• 대한치과마취과학회지
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• 제13권4호
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• pp.195-201
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• 2013

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease caused by the degeneration of upper and lower motor neurons. The disorder causes muscle weakness and atrophy in airway muscles including pharyngeal, laryngeal and other respiratory muscles. The response to muscle realxant is also altered in patients with ALS. Because of the inherent muscle weakness and associated respiratory insufficiency, particular attentions are needed in anesthetic management of ALS patients. We used proper doses of inhalation anesthetics and opioids under EEG-entropy (electroencephalography-entropy)-monitoring without the use of muscle realxants in the anesthetic management of a patient with ALS. The patient early recovered and was discharged on the same day without any respiratory complications.

• BMB Reports
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• 제55권12호
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• pp.621-626
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• 2022

Amyotrophic lateral sclerosis (ALS) is an incurable neurodegenerative disease characterized by the degeneration of motor neurons in the spinal cord. Main symptoms are manifested as weakness, muscle loss, and muscle atrophy. Some studies have reported that alterations in sphingolipid metabolism may be intimately related to neurodegenerative diseases, including ALS. Acid sphingomyelinase (ASM), a sphingolipid-metabolizing enzyme, is considered an important mediator of neurodegenerative diseases. Herein, we show that ASM activity increases in samples from patients with ALS and in a mouse model. Moreover, genetic inhibition of ASM improves motor function impairment and spinal neuronal loss in an ALS mouse model. Therefore, these results suggest the role of ASM as a potentially effective target and ASM inhibition may be a possible therapeutic approach for ALS.

• Annals of Clinical Neurophysiology
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• 제6권1호
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• pp.1-13
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• 2004

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that primarily affects motor neurons. The characteristic features of this devastating disorder are the simultaneous presence of upper and lower motor neuron (LMN) signs with progression from one region of the neuraxis to the next and eventual death, typically from respiratory compromise. Electrophysiological studies are an indispensible part of the ALS evaluation, especially serving as an extension of the clinical examination, and most useful in identifying LMN dysfunction. Not only may electrodiagnostic studies reveal characteristic changes in those regions clinically manifesting signs, but it also serves to disclose asymptomatic areas of involvement.

• Annals of Clinical Neurophysiology
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• 제5권1호
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• pp.27-33
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• 2003

Background: Motor unit number estimation (MUNE) can directly assess motor neuron populations in muscle and quantify the degree of physiologic and/or pathologic motor neuron degeneration. A high degree of reproducibility and reliability is required from a good quantitative tool. MUNE, in various ways, is being increasingly applied clinically and statistical MUNE has several advantages over alternative techniques. Nevertheless, the optimal method of applying statistical MUNE to improve reproducibility has not been established. Methods: We performed statistical MUNE by selecting the most compensated compound muscle action potential (CMAP) area as a test area and modified the results obtained by weighted mean surface-recorded motor unit potential (SMUP). Results: MUNE measures in amyotrophic lateral sclerosis (ALS) patients showed better reproducibility with sizeweighted modification. Conclusions: We suggest size-weighted MUNE testing of "neurogenically compensated"CMAP areas present an optimal method for statistical MUNE in ALS patients.

• 대한간호학회지
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• 제38권6호
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• pp.802-812
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• 2008

Purpose: The purpose of this study was to identify and describe phenomenological structures of the lived experience of struggling against an illness for patients with Amyotrophic Lateral Sclerosis (ALS). Methods: The participants were 7 patients with ALS recruited by snowball sampling who agreed to participate in this research and could verbally communicated with the researcher. Data were collected by long term-repeated interviews with participants in their own homes. Data were analyzed using Colaizzi's method of phenomenology. Results: Four categories were extracted as follows: 'Being seized with fear of death', 'Living a marginal life', 'Accepting hard fate', and 'Clinging to faint life'. Seven theme clusters were identified as: 'Wandering to find a healing method with ominous signs in the body', 'Having a diagnosis of ALS is like a bolt from the blue and struggling against illness with faint hope', 'Being forced out to the edge of life with anguish', 'Filling one's heart with hatred and longing toward becoming estranged from the world', 'Living with stigma as a stumbling block with bitter grief in one's heart', 'Accepting every things as one's fate with self controlled fear of death', and 'Attaching to desire to live'. Conclusion: The results of this study can be used to develop the programs to support patients with ALS and their family.

• Molecules and Cells
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• 제41권9호
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• pp.818-829
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• 2018

Significant research efforts are ongoing to elucidate the complex molecular mechanisms underlying amyotrophic lateral sclerosis (ALS), which may in turn pinpoint potential therapeutic targets for treatment. The ALS research field has evolved with recent discoveries of numerous genetic mutations in ALS patients, many of which are in genes encoding RNA binding proteins (RBPs), including TDP-43, FUS, ATXN2, TAF15, EWSR1, hnRNPA1, hnRNPA2/B1, MATR3 and TIA1. Accumulating evidence from studies on these ALS-linked RBPs suggests that dysregulation of RNA metabolism, cytoplasmic mislocalization of RBPs, dysfunction in stress granule dynamics of RBPs and increased propensity of mutant RBPs to aggregate may lead to ALS pathogenesis. Here, we review current knowledge of the biological function of these RBPs and the contributions of ALS-linked mutations to disease pathogenesis.

