• Journal of the Korean Institute of Intelligent Systems
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• v.24 no.6
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• pp.609-614
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• 2014

Cerebellar ataxia is a steadily progressive neurodegenerative disease associated with loss of motor control, leaving patients unable to walk, talk, or perform activities of daily living. Direct motor instruction in cerebella ataxia patients has limited effectiveness, presumably because an inappropriate closed-loop cerebellar response to the inevitable observed error confounds motor learning mechanisms. Recent studies have validated the age-old technique of employing motor imagery training (mental rehearsal of a movement) to boost motor performance in athletes, much as a champion downhill skier visualizes the course prior to embarking on a run. Could the use of EEG based BCI provide advanced biofeedback to improve motor imagery and provide a "backdoor" to improving motor performance in ataxia patients? In order to determine the feasibility of using EEG-based BCI control in this population, we compare the ability to modulate mu-band power (8-12 Hz) by performing a cued motor imagery task in an ataxia patient and healthy control.

• The Journal of the Korea Contents Association
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• v.10 no.4
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• pp.236-246
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• 2010

The 3-Acetylpyridine(3-AP) induces cerebellar injury which is chemoablation of the inferior olive nucleus. The purpose of this study was to investigate the effects of exercise(Ex) and electroacupuncture(EA) on muscle activity of hindlimb and serum brain derived neurotrophic factor(BDNF) in the ataxia rats by the 3-AP. 12-week-aged male Sprague-Dawley rats were randomly divided into the 5 groups: Control, 3-AP, 3-AP+Ex, 3-AP+EA and 3-AP+Ex+EA groups. Maximal Height Vertical Jump(MHVJ) was significantly decreased in the 3-AP compared with control group, and it was increased in Ex, EA and Ex+EA groups than 3-AP group. The muscle activity of hindlimb was significantly increased in the 3-AP groups than control group, also it was most decreased in Ex+EA group. The concentration of BDNF of the serum was significantly decreased in the Ex, EA and Ex+EA groups than 3-AP group. The results of this study showed that dynamic exercise and electroacupuncture have a positive effect on functional recovery and in the ataxia rats by the 3-AP.

• Journal of Industrial Convergence
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• v.22 no.3
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• pp.51-65
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• 2024

This study aims to understand positive changes in balance and gait function and difficulties in the instructor's guidance process through repetitive basic motor skill-based physical activities targeting people with cerebellar ataxia. For this purpose, five adults with cerebellar ataxia were selected as research subjects, and their three instructors participated as research participants. To collect quantitative data, the average and standard deviation were examined through pre-and post-evaluation of the research participants' physical activity classes for 16 weeks. The mean and standard deviation of the collected data were calculated using the Shapiro-Wilk test in the SAS 9.1 statistical program (p<.05). As a qualitative data collection method, the cultural description method of developmental research(DSR) proposed by Spradley(1980) was adopted, and the collected data were analyzed inductively according to the analysis method of Mertens(1990). Through this, 31 concepts, 10 subcategories, and 4 categories were discovered. As a result, the difficulties experienced by the research participants included insufficient guidance environment, dissatisfaction of consumers, difficulty in guidance, and non-cooperation of colleagues. Based on these research results, it was found that institutional, legal, and policy support should be provided not only to public institutions but also to private physical activity institutions that can use vouchers in order to maintain the balance of adults with cerebellar ataxia as well as to guide their physical activities.

• The Journal of the Korea Contents Association
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• v.18 no.11
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• pp.634-642
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• 2018

People with spinocerebellar ataxia, a hereditary and progressive neurogenic disorder, suffer from ataxic dysarthria due to cerebellar dystrophy. This study was designed to examine if intensive motor speech treatment yields improvement in progressive ataxic dysarthria and if then, to investigate magnitude of therapeutic effect. SPEAK $OUT!^{(R)}$ was provided to a 55-year old female diagnosed with SCA for improving motor speech functions. Magnitude of therapeutic effect was large in changes of MPT and vocal intensity across speech tasks. Small effect size was found in changes of fundamental frequency, however, large therapeutic effect was observed in changes of frequency range. In addition, improvement of vocal quality based on jitter, shimmer, and HNR was observed with large therapeutic effect size and vowel space was expanded, particularly, due to F1. Lastly, VHI scores were decreased. Intensive motor speech treatment, called as SPEAK $OUT!^{(R)}$ was effective enough to observe improvement in vocal intensity, frequency range, and vocal quality, expanding vowel space and lowering VHI scores. Based on the results of this case study, further efficacy evaluation of SPEAK $OUT!^{(R)}$ for improving progressive ataxic dysarthria in people with SCA is required.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.3 no.1
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• pp.28-31
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• 2003

• Clinical and Experimental Reproductive Medicine
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• v.34 no.3
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• pp.179-188
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• 2007

