• Title/Summary/Keyword: Amyotrophic lateral sclerosis

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Coenzyme Q10: a progress towards the treatment of neurodegenerative disease

  • Kumar, Peeyush;Kumar, Pramod;Ram, Alpana;Kuma, Mithilesh;Kumar, Rajeev
    • Advances in Traditional Medicine
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    • v.10 no.4
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    • pp.239-253
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    • 2010
  • Coenzyme $Q_{10}$ ($CoQ_{10}$, or ubiquinone) is an electron carrier of the mitochondrial respiratory chain (electron transport chain) with antioxidant properties. In view of the involvement of $CoQ_{10}$ in oxidative phosphorylation and cellular antioxidant protection a deficiency in this quinone would be expected to contribute to disease pathophysiology by causing a failure in energy metabolism and antioxidant status. Indeed, a deficit in $CoQ_{10}$ status has been determined in a number of neuromuscular and neurodegenerative disorders. Primary disorders of $CoQ_{10}$ biosynthesis are potentially treatable conditions and therefore a high degree of clinical awareness about this condition is essential. A secondary loss of $CoQ_{10}$ status following HMG-CoA reductase inhibitor (statins) treatment has been implicated in the pathophysiology of the myotoxicity associated with this pharmacotherapy. $CoQ_{10}$ and its analogue, idebenone, have been widely used in the treatment of neurodegenerative and neuromuscular disorders. These compounds could potentially play a role in the treatment of mitochondrial disorders, Parkinson's disease, Huntington's disease, amyotrophic lateral sclerosis, Friedreich's ataxia, and other conditions which have been linked to mitochondrial dysfunction. This article reviews the physiological roles of $CoQ_{10}$, as well as the rationale and the role in clinical practice of $CoQ_{10}$ supplementation in different neurological diseases, from primary $CoQ_{10}$ deficiency to neurodegenerative disorders. These will help in future for treatment of patients suffering from neurodegenerative disease.

A Case Report on the Use of Korean Medicine Treatment for a Patient with Dysarthria Caused by Progressive Bulbar Palsy (PBP)

  • Ahn, Jae Hyun;Kim, Sung Yoon;Park, Jun Hyeong;Cho, Jeongjae;Choi, Do Young;Lee, Seung Hoon;Lee, Jae Dong
    • Journal of Acupuncture Research
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    • v.34 no.1
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    • pp.49-58
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    • 2017
  • Objectives : To introduce a rare case of a patient with Progressive Bulbar Palsy (PBP) and suggest the possibility of treatment using electroacupuncture and Korean Medicine. Methods : A 61-year-old man with PBP, complaining of dysarthria, was treated with electroacupuncture and Korean Medicine, from June 16 to July 10, 2015. Improvements in symptoms were measured using the speech mechanism screening test (SMST), measurement of tongue and orbicularis oris motility, and speech handicap index (SHI). Results : The scores of SMST, motility of tongue and orbicularis oris showed a tendency for gradual improvement with 25 days of Korean Medicine Treatment after admission, but conversation was still impossible. In SHI scores, one point increased in the speech domain and one point decreased in the psycho-social domain, and three points increased in the other domain. Conclusion : Electroacupuncture and Korean Medicine Treatment improved dysarthria caused by PBP, but not completely. Korean Medicine Treatment seems effective in the management of accompanying symptoms such as black hairy tongue, dry mouth, and general condition. The symptoms of PBP are similar to those of amyotrophic lateral sclerosis and there is controversy regarding the classification of PBP. The most important aspect of treating a patient with PBP is an early diagnosis and devising appropriate rehabilitation strategies.

