태양인 해역증으로 진단한 근위축성 측삭경화증 환자 치험 1례

김소형,이미숙,박유경,배나영,Kim, So-Hyoung,Lee, Mi-Suk,Park, Yu-Gyeong,Bae, Na-Young 사상체질의학회 2016 사상체질의학회지 Vol.28 No.3

Objectives This case study was about a Taeyangin patient with Amyotrophic Lateral Sclerosis identified as Hae-Yeok pattern. In this study, we report the significant improvement of extremity weakness of this patient after Sasang Constitutional medicine treatment.Methods The patient was identified as Taeyangin Hae-Yeok pattern and treated with Ogapijangchuk-tang and acupuncture. Korean-Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (K-ALSFRS-R) was used to assess the overall function of the patient. And the Global Assessment Scale (GAS) was used to assess the change of upper and lower extremities weakness after the treatment.Result and Conclusion The K-ALSFRS-R total score decreased from 30 points to 26 points and extremity weakness showed significant improvement in GAS after 12 days treatment. Furthermore, walking distance was much longer after treatment. In conclusion, this study shows that Sasang constitutional medicine can be effective treatment for Taeyangin patient with Amyotrophic Lateral Sclerosis.

한방 치료로 호전된 근위축성측색경화증에 대한 증례 1례 : 증례보고

허기윤,강희경,김민화,남이랑,김마리아,김소연,박소정,윤영주,이인,최준용,한창우,홍진우,권정남,Heo, Gi-yoon,Kang, Hee-kyung,Kim, Min-hwa,Nam, Irang,Kim, Mariah,Kim, So-yeon,Park, So-jung,Yun, Young-ju,Lee, In,Choi, Jun-yong,Han, Chang-woo,Hong 대한한방내과학회 2022 大韓韓方內科學會誌 Vol.43 No.5

Purpose: The purpose of this paper is to report the improvement of a patient with amyotrophic lateral sclerosis after long-term combined Korean medical treatment. Methods: A patient diagnosed with amyotrophic lateral sclerosis was treated with herbal medicine, acupuncture, pharmacopuncture, moxibustion, and rehabilitation for four separate hospital stays. To evaluate their respiratory discomfort and limb weakness, we used Manual Muscle Testing, the Pulmonary Function Test, and the Korean Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised. Results: The weakness of the muscles of the lower extremities and respiratory function was improved. Conclusion: We consider that combined Korean medicine treatments might be an effective treatment for muscle weakness and respiratory discomfort of amyotrophic lateral sclerosis. To verify the effectiveness of these treatments, further research is required.

근위축성 측상경화증 환자의 전신 마취 경험

최용헌 ( Yong-heon Choi ),정준영 ( Jun-young Chung ) 경희대학교 경희의료원 2016 慶熙醫學 Vol.31 No.1

Amyotrophic lateral sclerosis is a neurodegenerative disease caused by the degeneration of upper and lower motor neurons. The disorder causes muscle weakness and atrophy throughout the body. Muscle relaxants, either depolarizing or non-depolarizing, are known to cause unexpected responses in ALS patients. The perioperative risk of respiratory failure is high. Thus, careful monitoring and adequate doses of anesthetics are required. We report a case of a patient with amyotrophic lateral sclerosis who underwent general anesthesia without using muscle relaxants. The patient recovered from anesthesia without respiratory complications.

운동치료가 근위축성측삭경화증 환자 삶의 질에 미치는 영향: 예비연구

천윤목,이강노,김대열 대한근전도전기진단의학회 2018 대한근전도 전기진단의학회지 Vol.20 No.1

Objective: Amyotrophic lateral sclerosis (ALS) is a fatal progressive neurodegenerative disorder. The objectives of this study are to examine the effect of exercise program on the health-related quality-of-life (HRQOL) in patients with ALS Method: We reviewed ALS patients who came to the hospital’s outpatient clinic from May 2014 to June 2015. The ALS patient received the hospital based exercise program, exercising 1 or 2 days/week or education of self-trained exercise program. We assessed the patients with Korean version of Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (K-ALSFRS-R) for functional capacity and SF-36 for QOL. Results: Ten patients (6 males and 4 females) were evaluated for functional capacity and QOL, and four of them were reassessed. In the 2nd evaluation, Re-evaluated patients’ K-ALSFRS-R score were decreased but three of them were rated as Physical Component Score of SF-36 improved. Conclusion: The results of our study suggest that an exercise program or education may improve HRQOL of patients with ALS.

