• Physical Therapy Korea
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• v.8 no.4
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• pp.71-80
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• 2001

근위축성 측색 경화증 (amyotrophic lateral sclerosis: ALS) 환자에게 있어 호흡기능장애는 죽음에 이르게 하는 주요 원인 중 하나이다. 본 연구는 근위축성 측색 경화증이 있으며 호흡기능이 약화되어 있는 51세의 여성 환자를 대상으로 호흡운동 치료를 시행한 후 폐기능(pulmonary function)이 증진되었는지를 알아보고자 실시하였다. 연구 대상자는 6주간의 호흡운동 치료 프로그램에 참여하였다. 호흡운동 치료 프로그램은 횡경막 호흡(diaphragmatic breathing), 복부근육강화(abdominal mu scles strengthening), 지갑입술 호흡(pursed lip breathing), 그리고 동기 유발성 흡기폐활량계(incentive spirometer)를 이용한 흡기운동 등으로 구성되었다. 폐기능 검사는 이동식 호흡측정기(spirometer: MICROSPIROHI-198)를 이용해서 시행하였다. 또한 하지 에르고미터(cycle- ergometer)를 이용해 운동 시간을 측정함으로써 폐기능의 증진 여부를 알아보았다. 연구 대상자는 6주간의 호흡운동 치료 기간 동안 노력성 폐활량(forced vital capacity: FVC)과 정상 예측치에 대한 노력성 폐활량의 비율(percentage of the predicted forced vital capacity: %FVC), 그리고 하지 에르고미터의 운동 시간에 있어 현저한 증가를 보였다. 그러나 노력성 폐활량에 대한 1초간 노력성 폐활량 비(FEV1/ FVC)에 있어서는 약간의 감소를 보였다. 근위축성 측색 경화증 환자에게 6주간의 호흡운동 치료를 실시한 결과, 폐기능의 증진에 효과적임을 알 수 있었으며, 앞으로 더 많은 연구 대상자에게 그 효과를 알아보는 연구가 필요할 것이다.

• Journal of muscle and joint health
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• v.13 no.2
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• pp.130-139
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• 2006

Purpose: This study was designed to present preliminary data for the development of appropriate nursing care system for the patients with ALS by analyzing their physical function. Method: The clinical data of 36 ALS patients, who visited ALS Clinic of H University Hospital in Seoul, were collected from January, 2006 to August, 2006. To determine the physical function, Norris ALS scale and Appel ALS Rating Scale were used. The data were analyzed by frequency, percentage, mean, standard deviation, range, t-test, ANOVA, using SPSS PC program. Results: The mean score of physical activity, muscle strength, upper extremity function, lower extremity function was 18.08, 27.72, 25.94, 25.19 respectively. There were significant differences in physical activity, muscle strength, and upper extremity function according to sender and comorbid disease(diabetes). Although sites of symptom onset were not statistically significant with all physical function, patients with bulbar onset showed relatively severe physical disabilities. Conclusion: The preliminary data on physical function of patients with ALS would be helpful for the development of ALS nursing guideline system.

• Journal of the Korea Convergence Society
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• v.8 no.6
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• pp.275-281
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• 2017

Human endogenous retroviruses (HERVs) are fossil viruses that began to be assimilated into the human genome some 30~40 million years ago, and now constitute nearly 8% of the human genome. These ancient retroviruses have since accumulated mutations that have rendered them defective; thus, they have been termed junk DNA. However, recent research indicates that not all HERVs remain silent passengers. Although they have not been shown to be causative of any human disease, endogenous retroviral sequences may become expressed under select pathological circumstances such as neurological disorders, including multiple sclerosis (MS), schizophrenia, and Amyotrophic Lateral Sclerosis (ALS); viral infections, including human immunodeficiency virus (HIV) and herpesvirus; and multiple types of cancers. This review focused on the possible interactions of HERVs and neurological diseases.

• Annals of Clinical Neurophysiology
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• v.10 no.2
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• pp.116-118
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• 2008

Mills' syndrome is rare clinical syndrome described in 1900 by Mills, namely ascending (or less often, descending) progressive hemiplegia. Mills' syndrome could be considered as a variant of primary lateral sclerosis (PLS) or amyotrophic lateral sclerosis (ALS), but is still controversial. According to clinical course, imaging and electrophysiological findings, our case is more compatible with Mills' syndrome rather than PLS or ALS. To our knowledge, this is the first report of Mills' syndrome in Korea.

