A Validation Study of the Korean Version of Social Communication Questionnaire

OBJECTIVES: The purposes of this study were to examine the reliability and validity of the Korean version of Social Communication Questionnaire (K-SCQ) and to determine cut-off scores for diagnosis of autism spectrum disorder (ASD). METHODS: A total of 166 subjects with ASD and their 186 unaffected siblings were recruited through child psychiatry clinics of university hospitals. Board certified child psychiatrists screened all probands suspected to have ASD based on the Diagnostic and Statistical Manual of Mental Disorders, fourth edition. To confirm the diagnoses, the Korean versions of the Autism Diagnostic Observation Schedule and the Autism Diagnostic Interview-Revised (K-ADI-R) were administered to all the subjects. All parents completed the K-SCQ and Social Responsiveness Scale (SRS). The non-ASD siblings were evaluated with the same instruments as the probands with ASD. We performed a factor analysis to examine the structure of K-SCQ. For testing the validity of K-SCQ, we compared the difference in Lifetime and Current scores of probands with ASD and their non-ASD siblings using t-test and analysis of covariance. Correlations between the K-SCQ and other measurements of ASD symptomatology, including K-ADI-R totals and domain scores and SRS, were examined. Receiver operation characteristic curve analysis was performed to extract cutoff scores discriminating affection status. RESULTS: Four factors were extracted through factor analysis of K-SCQ ; 1) social relation and play, 2) stereotyped behavior, 3) social behavior, and 4) abnormal language. Cronbach's internal consistency was .95 in K-SCQ Lifetime, and .93 in K-SCQ Current. There were significant differences in total score of K-SCQ, both in Lifetime and Current between the ASD group and non-ASD siblings group (p<.001). K-SCQ scores were significantly correlated with K-ADI-R subdomain scores and SRS total scores (p<.001). The best-estimate cut-off scores of K-SCQ for diagnosis of ASD were 12 for 48 months and over, and 10 for below 47 months. CONCLUSION: Our findings suggest that the K-SCQ is a reliable and valid instrument for screening autistic symptoms in the Korean population. Lower cut-off scores than the original English version might be considered when using it as a screening instrument of ASD.

Neurosarcoidosis after Uveitis

No abstract available.

Effects of Art & Culture Education on Behavior and Social Skill in Children: A Preliminary Study

OBJECTIVES: Art and culture education might play a good role in children's emotions ; however, few studies to verify this have been conducted. The objective of this study was to examine the effect of an art and culture education program on behavior and social skills in children aged 7-10 years. METHODS: Fifteen children (mean age 7.31+/-0.95 years) were recruited from a community center and participated in an art and culture education program. The participants received a once a week program for 17 sessions, and we compared scores on the Child Behavior Checklist (CBCL) and Social Skills Improvement System-Rating Scales (SSIS-RS) before and after intervention. RESULTS: The participants' subscale scores of CBCL-aggressive behavior, social immaturity, internalizing behavior, externalizing behavior, total behavior problems and social skill scores on the SSIS-RS showed improvement (p<.05) after 15-weeks of the art and culture education program. CONCLUSION: These results suggest a positive effect of an art and culture education program on the reduction of externalizing and internalizing behaviors and improvement of social skills in children. Further prospective, controlled studies in large samples are needed in order to confirm our findings.

Wernicke's Encephalopathy With Reversible Cortical Involvement

No abstract available.

Delayed-Onset Subdural Hematoma and Oculomotor Nerve Palsy After Improving Spontaneous Intracranial Hypotension

Spontaneous intracranial hypotension (SIH) is a well-known disorder improving with conservative treatment or epidural blood patch in the majority of cases. However, SIH may develop neurological complications such as cranial nerve palsy, subdural hematoma, and altered consciousness. Subdural hematoma in SIH is usually found during intracranial hypotension state and delayed subdural hematoma is rarely reported. We report a case of delayed subdural hematoma and oculomotor nerve palsy after improving spontaneous intracranial hypotension.

Patterns of Drug Use and Factors Associated with Polypharmacy in Elderly Patients Visiting Psychiatric Outpatient Clinic / 신경정신의학

OBJECTIVES: The objective of this study was to investigate the patterns and recent trends in polypharmacy among elderly patients visiting psychiatric outpatient clinic. METHODS: 289 elderly patients visiting psychiatric outpatient clinic were interviewed and their medical records were thoroughly reviewed. Inclusion criteria were as follows : 1) 65 years and older age patients, 2) diagnosed with one or more psychiatric disorder according to ICD-10 criteria, 3) who were willing to participate in this research. Demographic data and clinical data, including prevalence of polypharmacy, doses and types of drugs both prescribed and self-administered, were analyzed. Polypharmacy was defined as the long-term simultaneous use of six or more drugs. Data were statistically analyzed by t-test, analysis of variance, and simple regression analysis. RESULTS: 57.1% of the patients were prescribed with more than six drugs simultaneously. Mean number [+/-standard deviation (SD)] of psychiatric drug was 3.42+/-1.41. Prevalence of polyphamacy was different among psychiatric disorder groups, bipolar disorder group being the highest (mean+/-SD drug used=5.17+/-0.98). Regarding sedative-hypnotics, 59.5% of patients were prescribed (mean+/-SD drug used=2.02+/-1.07). Visual Analogue Scale (VAS) and total number of drugs prescribed were positively correlated (p<0.05). Herb medicine/dietary supplement had no influence on total number of used drugs. CONCLUSION: Elderly patients are suffering from many comorbidities and vulnerable to multiple drug use that can be potentially harmful, so it is important and essential to be aware of the possibility of polypharmacy and prescribe appropriate drugs to improve their quality of life.

