Amantadine and the Risk of Dyskinesia in Patients with Early Parkinson's Disease: An Open-Label, Pragmatic Trial

OBJECTIVE: We examined whether amantadine can prevent the development of dyskinesia. METHODS: Patients with drug-naïve Parkinson's disease (PD), younger than 70 years of age and in the early stage of PD (Hoehn and Yahr scale < 3), were recruited from April 2011 to December 2014. The exclusion criteria included the previous use of antiparkinsonian medication, the presence of dyskinesia, significant psychological disorders, and previous history of a hypersensitivity reaction. Patients were consecutively assigned to one of 3 treatment groups in an open label fashion: Group A-1, amantadine first and then levodopa when needed; Group A-2, amantadine first, dopamine agonist when needed, and then levodopa; and Group B, dopamine agonist first and then levodopa when needed. The primary endpoint was the development of dyskinesia, which was analyzed by the Kaplan-Meier survival rate. RESULTS: A total of 80 patients were enrolled: Group A-1 (n = 27), Group A-2 (n = 27), and Group B (n = 26). Twenty-four patients were excluded from the analysis due to the following: withdrawal of amantadine or dopamine agonist (n = 9), alternative diagnosis (n = 2), withdrawal of consent (n = 1), and breach in the protocol (n = 12). After exclusion, 5 of the 56 (8.93%) patients developed dyskinesia. Patients in Group A-1 and A-2 tended to develop dyskinesia less often than those in Group B (cumulative survival rates of 0.933, 0.929, and 0.700 for A-1, A-2, and B, respectively; p = 0.453). CONCLUSION: Amantadine as an initial treatment may decrease the incidence of dyskinesia in patients with drug-naïve PD.

Critical Care Nurses' Perception of Life-sustaining Treatment at End of Life: A Content Analysis / 중환자간호학회지

PURPOSE: The purpose of this study was to describe what critical care nurses perceived about life-sustaining treatment at end of life.METHODS: A qualitative content analysis method was utilized. The unit of analysis was interview text obtained from fifty critical care nurses of a general hospital.RESULTS: Seven categories in two content areas were abstracted. In the negative perception area, the following five categories were abstracted: patients' suffering, dying with damaged dignity, patients' isolation from family members, regret about choosing life-sustaining treatment, and family members' burden. In the positive perception area, the following two categories were abstracted: willingness to sustain life and duty as family members.CONCLUSIONS: Nurses have better competencies pertaining to understanding patients' responses and suffering than any other health care professions do. Nurses should play an important role in advocating for patients and their family in the process of end-of-life care decision making.

Pregnancy and Delivery in a Generalized Dystonia Patient Treated with Internal Globus Pallidal Deep Brain Stimulation: a Case Report

Internal globus pallidus (GPi) deep brain stimulation (DBS) has been widely accepted as an effective treatment modality of medically refractory dystonia. However, there have been few studies regarding the safety issue of pregnancy and childbirth related with DBS. This report describes a female patient who was pregnant and delivered a baby after GPi DBS surgery. A 33-year-old female patient with acquired generalized dystonia underwent bilateral GPi DBS implantation. She obtained considerable improvement in both movement and disability after DBS implantation. Four years later, she was pregnant and the obstetricians consulted us about the safety of the delivery. At 38-weeks into pregnancy, a scheduled caesarian section was carried out under general anesthesia. After induction using thiopental and succinylcholine, intubation was done quickly, followed by DBS turn off. For hemostasis, only bipolar electrocautery was used. Before awakening from the anesthesia, DBS was turned on as the same parameters previously adjusted. After delivery, she could feed her baby by herself, because the dystonia of left upper extremity and hand was improved. Until now, she has been showing continual improvement and being good at housework, carrying for children, with no trouble in daily life. This observation indicates that the patients who underwent DBS could safely be pregnant and deliver a baby.

