• 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.

• Journal of Pharmacopuncture
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• v.12 no.1
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• pp.53-65
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• 2009

Objectives : This study was to investigate the effect of Oriental medical treatment on ALS. Methods : We investigated 12 ALS patients which were admitted to Gwang-Ju O.M. hospital from Oct. 14, 2008 to Nov. 14, 2008. All patients were treated by SAAM-acupuncture, herb medication, Bee venom Pharmacopuncture therapy, Needle-embedding therapy, etc. We evaluated patients using the Amyotrophic lateral sclerosis Functional Rating Scale-Revised(ALSFRS-R), Medical Research Council (MRC) Scale. Results : After 30 days, mean ALSFRS-R score of patients was improved from $28.42{\pm}7.83$ to $29.08{\pm}7.99$, and mean MRC Scale of patients was improved from $24.79{\pm}8.37$ to $25.34{\pm}8.45$. But in both cases, the variation was not statistically significant. After 30 days, mean ALSFRS-R score and mean MRC Scale of patients was more improved in subjects with bulbar-onset, onset age: 51-60yrs., disease duration: 24-48mo. And the results showed partially significant difference. Conclusions : We think that the results of this case be a pilot study that proves the effect of Oriental Medical treatment on ALS.

• 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.

• Perspectives in Nursing Science
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• v.8 no.1
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• pp.62-72
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• 2011

Purpose: The purpose of this study was to investigate QOL and the factors related to family caregivers who care for Amyotrophic Lateral Sclerosis (ALS) patients. Methods: The subjects were 83 family caregivers caring for ALS patients who visited the neurology outpatient section of a tertiary hospital located in Seoul between January and July of 2008. Their demographic characteristics were assessed and data were collected using the amyotrophic lateral sclerosis functional rating scale (ALSFRS), the caregiver burden inventory (CBI), HADS, and SF-36. Results: The mean score of the physical health component (PHC) of the family caregiver's QOL was $62.6{\pm}24.4$, and the mean score of the mental health component (MHC) of their QOL was $57.7{\pm}22.4$. The mean score of caregiver burden was $76.5{\pm}30.7$. Anxious family caregivers amounted to 55.4% and depressed family caregivers accounted for 63.9% overall. In a multivariate analysis, the PHC of QOL was explained by caregiver burden (41.1%), depression (9.4%), caregiver gender (3.8%), and caregiver age (3.1%). Anxiety (39.8%), caregiver burden (10.9%), patient gender (5.4%) and depression (1.7%) were predictive factors of the MHC of QOL. Conclusion: Caregiver burden and depression affected both the PHC and the MHC. Caregiver burden affected the PHC more than it did the MHC of QOL, but anxiety affected the MHC more than it did the PHC of QOL.

• Molecules and Cells
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• v.47 no.1
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• pp.100005.1-100005.15
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• 2024

Amyotrophic lateral sclerosis is a devastating neurodegenerative disease with a complex genetic basis, presenting both in familial and sporadic forms. The hexanucleotide (G₄C₂) repeat expansion in the C9orf72 gene, which triggers distinct pathogenic mechanisms, has been identified as a major contributor to familial and sporadic Amyotrophic lateral sclerosis cases. Animal models have proven pivotal in understanding these mechanisms; however, discrepancies between models due to variable transgene sequence, expression levels, and toxicity profiles complicate the translation of findings. Herein, we provide a systematic comparison of 7 publicly available Drosophila transgenes modeling the G₄C₂ expansion under uniform conditions, evaluating variations in their toxicity profiles. Further, we tested 3 previously characterized disease-modifying drugs in selected lines to uncover discrepancies among the tested strains. Our study not only deepens our understanding of the C9orf72 G₄C₂ mutations but also presents a framework for comparing constructs with minute structural differences. This work may be used to inform experimental designs to better model disease mechanisms and help guide the development of targeted interventions for neurodegenerative diseases, thus bridging the gap between model-based research and therapeutic application.

• The Journal of Churna Manual Medicine for Spine and Nerves
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• v.5 no.2
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• pp.103-111
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• 2010

Objectives : This study was performed to report the effect of oriental medical treatment in the lower back pain(LBP) with amyotrophic lateral sclerosis(ALS). Methods : A 32-years-old man who diagnosed as ALS was admitted with LBP. We treated him by burning acupuncture, herbal medicine, acupuncture, electroacupuncture, moxibustion, cupping therapy and physical therapy from 11th May 2010 to 10th June 2010. Results : After treatment, there was improvement in LBP. Pain Intensity estimated by visual analog scale(VAS) as percentile Pain Intensity Difference(PID), Korean Owestry Disbility Index(KODI) was also improved. Korean version of Amyotrophic lateral sclerosis Functional rating scale-revised(K-ALSFRS-R) shows that the patient's physical ability has been improved. Conclusions : Our study suggests that oriental medical treatment are significantly effective in the LBP due to ALS. And further studies will be aid to identify underlying mechanism of treatment.

