• Journal of Korean Academy of Nursing
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• v.48 no.4
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• pp.454-464
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• 2018

Purpose: The purpose of this study was to identify factors influencing psychosocial well-being in family caregivers of patients with amyotrophic lateral sclerosis (ALS). Methods: A descriptive correlational design was used. The transactional model of stress and coping was used to investigate the psychosocial well-being of 137 family caregivers of patients with ALS. Data were collected through self-reported questionnaires from January to November 2016. Data were analyzed using an independent t-test, one-way ANOVA, Pearson's correlation, and hierarchical multiple regression analysis with the SPSS WIN 21.0 program. Results: The regression model had an adjusted $R^2$ of .49, which indicated that meaning-focused coping, social support, ALS patient-family caregiver relationship (especially a spousal relationship), and tracheostomy were significant predictors of caregivers' psychosocial well-being. Conclusion: Meaning-focused coping and social support significantly influenced caregivers' psychosocial well-being. Therefore, interventions to improve caregivers' psychosocial well-being must focus on increasing meaning-focused coping and social support resources.

• The Journal of Internal Korean Medicine
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• v.28 no.4
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• pp.937-947
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• 2007

Amyotrophic lateral sclerosis (ALS) is a progressive disorder that causes degeneration of motor neurons of the brain and spinal cord. ALS is a progressive, fatal neuromuscular disease characterized by loss of motor neurons leading to muscle weakness. Sensation and mental function stay intact during the course of the disease. ALS is characterized by both upper and lower motor neuron damage. Diagnosis includes magnetic response imaging (MRI) electromyogram (EMG), muscle biopsy, and blood test. There is no cure for ALS. We recently observed three cases of ALS. The patients were diagnosis with ALS by EMG and symptoms. This report was conducted to evaluate how oriental medical treatment can affect ALS. We report the change of their symptoms through oriental medical treatment compared with taking riluzole.

• Journal of Sasang Constitutional Medicine
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• v.26 no.2
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• pp.156-164
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• 2014

Objectives The purpose of this study was to analyze the Sasang constitution and psychological traits of amyotrophic lateral sclerosis (ALS) patients and to utilize the results in treatment and management. Methods The differences of Sasang Constitution distribution, psychological characteristics, comorbid disorders between ALS group of 26 patients and general group of 1132 persons were investigated and analyzed. Results There was no significant different distribution of Sasang Constitution between ALS group and general group but there was Yang-like personality tendency in ALS group than general group. In overall constitution, ALS group had the high prevalence rate of musculoskeletal disorders and diabetes than general group. In Soyangin constitution, ALS group had a high prevalence rate of musculoskeletal disorders than genral group. In Taeeumin constitution, ALS group had a high prevalence rate of diabetes and hypertension than general group. Conclusion ALS patients have the Yang-like personality. It is due to the adaptation of personality on the environmental change or coping strategy on diseases rather than the characteristics of congenital Sasang Constitution.

• Journal of Korean Academy of Nursing
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• v.45 no.2
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• pp.202-210
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• 2015

Purpose: The purpose of this study was to describe depression, caregiving burden and the correlation of the two variables in the families of patients with amyotrophic lateral sclerosis (ALS) and to clarify factors predicting caregiving burden. Methods: A descriptive and cross-sectional study was conducted with 139 family members who provided care to patients with ALS. The characteristics of patients and families, Korean-Beck Depression Inventory (K-BDI), Korean version of Zarit Burden Interview (K-ZBI) and Korean-Amyotrophic Lateral Sclerosis Functional Rating Scale - Revised (K-ALSFRS-R) were used as study measures. Results: The mean score for K-BDI was 19.39 out of 63 suggesting sub-clinical depression and 38.2% of the family members exhibited depression. The mean score for K-ZBI was 66.03 out of 88. The predictors for K-ZBI were K-BDI, age of family member, length of time spent per day in caring, relationship to patient and K-ALSFRS-R. Conclusion: The results of this study suggest that levels of depression and caregiving burden are high among family members caring for patients with ALS. As depression is associated with caregiving burden, screening and emotional supports should be provided to reduce the burden of care for these family. Support programs to alleviate the care burden are also needed, considering family demographics, time per day in caring giving and K-ALSFRS-R.

• Journal of Acupuncture Research
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• v.30 no.5
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• pp.185-192
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• 2013

Objectives : Weight loss and loss of appetite in amyotrophic lateral sclerosis patients are common symptoms, these are associated with survival as well as nutritional condition. The changes in weight and appetite were reported after treatment using Megestrol acetate and Korean medical treatments. Methods : Amyotrophic lateral sclerosis patient with weight loss and loss of appetite were treated by administering megestrol acetate a known anti-malignant tumor agent and Korean medical treatments such as acupuncture, pharmacopuncture and herbal medicine. The changes in weight and appetite were checked using body mass index(BMI) and simplified nutritional appetite questionnaire(SNAQ). Results : There was a more than 17 kg increase in weight. BMI increased from 15.94 $kg/m^2$ to 21.97 $kg/m^2$ and SNAQ score increased from 7 to 16. Owing to several side effects, Megestrol acetate was stopped after which only Korean medical treatments were provided. After which the lasting effects in BMI and appetite could be seen. Conclusion : There are few studies on weight loss and loss of appetite in ALS patients. Using only megestrol acetate can cause several considerable side effects. Which respect to this, the findings in this study could open up new clinical guideline possibilities.

