• Journal of Life Science
• /
• v.29 no.7
• /
• pp.800-808
• /
• 2019

Charcot-Marie-Tooth disease (CMT) is a group of rare peripheral neuropathies characterized by progressive muscle weakness and atrophy and areflexia in the upper and lower extremities. The most common subtype of CMT is CMT1A, which is caused by a tandem duplication of the PMP22 gene in the 17p12 region. Patients with CMT1A show a loose genotype-phenotype correlation, which suggests the existence of secondary genetic or association factors. Recently, polymorphisms of rs71428439 (n.83A>G) and rs2292832 (n.86T>C) in the MIR149 have been reported to be associated with late onset and mild phenotypic CMT1A severity. The aim of this study was to examine the intrafamilial heterogeneities of clinical phenotypes according to the genotypes of these two SNPs in MIR149. For this study, we selected 6 large CMT1A families who showed a wide range of phenotypic variation. This study suggested that both SNPs were related to the onset age and severity in the dominant model. In particular, the AG+GG (n.83A>G) and TC+CC genotypes (n.86T>C) were associated to late onset and mild symptoms. Motor nerve conduction velocity (MNCV) was not related to the MIR149 genotypes. These results were consistent with the previous studies. Therefore, we suggest that the rs71428439 and rs2292832 variants in MIR149 may serve as genetic modifiers of CMT1A intrafamilial phenotypic heterogeneity, as they have a role in the unrelated patients. This is the first study to show an association using large families with variable clinical CMT1A phenotypes. The results will be helpful in the molecular diagnosis and treatment of patients with CMT1A.

• Korean journal of psychology:General
• /
• v.36 no.1
• /
• pp.81-107
• /
• 2017

The study aims to investigate the clinical utility of Bayley-III using US norm in Korea. A total of 98 preterm infants and 93 term infants were assessed with the K-Bayley-III. The performance pattern of preterm infants was analyzed with mixed design ANOVA which examined the differences of scaled scores and composite scores of Bayley-III between full term- and preterm- infant group and within preterm infants group. Then, We have investigated agreement between classifications of delay made using the BSID-II and Bayley-III. In addition, ROC plots were constructed to identify a Bayley-III cut-off score with optimum diagnostic utility in this sample. The results were as follows. (1) Preterm infants have significantly lower function levels in areas of 5 scaled scores and 3 developmental indexes compared with infants born at term. Significant differences among scores within preterm infant group were also found. (2) Bayley-III had the higher scores of the Mental Development Index and Psychomotor Developmental Index comparing to the scores of K-BSID-II, and had the lower rates of developmental delay. (3) All scales of Bayley-III, Cognitive, Language and Motor scale had the appropriate level of discrimination, but the cut-off composite scores of Bayley-III were adjusted 13~28 points higher than 69 for prediction of delay, as defined by the K-BSID-II. It explains the lower rates of developmental delay using the standard of two standard deviation. This study has provided empirical data to inform that we must careful when interpreting the score for clinical applications, identified the discriminating power, and proposed more appropriate cut-off scores. In addition, discussion about the sampling for making the Korean norm of Bayley-III was provided. It is preferable that infants in Korea should use our own validated norms. The standardization process to get Korean normative data must be performed carefully.

• Journal of Korean Society of Forest Science
• /
• v.111 no.4
• /
• pp.567-582
• /
• 2022

Chainsaw use for motor-manual timber harvesting in South Korea is associated with worker safety issues. However, forestry operations such as timber harvesting have already been mechanized to reduce hazards to workers and increase productivity. This study analyzed the productivities and costs of felling and processing, felling and processing using an excavator-based stroke harvester for Pinus rigida and Quercus mongolica stands. To efficiently operate the stroke harvester, we developed a regression equation to estimate the productivities of felling and processing, felling, and processing operations,and we conducted sensitivity analysis of the operation costs using DBH and machine utilization. The felling and processing productivity was 6.53 and 4.02 m³/SMH for P. rigida a nd Q. mongolica, respectively, and the cost was 17,983 and 29,210 won/m³, respectively. The felling productivity for P. rigida a nd Q. mongolica wa s 40.9 and 23.0 m³/SMH, respectively, and the cost was 2,667 and 4,743 won/m³, respectively. The processing productivity for P. rigida and Q. mongolica was 8.25 and 7.75 m³/SMH, respectively, and the cost was 15,296 and 16,283 won/m³, respectively. In the developed regression equation, the DBH, traveling distance, and number of cuttings were found to be important factors (p<0.05). Therefore, it is necessary to construct a DB considering the various conditions and species associated with harvester operations, and further research is needed to increase the accuracy of predicting operation productivity and costs.