• Journal of Life Science
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• v.32 no.8
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• pp.611-621
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• 2022

Dimethyl sulfoxide (DMSO) is commonly used as control or vehicle solvent in preclinical research of neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS) due to its ability to dissolve lipophilic compounds and cross the blood brain barrier. However, the biochemical effects of DMSO on the outcomes of preclinical research are often overlooked. In the present study, we investigated whether the long-term oral administration of 5% DMSO affects the neurological, functional, and histological disease phenotype of the copper/zinc superoxide dismutase glycine 93 to alanine mutation (SOD1-G93A) mouse model of amyotrophic lateral sclerosis. SOD1-G93A transgenic mice showed shortened survival time and reduced motor function. We found that administration with DMSO led to increased mean survival time, reduced neurological scores, and improved motor performance tested using the rotarod and grip strength tests. On the other hand, DMSO treatment did not attenuate motor neuron loss in the spinal cord and denervation of neuromuscular junctions in the skeletal muscle. These results suggest that DMSO administration could improve the quality of life of the SOD1-G93A mouse model of ALS without affecting motor neuron denervation. In conclusion, the use of DMSO as control or vehicle solvent in preclinical research may affect the behavioral outcomes in the SOD1-G93A mouse model. The effect of the vehicle should be thoroughly considered when interpreting therapeutic efficacy of candidate drugs in preclinical research.

• Proceedings of the PSK Conference
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• 2003.10b
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• pp.79.2-79.2
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• 2003

Minocycline is known to protect neurons from microglia-mediated cell death in many experimental models of brain diseases including ischemic stroke, Huntington's disease (HD), amyotrophic lateral sclerosis (ALS), traumatic brain injury, multiple sclerosis, and Parkinson's disease. Activation of caspase-2, 3, 8, and 9 was evident within 2-8 hr following oxidative insult with 0.5 mM hydrogen peroxide in PC12 cells. Minocycline significantly attenuated activation of these caspases up to 18 hr, resulting a significant increase in cell viability as assessed by MTT assay. (omitted)

• Annals of Clinical Neurophysiology
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• v.2 no.2
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• pp.107-113
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• 2000

Background & Objectives : Motor evoked potentials(MEPs) to magnetic trans cranial stimulation were performed to evaluate upper motor neuron involvement and relationship to lower motor neuron involvement in motor neuron disease patients. Method : MEPs were obtained in the 17 consecutive patients with motor neuron disease. These patients were divided into three group based on clinical evidence of upper and lower motor neuron involvement, bulbar symptom; amyotrophic lateral sclerosis(ALS), progressive muscular atrophy(PMA), progressive bulbar palsy(PBP). MEPs were recorded from abductor pollicis brevis and abductor hallucis muscles. Abnormal MEPs were defined by delayed central motor conduction time or absent MEP. Results : MEPs were abnormal in 64%(11/17) of patients; 100%(7/7) in ALS, 64%(4/7) in PMA, 0%(0/3) in PBP respectively. In 68 total recording muscles, 34 muscles had evidence of motor weakness and showed abnormal responses in 59%(20/34). Whereas 34 muscles with normal strength, only 3%(1/34) of muscles showed abnormal response. Conclusion : MEPs are well correlated with upper motor neuron signs in ALS and may detect masking upper motor neuron signs in PMA. The muscles with lower motor neuron sign(weakness) usually relate with abnormal MEPs reponses.

• The Journal of Korean Physical Therapy
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• v.19 no.5
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• pp.77-85
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• 2007

Purpose: This study was to investigated the effect of physical function and quality of life through physical therapy and occupational therapy by Bobath approach to patient with ALS. Methods: The data of 1 patient with ALS, who visited D hospital in Busan, was collected from June 5, 2006 to August 19, 2007. To determine the physical function and quality of life, Berg Balance Scale (BBS), Gait, Skate. Purdue Pegboard, WHOQOL-BREF, COPM were used. Results: BBS score was increased 0 to 33 in the pre and post test. Gait distance was increased 0m to 500m in pre and post test. Skate score was increased 13 to 22 in the pre and post test, Purdue Pegboard score was increased 0 to 3 in the pre and post test, WHOQOL-BREF was increased 1.92 to 2.46 in the pre and post test. Average COPM score of performance and satisfaction grade of pre test score was 1 then Post test score of performance score increased to 5.7 and satisfaction increased to 4. Conclusion: Physical therapy and Occupational therapy increase physical function and quality of life in ALS patients. Physical therapy and occupational therapy should study more about patients with ALS.