Ocular Involvement Occurs Frequently at All Stages of Amyotrophic Lateral Sclerosis: Preliminary Experience in a Large Italian Cohort

Federica Cozza,Andrea Lizio,Lucia Catherine Greco,Stefania Bona,Giordana Donvito,Elena Carraro,Silvia Tavazzi,Nicola Ticozzi,Barbara Poletti,Valeria Ada Sansone,Christian Lunetta 대한신경과학회 2021 Journal of Clinical Neurology Vol.17 No.1

Background and Purpose The study aimed to obtain optometric findings of amyotrophic lateral sclerosis (ALS) patients in different stages of the disease, and to determine the relation between ocular data and ALS-related features; that is, functional and cognitive impairment and staging. Methods The optometric protocol included tests of the ocular motility [broad-H test and Northeastern State University College of Optometry (NSUCO) test], near point of convergence (NPC), error refraction, best-corrected visual acuity, and binocular visual alignment, and an ocular symptoms questionnaire. The functional measures included the Amyotrophic Lateral Sclerosis Functional Rating Scale–revised (ALSFRS-r) and Milano-Torino staging (Mi- ToS), and cognitive impairment was assessed using the Edinburgh Cognitive and Behavioural ALS Screen (ECAS). Demographic and clinical features were also collected, including whether the patients used an eye-tracking communication device (ETCD). Results Two-hundred consecutive ALS patients (median age of 64 years, 118 males and 82 females) in different stages of disease were recruited. Nearly 70% of patients reported at least one ocular symptom, and the use of an ETCD was found to be significantly related to the presence of most symptoms. Moreover, the severely symptomatic group was characterized by significantly lower ALSFRS-r total and subscale scores, and higher MiToS. Abnormal NPC values were significantly related to lower ALSFRS-r total and bulbar-subscale scores. Patients with acceptable NSUCO test values exhibited significantly higher ECAS scores. Conclusions The presence of ocular alteration in patients in different stages of ALS supports the idea that this is a multisystem disorder and emphasizes the importance of optometric evaluations in multidisciplinary assessments to address ocular impairment early in the disease process

Complementary and alternative medicine for treating amyotrophic lateral sclerosis: a narrative review

Mudan Cai,Eun Jin Yang 한국한의학연구원 2019 Integrative Medicine Research Vol.8 No.4

Background: Amyotrophic lateral sclerosis (ALS) is a rare neurodegenerative disease that is characterized by selective motor neuron cell death in the motor cortex, brainstem, and spinal cord. Two drugs for ALS, riluzole and edaravone, have been approved by FDA for the treatment of ALS patients. However, they have many side effects, and riluzole extends the patient’s life by only 2–3 months. Therefore, ALS patients seek an effective therapy for treating the symptoms or delaying the progression of ALS. Based on this, we review the effects of complementary and alternative medicine (CAM) in ALS animals or patients to verify the efficacy of CAM in incurable diseases. Methods: For this review, we searched published papers focusing on the effect of CAM in pre-clinical and clinical study in ALS. The search keywords included amyotrophic lateral sclerosis, acupuncture, herbal medicine, Traditional Chinese medicine, CAM, animals, and clinical study through electronic databases PubMed and Google Scholar from their inception until March 2019. Results: In the ALS animal model, CAM modulated the immune system to increase motor function by reducing the expression levels of neuroinflammatory proteins in the spinal cord. Besides this, ALS patients treated with herbal medicine showed improved disease symptoms, but clinical trials with larger sample sizes are needed to develop a treatment with this herbal medicine. Conclusion: This review shows that CAM may be useful for ALS treatment, but more evidence regarding the efficacy and molecular mechanisms is required to establish CAM as a good therapy for the treatment of ALS patients.

Tissue inhibitor of metalloproteinases-3 (TIMP-3) expression is increased during serum deprivation-induced neuronal apoptosis in vitro and in the G93A mouse model of amyotrophic lateral sclerosis: A potential modulator of Fas-mediated apoptosis

Lee, J.K.,Shin, J.H.,Suh, J.,Choi, I.S.,Ryu, K.S.,Gwag, B.J. Blackwell Science ; Academic Press 2008 Neurobiology of disease Vol.30 No.2