• The Journal of Internal Korean Medicine
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• v.22 no.2
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• pp.279-283
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• 2001

We had one female ALS patient. The patient was diagnosed with ALS by EMG and Symptoms. We diagnosed the patient as Jinshang(筋傷) and Pishenyangxu(脾腎陽虛). We gave Shaoyaogancao-tang jiami(芍藥甘草湯加味) and Sijunzi-tang he Lizhong-tang jiami(四君子合理中湯加味) based on the differentiation of symtoms. We report the changes of the ALS patient's disphagia, lalopathy, and hypotonia after two months treatment with Riluzole and oriental medical treatment.

• Molecules and Cells
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• v.25 no.1
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• pp.55-63
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• 2008

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by the selective death of motor neurons. Mutations in the SOD1 gene are responsible for a familial form of ALS (FALS). Although many studies suggest that mutant SOD1 proteins are cytotoxic, the mechanism is not fully understood. To investigate the role of mutant SOD1 in FALS, human SOD1 genes were fused with a PEP-1 peptide in a bacterial expression vector to produce in-frame PEP-1-SOD fusion proteins (wild type and mutants). The expressed and purified PEP-1-SOD fusion proteins were efficiently transduced into neuronal cells. Neurones harboring the A4V, G93A, G85R, and D90A mutants of PEP-1-SOD were more vulnerable to oxidative stress induced by paraquat than those harboring wild-type proteins. Moreover, neurones harboring the mutant SOD proteins had lower heat shock protein (Hsp) expression levels than those harboring wild-type SOD. The effects of the transduced SOD1 fusion proteins may provide an explanation for the association of SOD1 with FALS, and Hsps could be candidate agents for the treatment of ALS.

• Journal of Acupuncture Research
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• v.29 no.5
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• pp.187-195
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• 2012

Objectives : Amyotrophic lateral sclerosis(ALS) is a progressive and incurable disease that causes degeneration of the motor neurons of the brain stem and spinal cord. The purpose of this study was to report the effectiveness of Sa-am acupuncture treatment on ALS patient. Methods : Following the stabilization period, a ALS patient respiratory figures(Et $C_{O2}$, Sp $_{O2}$, RR, pulse) were measured by using capnography & pulse oximetry for 30 minutes before acupuncture treatment. Acupuncture treatment such as lung tonification($SP_3(+){\cdot}LU_9(+){\cdot}HT_8(-){\cdot}LU_{10}(-)$), heart tonification($HT_9(+){\cdot}LR_1(+){\cdot}SI_8(-){\cdot}KI_{10}(-)$), liver tonification ($KI_{10}(+){\cdot}LR_8(+){\cdot}LU_8(-){\cdot}LR_4(-)$) were conducted for 5 days. Each Sa-am acupuncture treatment conducted at AM 7 : 00, AM 11 : 00, PM 4 : 30 of the day. During acupuncture treatment, the patient respiratory figures were measured equally for 30 minutes. The patient was treated by using Dong-bang acupuncture needles($0.30{\times}4.0$) and also stimulated using infra red. Results : The value of Et $C_{O2}$ has decreased more after Sa-am acupuncture lung tonification treatment than others. The value of pulse has decreased more after Sa-am acupuncture heart tonification treatment than others. The value of raspiration rate has decreased more after Sa-am acupuncture liver tonification treatment than others. And the value of Sp $O_2$ has increased more after Sa-am acupuncture liver tonification treatment than others. Conclusions : Although this study was subject to a few limitations, but it shows that Sa-am acupuncture treatment for ALS patients has a meaningful effect. This study needs to be developed further using a larger sample size to obtain more valuable and meaningful data.

