• Annals of Clinical Neurophysiology
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• 제15권2호
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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.

• 대한약침학회지
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• 제12권1호
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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.

• 사상체질의학회지
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• 제26권2호
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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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• 제8권1호
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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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• 제47권1호
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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.

• 척추신경추나의학회지
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• 제5권2호
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• pp.103-111
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• 2010

ALS를 진단받았으며 2010.5.11부터 2010.6.10까지 ${\bigcirc}{\bigcirc}$한방병원에 입원한 요통을 호소하는 남환에 대하여 화침치료, 전침치료, 침치료, 구치료, 약물치료, 이학요법 등을 시행한 결과 K-ODI, VAS, K-ALSFRS-R 등의 평가 항목에 대하여 호전 경향을 보였다. ALS는 기본적으로 진행성 경과를 밟는 질환이기 때문에, 병의 진행을 늦추는 것과 대증치료를 통해 삶의 질을 높이는 것이 중심이 된다고 볼 수 있다. 본 환자의 경우 상기 한의학적 처치에 의해 일정한 효과가 나타났다. ALS에서 수반되는 요통의 호전에 의해 기능수행능력이 향상된 결과라고 볼 수 있다. 이는 한의학적 치료 방법이 ALS 환자의 치료에 있어서 통증을 감소시키고 일상생활에서의 기능수행능력을 향상시킴으로써 삶의 질을 높이는 대중치료의 역할을 수행할 수 있다는 단서라고 할 수 있다. 치료효과의 검증을 위해서는 추후 지속적인 증례 연구가 필요할 것으로 사료된다.

• 생명과학회지
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• 제32권8호
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• pp.611-621
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• 2022

DMSO (dimethyl sulfoxide)는 친유성 화합물을 용해하는 성질과 뇌혈관장벽(Blood-brain barrier)을 투과하는 화학적 특성으로 인해 근위축성 측삭경화증(amyotrophic lateral sclerosis) 등의 퇴행성 뇌신경질환을 타겟으로 하는 전임상 연구에서 용매로 널리 활용되고 있다. 그러나 DMSO를 활용한 연구 결과에 대하여 본 물질에 대한 생화학적 효과는 간과되고 있다. 본 연구에서는 근위축성 측삭경화증의 질환동물 모델인 SOD1-G93A형질 전환 마우스에 5% DMSO를 장기간 경구 투여하여 질병 표현형에 미치는 영향을 생존기간을 포함하여 신경학적, 기능학적, 조직학적으로 분석하였다. DMSO를 투여한 SOD1-G93A 동물군에서 DMSO 비투여군 보다 생존 기간과, 로타로드와 악력 측정으로 평가한 근육 기능이 유의미하게 증가했고, neurological score 가 감소했다. 반면, DMSO 투여군에서 DMSO 비투여군 대비하여 척수 운동 신경 세포와 신경근접합부가 보존되지 않았다. DMSO 투여는 SOD1-G93A형질 전환 마우스의 운동 신경 세포의 조직학적 영향을 미치지 않았지만, 신경 증상 완화와 생존 기간 등 개선된 마우스의 quality of life을 확인하였다. 본 연구 결과, DMSO를 이용한 퇴행성 뇌 질환 전임상 연구 및 후보 약물 효능 평가 시 DMSO의 생화학적 특성에 대한 종합적인 고려가 필요한 것으로 보인다.

• 대한약학회:학술대회논문집
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• 대한약학회 2003년도 Proceedings of the Convention of the Pharmaceutical Society of Korea Vol.2-2
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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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• 제2권2호
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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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• 제19권5호
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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.