Objectives: Many neurological diseases are known to be caused by expansion of trinucleotide repeats (TNRs). It is hard to diagnose the alteration of TNRs with single cell level for preimplantation genetic diagnosis (PGD). In this study, we describe methods optimized for PGD of TNRs related diseases such as Huntington's disease (HD), spinocerebellar ataxia 3 (SCA3) and fragile X syndrome (FXS). Methods: We performed the preclinical assays with heterozygous patient's lymphocytes by single cell PCR strategy. Fluorescent semi-nested PCR and fragment analysis using automatic genetic analyzer were applied for HD and SCA 3. Whole genome amplification with multiple displacement amplification (MDA) method and fluorescent PCR were carried out for FXS. Amplification and allele drop-out (ADO) rate were evaluated in each case. Results: The fluorescent semi-nested PCR of single lymphocyte showed 100.0% of amplification and 14.0% of ADO rate in HD, and 94.7% of amplification and 5.6% of ADO rate in SCA3, respectively. We could not detect the PCR product of CGG repeats in FXS using the fluorescent semi-nested PCR alone. After applying the MDA method in FXS, 84.2% of amplification and 31.3% of ADO rate were achieved. Conclusions: Fluorescent semi-nested PCR is a reliable method for PGD of HD and SCA3. The advanced MDA method overcomes the problem of amplification failure in CGG repeats of FXS case. Optimization of methods for single cell analysis could improve the sensitivity and reliability of PGD for complicated single gene disorders of TNRs.

• Proceedings of the Korean Information Science Society Conference
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• 1998.10c
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• pp.291-293
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• 1998

운동거리이상(dysmetria)은 운동경로, 속도 및 힘에 대한 조절 능력이 감소되는 것으로서 운동 기능에 심각한 지장을 초래할 수 있다. 본 논문에서는 운동거리이상을 과학적으로 계측하고 분석함으로써 환자의 운동 조절능력을 정량적으로 평가할 수 있는 도구를 개발하였다. 이를 위하여, 터치 스크린상에 그려진 수직 및 수평선의 모든 위치좌표를 추출하기 위한 거북이 알고리즘을 제안하였으며, 이를 이용하여 운동 경로와 운동성을 분석함으로써 상지의 운동실조를 정량적으로 측정할 수 있는 알고리즘을 제안하고 구현하였다.

• The Korean Journal of Pharmacology
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• v.32 no.3
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• pp.293-300
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• 1996

During the development of hepatic encephalopathy after thioacetamide (TAA) injection to rat, EEG was recorded at two different states: without or with tactile stimulation of tail at regular intervals. Calculations based on the spectral and band analysis were used. The changes in the power spectra and bands were examined in 3 different behavioral stages: normal, mild ataxia and severe ataxia. In normal rats, the stimulation produced the increase in the power of the theta $(3.5{\sim}8\;Hz)$ and the gamma $(30{\sim}50\;Hz)$ bands. These changes could not be produced in rats with the mild and severe ataxia. The changes in the power of the theta band occurred earlier than those of the beta3 and the gamma bands in the stimulated state. Gradual decreases in the spectral power of the beta3 $(21{\sim}30\;Hz)$ and the gamma bands were correlated with the progress of the stages from normal condition to mild to severe ataxia in both unstimulated and stimulated states. The results indicate that the spectral and band analysis used in this study can quantify the severity of the neurological malfunction during HE.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.3 no.1
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• pp.32-37
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• 2003

Ornithine transcarbamylase(OTC) deficiency is the most common of all the urea cycle disorders. In this X-linked disorder, the hemizygote males are more severely affected than heterozygote females. The Heterozygote female may have mild episodic hyperammonemia symptoms in late infancy or childhood(late onset) or no clinical manifestations. Here we report a 6 year-old girl with late onset OTC deficiency who showed recurrent episodic lethargy, mental confusion and ataxia. On mutation analysis using DNA sequencing after PCR amplification of the 10 exons of OTC gene, G to T transversion in codon 221, causing substitution of asparagine for lysine was detected in exon 6.

• The Journal of Pediatrics of Korean Medicine
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• v.19 no.2
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• pp.41-50
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• 2005

Objective : The autosomal dominent cerebellar ataxia(ADCA) is an unusal familial herediatary disorder that has been called olivopontoerebellar atrophy. Recently ADCA referred to as spinocerebellar ataxia(SCA) by molecular genetic characteristics. The purpose of this study is to focus on the improvement of clinical symptoms in SCA patient by oriental medical treatment. Materials & Methods : We experienced a case of the 6-year-old female patient with SCA and the MRI showed atrophy of cerebellum. The patient's chief symptoms come within the purview of five kinds of retardation and five kinds of flaccidity. We treated her with herb medicine (Yukmijihwang-tang gamibang), acupuncture, scalp acupuncture. After we measured the progress of general condition by MBI(Modified Bathel Index). Results : After oriental medical treatment, chief symptoms (ataxia, weakness of low extremities, dysarthria, etc.) and general condition were improved. Conclusion : We suggest that oriental medical therapy is effective to the possibility of treatment on SCA, but more clinical study and observation should be needed.