• 한국노년학
• /
• v.31 no.1
• /
• pp.129-141
• /
• 2011

This study aims to find out effects of a tailored occupational activity program(TOAP) on the activities of daily living(ADL), cognitive function, depressive mood, and caregiver burden, who live in the community. Method : From October 2009 to May 2010, the TOAP was applied to 15 dementia patients and 15 of their caregivers, who was visitors of the Y-city Center for Managing Dementia in Gyunggi-do. The TOAP was designed for habituating patients and caregivers to the techniques acquired through goal activities and task and making it capable of being routinized regularly. The TOAP was applied to dementia patients and their cvaregivers twice a week for 7 weeks(one-time home visit, one-time phone inspection), a total of 14 times. Results: Significant differences among pre-test and post-test were found in the AMPS motor skills(1.10±1.14 and 1.34±1.2 respectively) scores, AMPS process skills(0.32±0.55 and 0.77±0.66 respectively) scores, ACL(3.86±0.65 and 4.17±0.64 respectively) scores, MMSE-KC(17.33±4.6 and 19.33±4.97 respectively) scores, GDS(11.73±6.87 and 8.53±7.09 respectively) scores, and caregiver burden(31.80±20.06 and 26.13±18.07 respectively) scores(p<0.05). A significant effect was confirmed from the TOAP which ADL, cognitive function, reduced patient's depression and caregiver burden(p<0.05). Conclusion: From the above results that a TOAP has an effect on the improvement of the ability to ADL, cognitive function and reduced depression and caregiver burden of dementia patients living in community. The present author hopes that, in the future, more diverse community based on tailored occupational activity programs will be developed to improve the functions of dementia patients living in community.

• Journal of The Korean Association For Science Education
• /
• v.29 no.8
• /
• pp.990-1010
• /
• 2009

To derive brain-based evolutionary educational principles, this study examined the studies on the structural and functional characteristics of human brain, the biological evolution occurring between- and within-organism, and the evolutionary attributes embedded in science itself and individual scientist's scientific activities. On the basis of the core characteristics of human brain and the framework of universal Darwinism or universal selectionism consisted of generation-test-retention (g-t-r) processes, a Model of Brain-based Evolutionary Scientific Teaching for Learning (BEST-L) was developed. The model consists of three components, three steps, and assessment part. The three components are the affective (A), behavioral (B), and cognitive (C) components. Each component consists of three steps of Diversifying $\rightarrow$ Emulating (Executing, Estimating, Evaluating) $\rightarrow$ Furthering (ABC-DEF). The model is 'brain-based' in the aspect of consecutive incorporation of the affective component which is based on limbic system of human brain associated with emotions, the behavioral component which is associated with the occipital lobes performing visual processing, temporal lobes performing functions of language generation and understanding, and parietal lobes, which receive and process sensory information and execute motor activities of the body, and the cognitive component which is based on the prefrontal lobes involved in thinking, planning, judging, and problem solving. On the other hand, the model is 'evolutionary' in the aspect of proceeding according to the processes of the diversifying step to generate variants in each component, the emulating step to test and select useful or valuable things among the variants, and the furthering step to extend or apply the selected things. For three components of ABC, to reflect the importance of emotional factors as a starting point in scientific activity as well as the dominant role of limbic system relative to cortex of brain, the model emphasizes the DARWIN (Driving Affective Realm for Whole Intellectual Network) approach.