Survival of Korean Huntington's Disease Patients

OBJECTIVE: The survival of Huntington’s disease (HD) patients is reported to be 15–20 years. However, most studies on the survival of HD have been conducted in patients without genetic confirmation with the possible inclusion of non-HD patients, and all studies have been conducted in Western countries. The survival of patients with HD in East Asia, where its prevalence is 10–50-fold lower compared with Western populations, has not yet been reported. METHODS: Forty-seven genetically confirmed Korean HD patients from independent families were included in this retrospective medical record review study. RESULTS: The mean age at onset among the 47 patients was 46.1 ± 14.0 years. At the time of data collection, 25 patients had died, and these patients had a mean age at death of 57.8 ± 13.7 years. The Kaplan-Meier estimate of the median survival from onset in the 47 patients was 14.5 years (95% confidence interval: 12.3–16.6). None of the following factors were associated with the survival time in the univariate Cox regression analysis: gender, age at onset, normal CAG repeat size, mutant CAG repeat size, and the absence or presence of non-motor symptoms at onset. CONCLUSION: This is the first Asian study on survival in HD patients. Survival in Korean HD patients may be shorter than that reported for Western populations, or at least is in the lower range of expected survival. A larger longitudinal observation study is needed to confirm the results found in this study.

Clinical Heterogeneity of Atypical Pantothenate Kinase-Associated Neurodegeneration in Koreans

OBJECTIVE: Neurodegeneration with brain iron accumulation (NBIA) represents a group of inherited movement disorders characterized by iron accumulation in the basal ganglia. Recent advances have included the identification of new causative genes and highlighted the wide phenotypic variation between and within the specific NBIA subtypes. This study aimed to investigate the current status of NBIA in Korea. METHODS: We collected genetically confirmed NBIA patients from twelve nationwide referral hospitals and from a review of the literature. We conducted a study to describe the phenotypic and genotypic characteristics of Korean adults with atypical pantothenate kinase-associated neurodegeneration (PKAN). RESULTS: Four subtypes of NBIA including PKAN (n = 30), PLA2G6-related neurodegeneration (n = 2), beta-propeller protein-associated neurodegeneration (n = 1), and aceruloplasminemia (n = 1) have been identified in the Korean population. The clinical features of fifteen adults with atypical PKAN included early focal limb dystonia, parkinsonism-predominant feature, oromandibular dystonia, and isolated freezing of gait (FOG). Patients with a higher age of onset tended to present with parkinsonism and FOG. The p.R440P and p.D378G mutations are two major mutations that represent approximately 50% of the mutated alleles. Although there were no specific genotype-phenotype correlations, most patients carrying the p.D378G mutation had a late-onset, atypical form of PKAN. CONCLUSIONS: We found considerable phenotypic heterogeneity in Korean adults with atypical PKAN. The age of onset may influence the presentation of extrapyramidal symptoms.

Wanting Extremely Low BMI May be Associated with Higher Depression and Undesirable Dietary Habits in High School Girls Who were Not Overweight / 대한지역사회영양학회지

The widespread pursuit for the thin physique may have detrimental impact on the wellbeing of the young generation, especially females. This study aimed to determine the effect of wanting very low body weight on dietary habits and psychological factors of female adolescents. Participants were 619 girls from 5 high schools in Kyeonggi, Korea. Information on dietary behaviors, psychological factors as well as current heights and weights and the desired weight for current height were obtained by questionnaire. Of total subjects, 38.1%, 35.5%, and 26.5% desired weights corresponding to normal (10~85 percentile), low (3~10 percentile) and very low BMI (< 3 percentile), respectively. The subjects who wanted to be very low weight had the average BMI of 18.57 kg/m2, which was significantly lower than 21.21 kg/m2 of those who wanted to be normal weight (p < 0.001). The subjects who desired very low weight had significantly higher scores for depression symptoms (p < 0.05), while there were no differences in obsession to lose weight and obesity stress. Moreover, more subjects in this group had undesirable dietary habits such as eating fast foods more than weekly (p < 0.05). These findings suggest that the desire for extreme thinness may lead female adolescents to have not only unreasonably similar obesity stress and obsession to lose weight but also higher depression symptoms, along with undesirable dietary habits. The findings suggest the potential harm from excessive weight concerns of female adolescents; thus efforts to teach this group about healthy weights are urgently needed.