Tolosa-Hunt Syndrome With Facial Nerve Palsy

Tolosa-Hunt syndrome is a painful ophthalmoplegia due to nonspecific granulomatous inflammation in the cavernous sinus. A 16-year-old man developed Tolosa-Hunt syndrome accompanied by facial nerve palsy. MRI showed an inflammatory lesion in the cavernous sinus with gadolinium enhancement. This rare presentation suggests that Tolosa-Hunt syndrome is a localized form of idiopathic hypertrophic pachymeningitis.

Unilateral Abdominal Distension After Herpes Zoster Infection

No abstract available.

A Case of Hyperprolactinemia with Galactorrhea Induced by Carbamazepine

Carbamazepine (CBZ) may alter the secretion of prolactin (PRL), however, there is no report about hyperprolactinemia with galactorrhea induced by CBZ medication. A 30-year-old woman presented with galactorrhea. She had been treated with CBZ for three years and hormonal study revealed increased serum PRL levels. Sella MRI showed no evidence of pituitary lesion. Two months after changing CBZ to oxcarbazepine, galatorrhea was improved and the serum PRL level was normalized.

A Case of Acute Respiratory Muscle Weakness Complicated by Sjogren Syndrome

Sjogren syndrome is a chronic autoimmune disease characterized by lymphocytic infiltration of exocrine glands resulting in dry mouth and eyes. Approximately one-third of patients present with systemic manifestations, but respiratory muscle involvements have been rarely reported. We report a case of acute respiratory failure complicated by primary Sjogren syndrome. Muscle biopsy revealed perivascular lymphocytic infiltrations. Corticosteroid therapy improved respiratory muscle weakness. Sjogren syndrome should be considered as one of the underlying diseases causing acute respiratory failure.

Two Families of Andersen's Syndrome with Cardiac Arrhythmia, Periodic Paralysis, and KCNJ2 Gene Mutations

Andersen's syndrome is a rare autosomal dominant disorder characterized by periodic paralysis, dysmorphic features and cardiac arrhythmias. This syndrome is known to be a type of potassium channelopathies with a mutation in the KCNJ2 (Kir2.1) gene. Here, we present two families with genetically confirmed Andersen's syndrome through clinical and electrophysiological findings. They showed all features of the triad, and one of them had a novel mutation c.307G>A (Met307Ile).

A Life-Threatening Case of Tubular Esophageal Duplication Complicated with Aneurysm of the Aorta / 소아과

Esophageal duplication cysts are rare congenital lesions that occur as a result of a failure in the tubulation of the esophagus. They are most frequently single, tubular, or cystic. They may cause compressive symptoms or may be discovered incidentally on chest radiographs. They become symptomatic when complications develop. Symptoms often are related to the location of the duplication; esophageal lesions can create respiratory difficulties. The definitive diagnosis of esophageal duplication cysts requires the pathological evaluation of the cyst after surgical removal. We experienced a rare tubular esophageal duplication, in a 2-month old girl who presented with fever and grunting. This is the first reported case in which the sequence of events of ruptured tubular esophageal duplication with empyema, mediastinitis and aneurysm occured.

Increased Serum Endothelin (ET) -1 in Children with Mycoplasma pneumoniae Pneumonia / 소아알레르기및호흡기학회지

PURPOSE: Mycoplasma pneumoniae frequently causes lower respiratory illnesses in children, and is known to be associated with acute wheezing and the exacerbation of asthma. However, the mechanism by which this pathogen contributes to the development of wheeze- related symptoms is not fully understood. Previous studies have shown that ET-1 is a potent bronchoconstrictor and implicated in the pathogenesis of bronchial asthma. The aim of our study was to examine the serum ET-1 and other cytokines in M. pneumoniae pneumonia and investigate if there is any difference in relation to the presence of wheezing. METHODS: Patients admitted with pneumonia were divided into three groups: M. pneumoniae pneumonia with wheeze (group 1) and without wheeze (group 2), and patients with pneumonia due to other pathogens (group 3). The serum levels of ET-1, IL-18, IL-5 were measured by ELISA in patient groups and controls. RESULTS: Serum ET-1 increased significantly in the patients with M. pneumoniae pneumonia (groups 1 and 2) compared with group 3 and controls. ET-1 in group 1 was significantly higher than in group 2. IL-5 and IL-18 were higher in patient groups than in controls with no difference between groups. Serum total IgE was significantly higher in group 1 than in group 2. A positive correlation was observed between serum ET-1 and total IgE. CONCLUSION: Our results suggest that ET-1 may be implicated in the pathogenesis of bronchoconstriction and allergic inflammation in the airways of the patients with M. pneumoniae pneumonia.