• The Sea:JOURNAL OF THE KOREAN SOCIETY OF OCEANOGRAPHY
• /
• v.15 no.1
• /
• pp.41-50
• /
• 2010

We have developed an in-situ benthic chamber (BelcI) for use in coastal studies that can be deployed from a small boat. It is expected that BelcI will be useful in studying the benthic boundary layer because of its flexibility. BelcI is divided into three main areas: 1) frame and body chamber, 2) water sampler, and 3) stirring devices, electric controller, and data acquisition technology. To maximize in-situ use, the frame is constructed from two layers that consist of square cells. All electronic parts (motor controller, pA meter, data acquisition, etc.) are low-power consumers so that the external power supply can be safely removed from the system. The hydrodynamics of BelcI, measured by PIV (particle image velocimetry), show a typical "radial-flow impeller" pattern. Mixing time of water in the chamber is about 30 s, and shear velocity ($u^*$) near the bottom layer was calculated at $0.32\;cm\;s^{-1}$. Measurements of diffusivity boundary layer thickness showed a range of $180-230\;{\mu}m$. Sediment oxygen consumption rate, measured in-situ，was $84\;mmol\;O_2\;m^{-2}\;d_{-1}$, more than two times higher than on-board incubation results. Benthic fluxes assessed from in-situ incubation were estimated as follows: nitrate + nitrite = $0.18\;{\pm}\;0.07\;mmol\;m^{-2}\;d^{-1}$ ammonium $23\;{\pm}\;1\;mmol\;m^{-2}\;d^{-1}$ phosphate = $0.09\;{\pm}\;0.02\;mmol\;m^{-2}\;d^{-1}$ and silicate = $23\;{\pm}\;1\;mmol\;m^{-2}\;d^{-1}$.

• The Korean Journal of Physiology
• /
• v.8 no.2
• /
• pp.1-17
• /
• 1974

This experiment was designed to explore the specific functional interrelations between the vestibular semicircular canals and the extraocular muscles which may disclose the neural organization, connecting the vestibular canals and each ocular motor nuclei in the brain system, for vestibuloocular reflex mechanism. In urethane anesthetized rabbits, a fine wire insulated except the cut cross section of its tip was inserted into the canals closely to the ampullary receptor organs through the minute holes provided on the osseous canal wall for monopolar stimulation of each canal nerve. All extraocular muscles of both eyes were ligated and cut at their insertio, and the isometric tension and EMG responses of the extraocular muscles to the vestibular canal nerve stimulation were recorded by means of a physiographic recorder. Upon stimulation of the semicircular canal nerve, direction if the eye movement was also observed. The experimental results were as follows. 1) Single canal nerve stimulation with high frequency square waves (240 cps, 0. 1 msec) caused excitation of three extraocular muscles and inhibition of remaining three muscles in the bilateral eyes; stimulation of any canal nerve of a unilateral labyrinth caused excitation (contraction) of the superior rectus, superior oblique and medial rectus muscles and inhibition (relaxation) of the inferior rectus, inferior oblique and lateral rectos muscles in the ipsilateral eye, and it caused the opposite events in the contralateral eye. 2) By the overlapped stimulation of triple canal nerves of a unilateral labyrinth, unidirectional (excitatory or inhibitory) summation of the individual canal effects on a given extraocular muscles was demonstrated, and this indicates that three different canals of a unilateral vestibular system exert similar effect on a given extraocular muscles. 3) Based on the above experimental evidences, a simple rule by which one can define the vestibular excitatory and inhibitory input sources to all the extraocular muscles is proposed; the superior rectus, superior oblique and medial rectus muscles receive excitatory impulses from the ipsilateral vestibular canals, and the inferior rectus, inferior oblique and lateral rectus muscles from the contralateral canals; the opposite relationship applies for vestibular inhibitory impulses to the extraocular muscles. 4) According to the specific direction of the eye movements induced by the individual canal nerve stimulation, an extraocutar muscle exerting major role (a muscle of primary contraction) and two muscles of synergistic contraction could be differentiated in both eyes. 5) When these experimental results were compared to the well known observations of Cohen et al. (1964) made in the cats, extraocular muscles of primary contraction were the same but those of synergistic contraction were partially different. Moreover, the oblique muscle responses to each canal nerve excitation appeared to be all identical. However, the responnes of horizontal (medial and lateral) and vertical (superior and inferior) rectus muscles showed considerable differences. By critical analysis of these data, the author was able to locate theoretical contradictions in the observations of Cohen et al. but not in the author's results. 6) An attempt was also made to compare the functional observation of this experiment to the morphological findings of Carpenter and his associates obtained by degeneration experiments in the monkeys, and it was able to find some significant coincidence between there two works of different approach. In summary, the author has demonstrated that the well known observations of Cohen et al. on the vestibulo-ocular interrelation contain important experimental errors which can he proved by theoretical evaluation and substantiated by a series of experiments. Based on such experimental evidences, a new rule is proposed to define the interrelation between the vestibular canals and the extraocular muscles.