• The Korean Journal of Physiology and Pharmacology
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• v.3 no.1
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• pp.101-117
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• 1999

Amyotrophic lateral sclerosis (ALS) is a degenerative neuromuscular disease of unknown etiology in which the upper and lower motor neurons are progressively destroyed. Recent evidences support the role of autoimmune mechanisms in the pathogenesis of ALS. This study investigated the effects of sera from ALS patients on neuromuscular transmission in phrenic nerve-hemidiaphragm preparations and on calcium currents of single isolated dorsal root ganglion (DRG) cells in mice. Mice were injected with either control sera from healthy adults or ALS sera from 18 patients with ALS of sporadic form, for three days. Miniature end plate potential (MEPP) and nerve-evoked end plate potential (EPP) were measured using intracellular recording technique and the quantal content was determined. Single isolated DRG cells were voltage-clamped with the whole-cell configuration and membrane currents were recorded. Sera from 14 of 18 ALS patients caused a significant increase in MEPP frequency in normal Ringer's solution $(4.62{\pm}0.14\;Hz)$ compared with the control $(2.18{\pm}0.15\;Hz).$ In a high $Mg^{2+}/low\;Ca^{2+}$ solution, sera from 13 of 18 ALS patients caused a significant increase in MEPP frequency, from $2.18{\pm}0.31$ Hz to $6.09{\pm}0.38$ Hz. Sera from 11 of 18 patients produced a significant increase of nerve-evoked EPP amplitude, from $0.92{\pm}0.05$ mV to $1.30{\pm}0.04$ mV, while the other seven ALS sera did not alter EPP amplitude. In the ALS group, EPP quantal content was also elevated by the sera of 14 patients (from $1.49{\pm}0.07$ to $2.35{\pm}0.07).$ MEPP frequency and amplitude in wobbler mouse were $4.03{\pm}0.53$ Hz and $1.37{\pm}0.18$ mV, respectively, which were significantly higher than those of wobbler controls (wobblers without the symptoms of wobbler). Sera from ALS patients significantly reduced HVA calcium currents of DRG cells to 42.7% at -10 mV. Furthermore, the inactivation curve shifted to more negative potentials with its half-inactivation potential changed by 6.98 mV. There were, however, significant changes neither in the reversal potential of $I_{Ca}$ nor in the I-V curve. From these results it was concluded that: 1) The serum factors of sporadic ALS patients increase neuromuscular transmission and can alter motor nerve terminal presynaptic function. This suggests that ALS serum factors may play an important role in the early stage of ALS, and 2) Calcium currents in DRG cells were reduced and rapidly inactivated by ALS sera, suggesting that in these cells, ALS serum factors may exert interaction with the calcium channel.

• 대한상한금궤의학회지
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• v.4 no.1
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• pp.67-74
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• 2012

Objective : This research is to prove the effectiveness of Jinmutang in curing muscular atrophy Method : To achieve the goal of this research, we gave Jinmutang to the selected patients and observed the progress. Results & Conclusions: The results indicate the followings 1. After the treatment with Jinmu-tang, the symptoms of muscular atrophy were significantly improved. 2. JinmuTang based on sanghanron, as shown in the example above, has an effect on Muscle atrophy of the legs, But we need to study this pharmacologic and biological mechanism.

• Journal of Korean Academy of Nursing
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• v.41 no.3
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• pp.354-363
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• 2011

Purpose: The purpose of this cross-sectional study was to examine the relationship between characteristics of severe ALS patient-caregiver couples and health related quality of life (HRQoL) in family caregivers. Methods: The participants in this study were 89 pairs of ALS patients using ventilators and a family caregiver. The characteristics of the ALS patients and caregivers, Korean-Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised, Zarit Burden Interview and SF-36 were measured in this study. The data were collected from August 2008 to April 2009. Descriptive statistics, Pearson correlation coefficients, and canonical correlation were used for data analysis. Results: The physical component summary and mental component summary of the HRQoL score for family caregivers were $147.49{\pm}31.63$ and $129.09{\pm}35.83$, respectively. HRQoL for caregivers was related to characteristics of the ALS patient-caregiver couples, such as patient's gender, caregiver's age, gender, marital status, daily time spent in caregiving and burden with one significant canonical variable. The significant variate showed that the lower the age, the time spent in caregiving and the burden of caregivers, the higher the HRQoL of caregivers. Conclusion: The support systems for caregivers considering caregiver characteristics such as demographics and burden should be implemented to improve the HRQoL of caregivers.