleukin-13 ; Mass Spectrometry ; Metals, Heavy ; Parturition ; Plasma ; Umbilical Cord

The plasma ghrelin has been reported to be elevated in Prader-Willi syndrome (PWS) and modulated by insulin. It was hypothesized that insulin might have a more pronounced effect on reducing plasma ghrelin in PWS patients, which would influence appetite. This study investigated the degree of ghrelin suppression using an euglycemic hyperinsulinemic clamp in children with PWS (n=6) and normal children (n=6). After a 90-min infusion of insulin, the plasma ghrelin level decreased from a basal value of 0.86+/-0.15 to 0.58+/-0.12 ng/mL in the controls, and from 2.38+/-0.76 to 1.12+/-0.29 ng/mL in children with PWS (p=0.011). The area under the curve below the baseline level over the 90 min insulin infusion was larger in children with PWS than in controls (-92.82+/-44.4 vs. -10.41+/-2.87 ng/mL/90 min) (p=0.011). The insulin sensitivity measured as the glucose infusion rate at steady state was similar in the two groups (p=0.088). The decrease in the ghrelin levels in response to insulin was more pronounced in the children with PWS than in the controls. However, the level of ghrelin was always higher in the children with PWS during the clamp study. This suggests that even though insulin sensitivity to ghrelin is well maintained, an increase in the baseline ghrelin levels is characteristic of PWS.
Prader-Willi Syndrome/*blood ; Peptide Hormones/*blood/*drug effects ; Metabolic Clearance Rate/drug effects ; Male ; Insulin/*administration & dosage/blood ; Infusions, Intravenous ; Humans ; Female ; Down-Regulation/drug effects ; Child ; Adolescent