• 대한의료기공학회지
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• 제14권1호
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• pp.39-51
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• 2014

Objects : This study is a report on treatment effect of the case that treated by using of oriental medical treatment to Amyotrophic Lateral Sclerosis(ALS) patient. Methods : The patient diagnosed with ALS was treated by using of acupuncture, physical therapy, Su-gi therapy, thermotherapy and herbal medicine during 180 days. K-ALSFRS-R score was used as the tool of treatment effect. Results : Despite the K-ALSFRS-R score reduction, the oriental medical treatment maintain the respiratory function and a speech. Conclusions : The oriental medical treatment is effective on local symptoms of ALS, but there were no functional improvement of ALS in this case study. It is necessary to have more examination about ALS.

• 한국융합학회논문지
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• 제10권9호
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• pp.193-198
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• 2019

본 연구는 근위축성측삭경화증(amyotrophic lateral sclerosis, ALS)으로 진단된 여성을 대상으로 음향음성학적 스펙트로그램 분석을 이용하여 11개월 동안 모음과 이중모음의 포먼트 변화(vowel formant variation)를 분석하였다. 검사어는 단모음 /a, i, u/와 이중모음 /h + ja + da/, /h + wi + da/, /h +ɰi+ da/를 이용하였다. 발화자료는 'Alvin' 프로그램을 이용하여 모니터에 제시된 단어읽기과제를 통해 수집되었고, 녹음환경은 nyquist frequency는 5,500Hz, sampling rate는 11,000Hz으로 설정하였다. 녹음자료는 스펙트로그램을 이용하여 강도, 음도와 이중모음의 포먼트를 분석하였다. 분석결과, ALS의 진행과정에서 기본주파수와 강도가 저하되었고, 단모음에서의 포먼트 변화보다는 이중모음의 포먼트 기울기의 감소가 특징으로 확인되었다. 이 결과는 병의 진행에 따른 ALS의 모음왜곡이 혀와 턱의 협응력 감소에 기인함을 시사한다.

• 대한중풍순환신경학회지
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• 제16권1호
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• pp.89-102
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• 2015

■ Objectives This study aimed to investigate the domestic research trend about Amyotrophic Lateral Sclerosis(ALS) treated with Traditional Korean Medicine(TKM). ■ Methods Searches of two major TKM databases were conducted for articles published up to July 2015. Searching keyword was "Lou Gehrig's disease" OR "amyotrophic lateral sclerosis". Studies dealt TKM for ALS were included. ■ Results 18 studies were met our inclusion criteria(two prospective studies, one retrospective study, one follow-up study, 14 case reports). Complex TKM intervention of herbal medication, acupuncture and pharmacopuncture was the most frequently used treatment. Individually, herbal medication was the most frequent followed by acupuncture. Short-term assessment showed improvement in ALS symptoms but long-term assessment showed progression of disease. Accessory symptoms including depression, anxiety and insomnia were improved in four studies. ■ Conclusion For more definite evidence, larger studies with long-term follow-up period are needed. Moreover, benefits that TKM can provide for ALS patients as a part of multi-disciplinary approach should be studied in depth.

• Journal of Acupuncture Research
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• 제20권3호
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• pp.209-216
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• 2003

목적 : 대표적인 motor neuron disease(MND)이면서 가장 치명적인 신경퇴행성 질환인 ALS의 발생 양상과 한방치료효과에 대하여 연구하였다. 방법: 상지대학교 부속 한방병원에 래원한 ALS 환자들의 성별, 연령, 발병일, 발병양상, 래원 당시의 병태양상 등을 분석하였고, 이 중 3개월 이상 입원치료를 통하여 치료과정 평가가 가능하였던 18명의 환자들의 치료 전과 치료 후의 변화양상을 평가하였다. 결과 : 외국의 보고에 비하여 여성 환자가 많았고 발병 연령도 유의하게 낮았으며 상지에서 최초의 증상이 발현되는 비율이 상대적으로 높았다. Bulbar From의 비율도 높았고, 대부분의 환자가 여성인 것도 특이하였다. 환자들이 생각하는 질병의 발생원인 중 약 80%가 stress나 정신적 충격 등을 지적하여 정신적 불안상태가 유관함을 추정할 수 있었다. 한방치료가 ALS를 호전시키지는 못하였으나 진행을 억제하였고, 국소적인 주소증을 완화시키며 심리적 안정 상태를 유지하는데 도움이 된다고 평가되었다.