Effect of Bee Venom on Glutamate-mediated Excitotoxicity in NSC-34 Motor Neuronal Cells (Glutamate 매개 흥분성 신경독성에 대한 봉독의 NSC-34 신경세포사멸 억제 효과)

  • Lee, Sang-Min;Choi, Sun-Mi;Jung, So-Young;Yang, Eun-Jin
    • YAKHAK HOEJI
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    • v.55 no.5
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    • pp.385-390
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    • 2011
  • Bee venom (BV), which is extracted from honeybees, has been used in traditional Korean medical therapy. Glutamate-mediated excitotoxicity contributes to neuronal death in neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS) or Alzheimer's disease (AD). This study is to investigate the effect of BV on glutamate-induced neurotoxicity on NSC-34 motor neuron cells. To determine the viability of motor neuronal cells, we performed with MTT assays in glutamate-treated NSC-34 cell with BV or without. For the measurement of oxidative stress, DCF assay was used in glutamate-treated NSC-34 motor neuronal cells with BV or without. To investigate the molecular mechanism of BV against glutamate-mediated neurotoxicity in NSC-34 cells, western blot analysis was used. Glutamate significantly decreased cell viability by glutamate dose- or treatment time-dependent manner in NSC-34 cells. However, BV pre-treatment dramatically inhibited glutamate-induced neuronal cell death. Furthermore, we found that BV increased the expression of Bcl-2 protein that is anti-apoptotic protein and reduced the generation of oxidative stress. BV has a neuroprotective role against glutamate neurotoxicity by an increase of anti-apoptotic protein. It suggests that BV may be useful for the reduction of neuronal cell death in neuronal disease models.

A Survey of Hospital-Based Home Healthcare Utilization in Patients using Home Mechanical Ventilator (가정용 인공호흡기 적용 환자의 가정간호서비스 이용실태)

  • Lee, Mi Kyoung;Song, Chong Rye;Oh, Eun Kyung;Yoon, Young Mi
    • Journal of Home Health Care Nursing
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    • v.24 no.2
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    • pp.210-220
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    • 2017
  • Purpose: The study aimed to describe the utilization of home healthcare in patients using home mechanical ventilator(HMV) Method: A descriptive cross-sectional design was used in this study. A Questionnaires were sent to nation wide home healthcare agencies to assess their utilization status of home healthcare. A convenience sample of 158 patients data was reviewed. Result: A total of 88(55.7%) men with the mean age of 51.94(${\pm}19.52$) years were included in the study. Approximately 55.1% of patients at the outpatient department were referred to the home healthcare services after discharge. The underlying diseases were as follows : 129 amyotrophic lateral sclerosis and 27 muscular dystrophies. A total of 155 patients have invasive HMV. Efficient home healthcare nursing activities provided by a highly skilled home healthcare advanced practice nurses(HHCAPN) were tracheotomy and gastrostomy tube management and urinary catheterization. The average frequency of home visit for one patient was 2.52times per month. The duration of home healthcare utilization with >1 year was 82.9%. HHCAPNs have limited knowledge and skill for HMV. Conclusion: The government support is required to provide sufficient home healthcare services to the patients discharged with HMV. HHCAPNs should be properly educated on the effective HMV care.

Design of Computer Access Devices for Severly Motor-disability Using Bio-potentials (생체전위를 이용한 중증 운동장애자들을 위한 컴퓨터 접근제어장치 설계)

  • Jung, Sung-Jae;Kim, Myung-Dong;Park, Chan-Won;Kim, Il-Hwan
    • The Transactions of the Korean Institute of Electrical Engineers D
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    • v.55 no.11
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    • pp.502-510
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    • 2006
  • In this paper, we describe implementation of a computer access device for the severly motor-disability. Many people with severe motor disabilities need an augmentative communication technology. Those who are totally paralyzed, or 'locked-in' cannot use conventional augmentative technologies, all of which require some measure of muscle control. The forehead is often the last site to suffer degradation in cases of severe disability and degenerative disease. For example, In ALS(Amyotrophic Lateral Sclerosis) and MD(Muscular dystrophy) the ocular motorneurons and ocular muscles are usually spared permitting at least gross eye movements, but not precise eye pointing. We use brain and body forehead bio-potentials in a novel way to generate multiple signals for computer control inputs. A bio-amplifier within this device separates the forehead signal into three frequency channels. The lowest channel is responsive to bio-potentials resulting from an eye motion, and second channel is the band pass derived between 0.5 and 45Hz, falling within the accepted Electroencephalographic(EEG) range. A digital processing station subdivides this region into eleven components frequency bands using FFT algorithm. The third channel is defined as an Electromyographic(EMG) signal. It responds to contractions of facial muscles and is well suited to discrete on/off switch closures, keyboard commands. These signals are transmitted to a PC that analyzes in a time series and a frequency region and discriminates user's intentions. That software graphically displays user's bio-potential signals in the real time, therefore user can see their own bio-potentials and control their physiological signals little by little after some training sessions. As a result, we confirmed the performance and availability of the developed system with experimental user's bio-potentials.