Cortical neurons deprived of serum undergo apoptosis that is sensitive to inhibitors of macromolecule synthesis. Proteomic analysis revealed differential expression of 49 proteins in cortical neurons 8 h after serum deprivation. Tissue inhibitor of metalloproteinases-3 (TIMP-3), a pro-apoptotic protein in various cancer cells, was increased during serum deprivation-induced apoptosis (SDIA), but not during necrosis induced by excitotoxicity or oxidative stress. Levels of TIMP-3 were markedly increased in degenerating motor neurons in a transgenic model of familial amyotrophic lateral sclerosis. The TIMP-3 expression was accompanied by increase in Fas-FADD interaction, activated caspase-8, and caspase-3 during SDIA and in vulnerable spinal cord of the ALS mouse. SDIA and activation of the Fas pathway were prevented by addition of an active MMP-3. Timp-3 deletion by RNA interference attenuated SDIA in N2a cells. These findings provide evidence that TIMP-3 is an upstream mediator of neuronal apoptosis and likely contributes to neuronal loss in neurodegenerative diseases such as amyotrophic lateral sclerosis.

A Case of Frontotemporal Dementia with Amyotrophic Lateral Sclerosis Presenting with Pathological Gambling

Ozel-Kizil ET,Sakarya AL,Arica B,Haran S 대한신경과학회 2013 Journal of Clinical Neurology Vol.9 No.2

Background Pathological gambling (PG), which is characterized by consistent, repetitive gambling and unsuccessful quitting attempts, is classified as an impulse control disorder. PG has also been reported in patients with Parkinson’s disease, frontotemporal dementia, and amyotrophic lateral sclerosis. Case Report A 53-year-old male visited the outpatient clinic due to excessive gambling and personality changes. Based on electrophysiological findings and neuropsychiatric assessment,he was diagnosed as frontotemporal dementia-amyotrophic lateral sclerosis. Conclusions This case report underlines that PG can also be seen in patients with neurological disorders involving the orbitofrontal cortex.

Oral Solubilized Ursodeoxycholic Acid Therapy in Amyotrophic Lateral Sclerosis: A Randomized Cross-Over Trial

민주홍,홍윤호,성정준,김성민,이정복,이광우 대한의학회 2012 Journal of Korean medical science Vol.27 No.2

To evaluate the efficacy and safety of ursodeoxycholic acid (UDCA) with oral solubilized formula in amyotrophic lateral sclerosis (ALS) patients, patients with probable or definite ALS were randomized to receive oral solubilized UDCA (3.5 g/140 mL/day) or placebo for 3 months after a run-in period of 1 month and switched to receive the other treatment for 3 months after a wash-out period of 1 month. The primary outcome was the rate of progression, assessed by the Appel ALS rating scale (AALSRS), and the secondary outcomes were the revised ALS functional rating scale (ALSFRS-R) and forced vital capacity (FVC). Fifty-three patients completed either the first or second period of study with only 16 of 63 enrolled patients given both treatments sequentially. The slope of AALSRS was 1.17 points/month lower while the patients were treated with UDCA than with placebo (95% CI for difference 0.08-2.26, P = 0.037), whereas the slopes of ALSFRS-R and FVC did not show significant differences between treatments. Gastrointestinal adverse events were more common with UDCA (P < 0.05). Oral solubilized UDCA seems to be tolerable in ALS patients, but we could not make firm conclusion regarding its efficacy, particularly due to the high attrition rate in this cross-over trial.

Transduction of Familial Amyotrophic Lateral Sclerosis-related Mutant PEP-1-SOD Proteins into Neuronal Cells

Jae Jin An,Yeom Pyo Lee,So Young Kim,Sun Hwa Lee,김대원,Min Jung Lee,Min Seop Jeong,장상호,Jung Hoon Kang,권혁일,강태천,원무호,조성우,권오신,이길수,박진서,음원식,최수영 한국분자세포생물학회 2008 Molecules and cells Vol.25 No.1

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by the selective death of motor neurons. Mutations in the SOD1 gene are responsible for a familial form of ALS (FALS). Although many studies suggest that mutant SOD1 proteins are cytotoxic, the mechanism is not fully understood. To investigate the role of mutant SOD1 in FALS, human SOD1 genes were fused with a PEP-1 peptide in a bacterial expression vector to produce in-frame PEP-1-SOD fusion proteins (wild type and mutants). The expressed and purified PEP-1-SOD fusion proteins were efficiently transduced into neuronal cells. Neurones harboring the A4V, G93A, G85R, and D90A mutants of PEP-1-SOD were more vulnerable to oxidative stress induced by paraquat than those harboring wild-type proteins. Moreover, neurones harboring the mutant SOD proteins had lower heat shock protein (Hsp) expression levels than those harboring wild-type SOD. The effects of the transduced SOD1 fusion proteins may provide an explanation for the association of SOD1 with FALS, and Hsps could be candidate agents for the treatment of ALS.