• The Korean Journal of Nuclear Medicine
• /
• v.39 no.6
• /
• pp.413-420
• /
• 2005

Purpose: Neuroreceptor PET studies require 60-120 minutes to complete and head motion of the subject during the PET scan increases the uncertainty in measured activity. In this study, we investigated the effects of the data-driven head mutton correction on the evaluation of endogenous dopamine release (DAR) in the striatum during the motor task which might have caused significant head motion artifact. Materials and Methods: $[^{11}C]raclopride$ PET scans on 4 normal volunteers acquired with bolus plus constant infusion protocol were retrospectively analyzed. Following the 50 min resting period, the participants played a video game with a monetary reward for 40 min. Dynamic frames acquired during the equilibrium condition (pre-task: 30-50 min, task: 70-90 min, post-task: 110-120 min) were realigned to the first frame in pre-task condition. Intra-condition registrations between the frames were performed, and average image for each condition was created and registered to the pre-task image (inter-condition registration). Pre-task PET image was then co-registered to own MRI of each participant and transformation parameters were reapplied to the others. Volumes of interest (VOI) for dorsal putamen (PU) and caudate (CA), ventral striatum (VS), and cerebellum were defined on the MRI. Binding potential (BP) was measured and DAR was calculated as the percent change of BP during and after the task. SPM analyses on the BP parametric images were also performed to explore the regional difference in the effects of head motion on BP and DAR estimation. Results: Changes in position and orientation of the striatum during the PET scans were observed before the head motion correction. BP values at pre-task condition were not changed significantly after the intra-condition registration. However, the BP values during and after the task and DAR were significantly changed after the correction. SPM analysis also showed that the extent and significance of the BP differences were significantly changed by the head motion correction and such changes were prominent in periphery of the striatum. Conclusion: The results suggest that misalignment of MRI-based VOI and the striatum in PET images and incorrect DAR estimation due to the head motion during the PET activation study were significant, but could be remedied by the data-driven head motion correction.