Communication Support System for Person with Language Disabilities (중증 언어장애인을 위한 의사소통 시스템)

  • Hong Seung-Wook;Park Su-Hyun
    • Proceedings of the Korean Institute of Information and Commucation Sciences Conference
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    • 2006.05a
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    • pp.324-327
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    • 2006
  • The person who gets a ALS(Amyotrophic Lateral Sclerosis) has language disability and physical disability together. A common first symptom is a painless weakness in a hand, foot, arm or leg, which occurs in more than half of all cases. Other early symptoms include muscle weakness of speech. In the early stage of this disease they can communicate with other persons, but it will become increasingly difficult. In our research we have designed and implemented communication tools for them. We have implemented Chunjiin(the Korean computer keyboard) at PDA(personal digital assistant). And we have also implemented software which is consisted of frequently used words.

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26-Week Repeated Dose Oral Toxicity Study of KCHO-1 in Sprague-Dawley Rats

  • Yang, Muhack;Lee, Seongjin;Wang, Tingting;Cha, Eunhye;Jang, Jongwon;Kim, Dongwoung;Song, Bong-Keun;Son, Ilhong;Kim, Joonyup;Kang, Hyung Won;Kim, Sungchul
    • Journal of Pharmacopuncture
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    • v.22 no.3
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    • pp.192-199
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    • 2019
  • Objectives: KCHO-1(Mecasin), also called Gamijakyakgamchobuja-tang originally, is a combination of some traditional herbal medicines in East Asia. This medicine has been used mainly for alleviating neuropathic pains for centuries in Korean traditional medicine. KCHO-1 was developed to treat pain, joint contracture and muscular weakness in patients with amyotrophic lateral sclerosis. This study was carried out to investigate the chronic toxicity of KCHO-1 oral administration in rats for 26 weeks. Methods: Sprague-Dawely rats were divided into four groups and 10 rats were placed in the control group and the high-dose group, respectively. Group 1 was the control group and the remaining groups were the experimental groups. In the oral toxicity study, 500 mg/kg, 1,000 mg/kg, and 2,000 mg/kg of KCHO-1 were administered to the experimental group, and 10 ml/kg of sterile distilled water was administered to the control group. Survival rate, body weight, feed intake, clinical signs, and visual findings were examined. Urinalysis, ophthalmologic examination, necropsy, organ weight, hematologic examination, blood chemical examination and histopathologic examination were performed. Results: Mortality and toxicological lesions associated with the administration of test substance were not observed in all groups. Conclusion: NOAEL(No observed adverse effect level) of KCHO-1 is higher than 2000 mg/kg/day. And, the above findings suggest that treatment with KCHO-1 is relatively safe.

Probabilistic exposure assessment, a risk-based sampling plan and food safety performance evaluation of common vegetables (tomato and brinjal) in Bangladesh

  • Mazumder, Mohammad Nurun-Nabi;Bo, Aung Bo;Shin, Seung Chul;Jacxsens, Liesbeth;Akter, Tahmina;Bir, Md. Shahidul Haque;Aktar, Most Mohshina;Rahman, Md. Habibur;WeiQiang, Jia;Park, Kee Woong
    • Korean Journal of Agricultural Science
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    • v.48 no.1
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    • pp.33-43
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    • 2021
  • Along with the widespread use of pesticides in the world, concerns over human health impacts are rapidly growing. There is a large body of evidence on the relationship between the exposure to pesticides and the elevated rate of chronic diseases such as different types of cancers, diabetes, neurodegenerative disorders like Parkinson, Alzheimer, and amyotrophic lateral sclerosis (ALS), birth defects, and reproductive disorders. This research assessed the health risk of pesticide residues by the dietary intake of vegetables collected from the agro-based markets of Dhaka, Bangladesh. As some of the banned pesticides were also found in vegetable samples, they may pose a higher risk because of cheaper availability and hence the government of Bangladesh should take strong measures to control these banned pesticides. Five organo phosphorus (chlorpyrifos, parathion, ethion, acephate, fenthion) and two carbamate (carbaryl and carbofuran) pesticide residues were identified in twenty four samples of two common vegetables (tomato and brinjal). The pesticide residues ranged from below a detectable limit (< 0.01) to 0.36 mg·kg-1. Acephate, chlorpyrifos, ethion, and carbaryl were detected in only one sample, while co-occurrence occurred twice for parathion. Continuous monitoring and strict regulation should be enforced regarding the control of pesticide residues in fresh vegetables and other food commodities in Bangladesh.

