• Journal of Acupuncture Research
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• v.20 no.3
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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.

• Annals of Clinical Neurophysiology
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• v.19 no.1
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• pp.68-70
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• 2017

Flail arm syndrome (FAS), known as one of the atypical amyotrophic lateral sclerosis (ALS) variants, has a similar clinical course and pathologic findings as ALS. Therefore it is difficult to differentiate between ALS and FAS at a glance. There are few reports involving individual analysis of FAS patients to date. The findings of polysomnography (PSG) in patient with FAS are not well known. We report a male FAS patient with review of literatures and several issues related to the diagnostic process.

• Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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• v.28 no.2
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• pp.79-83
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• 2017

Neurodegenerative diseases such as Parkinson's disease and amyotrophic lateral sclerosis may induce impairment of speech motor system. This review discusses the characteristics of dysarthria and symptom management for these conditions. Given the progressive nature of the neurodegenerative diseases, speech-language pathologists must be aware of appropriate augmentative and alternative communication equipment at the early stage of the disease course. Patients with neurodegenerative diseases can maintain functional communication with augmentative and alternative communication supports.

• Molecules and Cells
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• v.41 no.9
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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.

• Journal of Korean Medical Ki-Gong Academy
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• v.14 no.1
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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.

• Annals of Clinical Neurophysiology
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• v.6 no.2
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• pp.85-91
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• 2004

Background: Needle electromyography (EMG) and motor evoked potential (MEP) of the genioglossus (tongue) are difficult to perform in evaluations of the craniobulbar region in amyotrophic lateral sclerosis (ALS). Therefore, we investigated the yields of needle EMG and MEP recorded from the upper trapezius, since it receives innervation from the lower medulla and upper cervical cord. Methods: Needle EMG and MEP of the upper trapezius were obtained in 17 consecutive ALS patients. The needle EMG parameters recorded included abnormal spontaneous activity and motor unit action potential (MUAP) morphology. An upper motor neuron (UMN) lesion was presumed when either response to cortical stimulation was absent, or the central conduction time was delayed (>mean+2SD). Results: Of the five patients with bulbar-onset ALS, four had abnormalities in the upper trapezius and four in the tongue by needle EMG. In contrast, of the 12 patients with limb-onset ALS, 11 had abnormalities in the upper trapezius, and only five in the tongue. When MEP was performed, it was found that three of the five patients with bulbar symptoms and three of the six patients with isolated limb involvement had abnormal MEP findings. Conclusions: Electrophysiological studies of the upper trapezius are more sensitive those of the tongue in patients without bulbar symptoms. Thus, needle EMG and MEP of the upper trapezius are alternative tools for assessing bulbar and rostral neuraxial involvement in the diagnosis of ALS.

• Journal of Pharmacopuncture
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• v.13 no.1
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• pp.121-128
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• 2010

Objectives : This study was to investigate the effects of Oriental medical treatment on ALS. Methods : We investigated 12 ALS patients which were admitted to Gwang-Ju O.M.hospital for 3 months and follow-up at 8 months after discharge. All patients were treated by SAAM-acupuncture, herb medication, Bee venom Pharmacopuncture therapy, Needle-embedding therapy, etc and after discharge self-therapy at home. We evaluated patients using the Amyotrophic lateral sclerosis Functional Rating Scale-Revised(ALSFRS-R), Medical Research Council (MRC) Scale. Results : After 1 month, mean ALSFRS-R score of patients was $29.08{\pm}7.99$, 2 months $28.70{\pm}7.17$, 3 months $28.16{\pm}8.23$, 1 year $21.33{\pm}9.93$ and mean MRC Scale of patients was $25.34{\pm}8.45$, 2 months $25.34{\pm}8.45$, 3months $21.56{\pm}9.20$. But in both cases, the variation was not statistically significant. Conclusions : We think that the results of this case be a pilot study that proves the effect of Oriental Medical treatment on ALS.

• 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 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.