• Biomolecules & Therapeutics
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• v.29 no.5
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• pp.498-505
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• 2021

Amyotrophic lateral sclerosis (ALS) is a lethal neurological disorder characterized by the deterioration of motor neurons. The aim of this study was to investigate alteration of cationic amino acid transporter (CAT-1) activity in the transport of lysine and the pretreatment effect of lysine on pro-inflammatory states in an amyotrophic lateral sclerosis cell line. The mRNA expression of cationic amino acid transporter 1 was lower in NSC-34/hSOD1^G93A (MT) than the control cell line (WT), lysine transport is mediated by CAT-1 in NSC-34 cell lines. The uptake of [³H]L-lysine was Na⁺-independent, voltage-sensitive, and strongly inhibited by inhibitors and substrates of cationic amino acid transporter 1 (system y⁺). The transport process involved two saturable processes in both cell lines. In the MT cell line, at a high-affinity site, the affinity was 9.4-fold higher and capacity 24-fold lower than that in the WT; at a low-affinity site, the capacity was 2.3-fold lower than that in the WT cell line. Donepezil and verapamil competitively inhibited [³H]L-lysine uptake in the NSC-34 cell lines. Pretreatment with pro-inflammatory cytokines decreased the uptake of [³H]L-lysine and mRNA expression levels in both cell lines; however, the addition of L-lysine restored the transport activity in the MT cell lines. L-Lysine exhibited neuroprotective effects against pro-inflammatory states in the ALS disease model cell lines. In conclusion, studying the alteration in the expression of transporters and characteristics of lysine transport in ALS can lead to the development of new therapies for neurodegenerative diseases.

• BMB Reports
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• v.50 no.11
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• pp.535-536
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• 2017

A novel approach has been used to identify functional interactions relevant to human disease. Using high-throughput human-yeast genetic interaction screens, a first draft of disease interactome was obtained. This was achieved by first searching for candidate human disease genes that confer toxicity in yeast, and second, identifying modulators of toxicity. This study found potentially disease-relevant interactions by analyzing the network of functional interactions and focusing on genes implicated in amyotrophic lateral sclerosis (ALS), for example. In the subsequent proof-of-concept study focused on ALS, similar functional relationships between a specific kinase and ALS-associated genes were observed in mammalian cells and zebrafish, supporting findings in human-yeast genetic interaction screens. Results of combined analyses highlighted MAP2K5 kinase as a potential therapeutic target in ALS.

• Journal of Acupuncture Research
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• v.31 no.3
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• pp.67-73
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• 2014

Objectives : Amyotrophic lateral sclerosis(ALS) is a progressive neurodegenerative disorder characterized by a selective death of motor neuron, leading to respiratory insufficiency. The purpose of this study was to assess the long term respiratory management of ALS patient with respiratory failure. Methods : One ALS patient applying a non-invasive BIPAP ventilator as well as Korean medical treatment such as acupuncture, pharmacopuncture and herbal medicine was measured on $SpO_2$, $EtCO_2$, Vte(expiratorytidalvolume) for 2 years 7 months. Results : The $SpO_2$, $EtCO_2$ of ALS patient were maintained in the normal range for 2 years 7 months. The Vte of ALS patient also wasn't worse in this study. Conclusions : In this study, the long term respiration management, combined administration of Korean medical treatment and non-invasive BIPAP ventilator, could be effective in ALS patient with respiratory failure.

• Annals of Clinical Neurophysiology
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• v.5 no.1
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• pp.21-26
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• 2003

Background & Objectives: Hyperexcitablity of motor system is a well-established characteristic pathophysiologic finding of amyotrophic lateral sclerosis (ALS). Whereas little is known about the source of excitability according to the progression of the disease. We evaluated the excitability and its source in advanced ALS patients using transcranial magnetic stimulation (TMS). Meterial & Methods: Motor evoked potentials (MEP) by TMS were recorded for abductor pollicis brevis muscles in 20 patients, 11 men and 9 women, with ALS. Mean age was $54.2{\pm}12.1years$, and mean disease duration was $13.9{\pm}13.4years$. Serial magnetic stimulations were applied to get the parameters; excitability threshold (ET), amplitude and latency of MEP. We also had a facilitated MEP (fMEP). Results: The parameters were analyzed according to the clinical settings. ET was higher in ALS(mean $63.5{\pm}18.1$) than normal control (mean $46.0{\pm}8.4$, p<0.01). Amplitudes of MEP were reduced in ALS ($2.6{\pm}3.6mV$; control $6.5{\pm}3.1mV$, p<0.01). Duration of the disease and ET showed significant inverse correlation (Spearson correlation coefficient = -0.57, p<0.01). Duration of the disease and fMEP/MEP ratio showed less but also significant inverse correlation (Spearson correlation coefficient, r = -0.52, p < 0.05). Conclusions: Lower ET in advanced ALS patients, in spite of decreased fMEP/MEP ratio, may indicate the hyperexcitability of lower motor neurons in these patients. This study suggests that lower motor neurons is hyperexcitable due to upper motor neuron dysfunction at advanced stage.

• Annals of Clinical Neurophysiology
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• v.15 no.2
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• pp.53-58
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• 2013

Background: The autophagy is the major route for lysosomal degradation of misfolded protein aggregates and oxidative cell components. We hypothesized that rapamycin (autophagy enhancer) would prolong the survival of motor neuron and suppress the disease progression in amyotrophic lateral sclerosis (ALS). Methods: A total of 24 transgenic mice harboring the human G93A mutated SOD1 gene were used. The clinical status involving rotarod test and survival, and biochemical study of ALS mice model were evaluated. Results: The onset of symptoms was significantly delayed in the rapamycin administration group compared with the control group. However, after the clinical symptom developed, the rapamycin exacerbated the disease progression and shortened the survival of ALS mice model, and apoptosis signals were up-regulated compared with control group. Conclusions: Even though further detailed studies on the relevancy between autophagy and ALS will be needed, our results revealed that the rapamycin administration was not effective for being novel promising therapeutic strategy in ALS transgenic mice and exacerbated the apoptosis.