A Case of Cerebellar Infarction Caused by Acute Subclavian Thrombus Following Minor Trauma

Subclavian steal syndrome caused by an acute thrombus is very rare. We present a case of cerebellar infarction with proximal subclavian artery thrombosis. A 56-year-old woman was admitted for sudden vertigo. One day prior to admission, she received a shoulder massage comprised of chiropractic manipulation. On examination, her left hand was pale and radial pulses were absent. Blood pressure was weak in the left arm. Downbeat nystagmus and a right falling tendency were observed. Brain MRI showed multiple acute infarctions in the left cerebellum. The findings of Doppler ultrasonography in the left vertebral artery were compatible with a partial subclavian artery steal phenomenon. Digital subtraction angiography demonstrated a large thrombus in the left subclavian artery. After heparin infusion, thrombus size markedly decreased. Cerebellar infarction caused by acute subclavian thrombosis following minor trauma is rare, but the thrombus can be successfully resolved with anticoagulation.

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.

Duplex Sonographic Findings of Isolated Left Brachiocephalic Artery / 대한뇌졸중학회지

Isolated left brachiocephalic artery with right aortic arch is extremely rare congenital anormaly. This malformation can cause subclavian steal phenomenon. We report the duplex sonographic and conventional angiographic findings of isolated left brachiocephalic artery in a 22-year-old female presented with dizziness.

Associations of Polymorphisms in Uncoupling Protein 2 and 3-Adrenergic Receptor with Obesity in Korean Adults / 대한내분비학회지

BACKGROUND: The genetic and environmental factors involved in the development of obesity and several candidate genes have been suggested to have an influence on energy and fuel metabolism. However, the specific genetic defects responsible for human obesity have not been identified yet. It is likely that a combination of polymorphisms in one or more candidate genes may affect energy metabolism and the development of obesity. We performed this study to determine the role of 45 bp insertion in the uncoupling protein (UCP)2 exon 8 and Trp64Arg polymorphism of beta3-adrenergic receptor ( 3-AR) gene in the regulation of body weight and the pattern of fat distribution. METHODS: In 114 subjects (male: 40, female: 74, mean body mass index: 24.1+/-2.7 kg/m2, 80 subjects with normal glucose tolerance, 34 subjects with impaired glucose tolerance), body fat distribution patterns were assessed by anthropometric measurement, bioelectric impedance analysis and computed tomogram. The genotypes of UCP genes were determined by polymerase chain reaction (PCR), and mutation in 3-AR gene by PCR followed by enzymatic digestion. RESULTS: In UCP2 genes, the frequency of deletion homozygote (DD) was 59.4%, heterozygote (DI) was 3.5% and insertion homozygote (II) was 3.1% Meanwhile, in 3-AR, the frequency of TrpTrp was 67.9%, TrpArg was 29.5% and ArgArg was 2.7%. In the lean group (subjects with a BMI less than 25 kg/m2), the frequencies of insertion allele and Arg64 allele were not significantly different than those among the overweight subjects (BMI > or = 25 kg/m2). There was not significant difference in clinical, biochemical or body fat distribution patterns between the groups according to UCP2 polymorphism. In the case of the polymorphism in 3-AR gene, the subjects with ArgArg homozygotes had lower HDL-cholesterol level (p<0.05). For the individuals over 40 years of age, BMI was greater among those with the deletion homozygotes and Arg64 allele, as compared to other groups according to the combination of UCP2 and 3-AR genotypes (p<0.05). CONCLUSION: These results suggest that genetic variations in UCP2 and 3-AR can synergistically affect metabolic rate and susceptibility to weight gain, thereby and contribute to the change in body weight in later life.

Job Satisfaction and Customer-oriented Service Performance of University Foodservice Employees

The aims of this study were to estimate the level of job satisfaction and customer-oriented service performance in university food service employees Two hundreds-six food service employees working in Seoul were enrolled and the response rate was 85.8%. Of respondents, 85.9% was female and most of the respondents were 40 years up (70.8%). Generally, respondents had approximately 5 years job experience and high school level. Mean score of job satisfaction was 3.23 score out of 5.00. The highest and lowest items on job satisfaction were responsibility and commitment(4.00). and promotions opportunity(2.69), respectively. Considering job satisfaction; relationships of supervisors, employment and career were significantly different in age(P<0.05), job-system and wage(P<0.05), respectively. Mean score of customer-oriented service performance was 3.75. The highest and lowest items on customer-oriented service performance were delivering on time(4.02) and service information for customers(3.21), respectively. Customer-oriented service performance was significantly different in age and wage(P<0.05). Job satisfaction had positive correlation to customer-oriented service performance. Relationship of supervisors was positively correlated with relationship of coworkers and career.