NLRP3 Inflammasome in Neuroinflammatory Disorders (NLRP3 인플라마좀 작용 기전 및 신경 질환에서의 역할)

  • Kim, Ji-Hee;Kim, YoungHee
    • Journal of Life Science
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    • v.31 no.2
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    • pp.237-247
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    • 2021
  • Immune responses in the central nervous system (CNS) function as the host's defense system against pathogens and usually help with repair and regeneration. However, chronic and exaggerated neuroinflammation is detrimental and may create neuronal damage in many cases. The NOD-, LRR-, and pyrin domain―containing 3 (NLRP3) inflammasome, a kind of NOD-like receptor, is a cytosolic multiprotein complex that consists of sensors (NLRP3), adaptors (apoptosis-associated speck like protein containing a caspase recruitment domain, ASC) and effectors (caspase 1). It can detect a broad range of microbial pathogens along with foreign and host-derived danger signals, resulting in the assembly and activation of the NLRP3 inflammasome. Upon activation, NLRP3 inflammasome leads to caspase 1-dependent secretion of the pro-inflammatory cytokines IL-1β and IL-18, as well as to gasdermin D-mediated pyroptotic cell death. NLRP3 inflammasome is highly expressed in CNS-resident cell types, including microglia and astrocytes, and growing evidence suggests that NLRP3 inflammasome is a crucial player in the pathophysiology of several neuroinflammatory and psychiatric diseases, such as Alzheimer's disease, Parkinson's disease, multiple sclerosis, stroke, traumatic brain injury, amyotrophic lateral sclerosis, and major depressive disorder. Thus, this review describes the molecular mechanisms of NLRP3 inflammasome activation and its crucial roles in the pathogenesis of neurological disorders.

Mechanism of Human Endogenous Retrovirus (HERV) in Inflammatory Response (인간 내생 레트로바이러스(Human Endogenous Retrovirus, HERV)의 염증반응 조절 기작)

  • Ko, Eun-Ji;Cha, Hee-Jae
    • Journal of Life Science
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    • v.31 no.8
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    • pp.771-777
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    • 2021
  • Human endogenous retroviruses (HERVs) were inserted into the human genome millions of years ago but they are currently inactive and non-infectious due to recombinations, deletions, and mutations after insertion into the host genome. Nonetheless, recent studies have shown that HERV-derived elements are actually involved in physiological phenomena and certain diseases including cancers. Among the various physiological phenomena related to HERV-derived elements, it is necessary to focus on inflammatory response. HERV-derived elements have been reported to be directly involved in various inflammatory diseases, including autoimmune diseases such as rheumatoid arthritis, multiple sclerosis, amyotrophic lateral sclerosis, and Sjogren's syndrome. As a mechanism for regulating inflammation through HERV-derived elements, the possibility that HERV-derived elements may cause nonspecific innate immune processes and that HERV-derived RNA or proteins may cause selective signaling mechanisms through specific receptors can be considered. However, the mechanism through which HERV-derived elements regulate inflammatory response, such as how silent HERV elements are activated in inflammatory response and what factors and signaling mechanisms are involved in HERV-derived elements, have not been identified to date, making it difficult to study the onset of HERV-related inflammatory disease. In this review, we introduce HERV-related autoimmune diseases and propose the mechanisms of HERV-derived elements at the molecular level of HERV in inflammatory response.