A Case of Ring Chromosome 20 with Mental Retardation and Epilepsy / 소아과

Ring chromosome 20 mosaicism [r(20)] is a rare chromosomal anomaly associated with minor dysmorphism, mental retardation, autistic behavior, and intractable epilepsy. The proposed mechanism of ring formation is breakage of both short and long arms of a chromosome with subsequent end-to- end fusion. We encountered an 18-month-old boy who presented with developmental delay and mental retardation with seizure episodes, but showed normal brain magnetic resonance imaging. Chromosome study from peripheral blood showed 46,XY, r(20)(p13q13.3) karyotype. The authors report a case of ring chromosome 20 with mental retardation and epilepsy, with a review of the literature.

Intraexaminer Variability of Nerve Conduction Study in Healthy Person

BACKGROUND: Nerve conduction study (NCS) is an objective and quantitative test in evaluating peripheral nerve disorders. Several physiological and technical factors are well known to influence the results of NCS, which can be controlled and regulated by standardization of environment and through the process to make range of normality. However, most electromyographers do not pay much attentions about inter- and intra-examiner variabilities, and there are only a few and incomplete reports on these topics. We examined the intra-examiner variability of NCS on the basis of periods of practice. METHODS: Twenty-eight electromyographers were divided into two groups: residents and neurologist-technicians. All, having variable NCS training periods, have performed NCS on one of other 27 electromyographers ten times within two weeks where each study was made once a day. RESULTS: Coefficient of variation and external quotient increased according to the following order - nerve conduction velocities (NCV), terminal latencies (TL), and amplitudes of compound action potentials (AMP). There were significant differences between the two groups in NCV and TL, but no statistical difference in AMP. CONCLUSIONS: Our results suggest that errors from intra-examiner variability should be considered when interpreting NCS and that those electromyographers who have enough training should perform NCS.

The Relationship between Developmental Quotient in Children with Delayed Language Development and Later Intellectual Quotient / 소아과

PURPOSE: The objective of this study was to test the predictive validity of developmental test for later intellectual development in young children with delayed language development. METHODS: The study subjects were 66 children who had presented delayed language development and had an initial developmental test and a follow-up intelligence test. The mean age at initial test was 34.2 months(17-48 months), and the mean age at the follow-up test was 55.0 months(40-79.5 months). RESULTS: According to CAT DQ(Cognitive Adaptive Test Developmental Quotient), 66 children were divided into 4 groups: group I(CAT DQ = or85). There was a moderate correlation between CAT DQ and later total IQ in group I(r=0.58) and II(r= 0.50), but a low correlation in group III(r=0.19) and IV(r=-0.16). IQs in follow-up tests of all children in group I and II were lower than 70, except two children whose IQs were 75 and 78. IQs of children in group III were lower than 85 except for three children whose IQs were 89, 89, and 90, respectively. CONCLUSION: The findings of this study suggest that if DQ is lower than 70, the child is highly probable to be mentally subnormal in later years. Although the correlation between CAT DQ and later total IQ is low in children with DQs over 70, if a DQ is between 70 and 84, the child is very likely to have learning disability in later years.

Apical Lung Cancer Associated with Neurofibromatosis Type 1

No abstract available.

Two Cases of Transient Cardiac Attacks Related to Steroid Pulse Therapy

No abstract available.

Two Cases of Evans Myopathy in a Family

Evans myopathy is a type of malignant hyperthermia showing characteristic distribution of myopathic changes. We present two cases of Evans myopathy, father and his son in a family. Five members of this family expired during ane sthesia and surgery and two of these cases were reported as the malignant hyperthermia. The muscle pathology shows moth-eaten appearance with or without unstained cores in NADH-TR stain and its difference between the father and his son is suggestive of rather progressive myopathy than static one. s

The Therapeutic Effect of Azathioprine in Four Patients with Idiopathic Hypertrophic Pachymeningitis

Idiopathic hypertrophic pachymeningitis is a rare inflammatory disease of unknown origin in which the recurrence is frequently observed despite an initial response to steroid therapy. Four patients, two men and two women aged 63 to 67 years, with severe headaches were evaluated by a brain MRI, and two patients were evaluated by follow up MRI receiving azathioprine therapy. All patients were given initial oral prednisolone 60mg or steroid pulse therapy followed by oral prednisolone and azathioprine therapy. Four patients improved with prednisolone but became steroid depen-dent. Azathioprine therapy permitted a reduction of the corticosteroid which may lead to clinical and radiological improvement. At present, high dose corticosteroid therapy is the treatment of choice, followed by immunosuppressive agents, such as azathioprine, if necessary. Further long-term follow-up studies of these patients are needed to clarify the outcome of this rare disease.