• Pediatric Infection and Vaccine
• /
• v.4 no.1
• /
• pp.64-72
• /
• 1997

Purpose : The incidence of tuberculous meningitis in Korean children has been markedly decreased after 1980s, but this disease has still occurred with low rate. Therefore, it may be suspected that delayed diagnosis and treatment will be happened because of lacking of clinical experiences and indistinguishable other meningitis, so it is important to make early diagnosis and treatment of tuberculous meningitis concerning with the prognosis. In this aspect, we conducted study to concern and investigate sustainly about the diagnostic criteria, clinical characteristics, radiological findings, complications, and prognosis of typical or atypical tuberculous meningitis in children. Methods : Forty four children who were hospitalized and treated due to tuberculous meningitis in pediatric wards of Our Lady of Mercy Hospital, St. Holy Hospital, St. Vincent Hospital and Uijungbu St. Mary Hospital from January 1985 to June 1996 were included in this study. We reviewed medical records of these patients retrospectively. Results : 1) The tuberculous meningitis has occured continuosly since mid-1980s. The highest 2) The diagnosis was made by contact history of active tuberculous patients, positive tuberculin test, responses of antituberculous antibiotics and discovery of Mycobacterium tuberculosis from CSF or other specimens. Among patients, 7 children(16%) were not vaccinated with BCG, and only 18 children(40%) were positive in tuberculin test. 3) The symptoms and signs of our patients on initial examinations were fever, vomiting, headache, lethargy, poor feeding, weight loss, neck stiffness, convulsion, abdominal pain and motor deficits. 4) The findings of initial CSF samples revealed leukocyte $239.5/mm^3$(mean) with lymphocyte predominant, elevated protein levels(mean;259.5mg%) and low sugar level(mean;40.7mg%). And the ratio of CSF/blood sugar was 0.407. But, atypical CSF findings were seen in 31.8% patients. 5) On brain imaging study, 34 out of 39 children had findings of hydrocephalus, basilar meningeal enhancement, infarction and subarachnoidal inflammations etc. On chest X-ray, the findings of miliary tuberculosis(34.1%), normal finding(29.5%), parenchymal infiltrations (11.4%) and calcifications(9.1%) were showed. 6) In neurological clinical stage, there were twenty-six children(59%) in stage 1, fourteen children(32%) in stage 2 and four children(9%) in stage 3. The late sequeles were encountered by 29.5% with mild and 4.6% with severe neurological injury. The most common neurological injury was quadriplegia and the mortality rate was 6.8%. 7) The SIADH was developed in 20 children(45.5%) after the 4th hospital day. Half of all SIADH patients were symptomatic. Conclusion : Tuberculosis meningitis is still an important extrapulmonary disease with high morbidity and mortality. Early diagnosis with clinical contact history of active tuberculosis and radiological imaging examinations and early treatments are essential in order to prevent and decrase the rate of late sequeles and death.

• Journal of Music and Human Behavior
• /
• v.4 no.2
• /
• pp.84-105
• /
• 2007

Cerebral palsy is a collection of motor disorders resulting from damage to the central nervous system that arise in multiple handicaps including cognitive disorders, speech disorders, epilepsy, perception disorders, and emotion disorders. Today spastic cerebral palsy has become more prevalent because intensive care for newborns has resulted in higher survival rates for very small premature babies. Since the children grow the fastest in order for a development during one year after birth, the therapeutic intervention is provided as early as possible to the children with cerebral palsy. After seven year old, there is no effect of intervention. So, the necessity of early intervention to spastic cerebral palsied infants is increasing. The purpose of this study is to develop the music therapy activity program using the techniques of neurological music therapy(NMT), the therapeutic application of music to dysfunctions due to neurologic disease of the human nervous system, for rudimentary movement phase of spastic cerebral palsied infant. This music therapy activity program was developed on the basis of the major developmental tasks of the rudimentary movement phase, the period that children can acquire the most basic movement function at the 0 to 2. Then the developmental characteristics of spastic cerebral palsy were applied to this music therapy activity program. This music therapy activity program was classified to three domains, those are stability, locomotion, and manipulation. This study has been consisted of three steps, those are the development of the activities, the evaluation of the activities by th panels, and the adjustment and complement of the activities. Reviewing literatures and interviews were done for the development of the activities, and the evaluation the activities was done by seven music therapists. In the evaluation steps, the questionnaire was used for estimating the content validity and application efficiency. The adjustment and complement of the activities were evaluated by the panels who were participating in the music therapy for cerebral palsied children in the clinical setting, and the results of the adjustment and complement were confirmed by the panels. The evaluation was presented in a mean value with the comment of the panels. In conclusion, the music therapy activity program for the spastic cerebral palsied infants using the techniques of NMT was developed on the basis of the major developmental tasks of the rudimentary movement phase. The program is comprised of 38 activities, those are 14 activities for developing the stability, 10 activities for developing the locomotion, and 14 activities for developing the manipulation. The programed activities would bring out the answers in the affirmative for the conformance with infants' development phase, the harmony between the objective and the activity, the conformance with the cerebral palsied infants, the properness of the music and the instruments, and the utility in the clinic field. This results mean that this developed music activity program is appropriate to help spastic cerebral palsied infants progress their movement development by stages.