• 제목/요약/키워드: Amyotrophic Lateral Sclerosis (ALS)

검색결과 90건 처리시간 0.023초

근위축성측삭경화증 환자에서의 myelin water fraction MRI 1예 (Myelin Water Fraction MRI in a Case of Clinically Probable Amyotrophic Lateral Sclerosis)

  • 양지원;이종호;김응엽;신동훈
    • Annals of Clinical Neurophysiology
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    • 제18권1호
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    • pp.18-20
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    • 2016
  • Amyotrophic lateral sclerosis (ALS) is a progressive motor neuron degenerative disease that clinically manifests both upper and lower motor neuron signs. However, it is unknown where and how the motor neuron degeneration begins, and conflicting hypotheses have been suggested. Recent advanced radiological techniques enable us to look into ALS neuropathology in vivo. Herein, we report a case with upper motor neuron-predominant ALS in whom the results of brain magnetic resonance imaging (MRI) and myelin water fraction MRI suggest axonal degeneration.

A case report on the use of processed Glycyrrhiza uralensis extract and Korean medical treatment for a patient with Amyotrophic Lateral Sclerosis(ALS)

  • Cha, Eun Hye;Lee, Seong Jin;Lee, Ji In;Song, In Ja;Kim, Sung Chul
    • Journal of Acupuncture Research
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    • 제33권2호
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    • pp.201-210
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    • 2016
  • Objectives : The serum creatine kinase(CK) level of Amyotrophic Lateral Sclerosis(ALS) patients suggests that it may be an independent prognostic factor for the survival of ALS. We report the changes of serum CK level of ALS patients treated by administering processed Glycyrrhiza uralensis extracts and Korean medical treatments. Methods : We provided an ALS patient with processed Glycyrrhiza uralensis extracts and Korean medical treatments including acupuncture, pharmacopuncture and herbal medicine. The serum CK level was checked every month. The changes of ALSFRS-R and MRC grade were checked every month for additional result. Results : The serum CK level as the prognostic factor for the survival of ALS gradually decreased for three months. However, ALSFRS-R decreased by two points at the second trial. MRC rate had no change for three months. Conclusion : Combined administration of processed Glycyrrhiza uralensis extracts and Korean medical treatment could be meaningful possibilities in the treatment of ALS. We should conduct further studies to solve the limitations of this case study.

근위축성 측삭 경화증 1예 (A Case of Amyotrophic Lateral Sclerosis)

  • 이범준;전진희;류재환;김태연
    • 대한한방내과학회지
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    • 제21권4호
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    • pp.661-665
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    • 2000
  • Amyotrophic Lateral Sclerosis(ALS) is a fatal neuromuscular disease characterized by progressive muscle weakness resulting in paralysis. ALS is characterized by both upper and lower motor neuron damage. Diagnostic tests include magnetic resonance imaging(MRI) electromyogram(EMG), muscle biopsy, and blood tests. In order for a definitive diagnosis of ALS to be made, damage must be evident in both upper and lower motor neurons. When three limbs are sufficiently affected, the diagnosis is ALS. There is no cure for ALS. We recently experienced one case of ALS, The patients was diagnosed as ALS by EMG and Symptoms. We diagnosed her as Wea jeung and treated by Herbal-medication based on the differentiation of symtoms. we report change of his symptoms through both western medical treatment and oriental medical treatment.

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가족성 근위축성측삭경화증을 유발시키는 두 번째 유전자 위치 (Second locus for late-onset familial Amyotrophic Lateral Sclerosis)

  • 홍성출
    • 생명과학회지
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    • 제11권3호
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    • pp.279-283
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    • 2001
  • Amyotrophic lateral sclerosis(ALS) is a progressive neurologic disorder resulting from the degeneration of upper and lower motor neurons, and is inherited in 10% of cases. About 20% of familial ALS, clinically indistinguishable from sporadic ALS, is caused by mutations of Cu/Zn superoxide dismutase on chromosome 21q22.21 inherited as an autosomal dominant trait. We now report a new locus in the non-SOD1 dominantly inherited ALS. We screened a large ALS family with 11 affected individuals and one obligate gene carrier with genome-wide ABI polymorphic markers using the ABI 377 automated system. No evidence of linkage was obtained with the autosomal markers. We next screened this family with X chromosome markers as there was no evidence of male-to-male tran-smission of the disease. Linkage was established with several X chromosome markers with a lod score up to 3.8; almost the maximum possible score in this family. Our finding imply that a gene for the dominant expression of a neuronal degeneration is coded on X chromosome and raise the question of the role of X-linked genes that escape inactivation in this pathogenesis. More importantly, our finding that a gene causing ALS is localized on X-chromosome has direct investigational relevance to sporadic ALS, where epidemiological studies show male gender predominance(1.3:1) and earlier onset in men by 5-10 years.

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근 위축성 측삭 경화증 환자 1례 보고 (A Case of Amyotrophic Lateral Sclerosis)

  • 김태정;문아지;이성근;이기상
    • 대한중풍순환신경학회지
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    • 제13권1호
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    • pp.77-85
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    • 2012
  • Amyotrophic lateral sclerosis(ALS) is a progressive disorder that causes degeneration of motor neurons of the brain and spinal cord. It is characterized by loss of motor neurons leading to muscle weakness, and sensation and mental function stay intact during the course of the disease. Diagnostic tests include magnetic response imaging(MRI), electromyogram(EMG), muscle biopsy, and blood tests. In order to a definitive diagnosis of ALS, damage must be evident in both upper and lower motor neurons. When three limbs are sufficiently affected, the diagnosis is ALS. There is no cure for ALS. We experienced one case of ALS, the patients was diagnosed as ALS by EMG and symptoms. We diagnosed her as ShinEumHur(SEH) and treated by Choakwiyeum(左歸飮). We report the change of her symptoms through oriental medical treatment.

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화병과 주요우울장애가 병발한 근위축성측삭경화증 환자 1례(例) 증례보고 (One case Report with a Amyotrophic lateral sclerosis(ALS) patient who has Hwabyung and Major Depressive Disorder)

  • 김상호;박종훈;박소정;변순임;황의완;김종우
    • 동의신경정신과학회지
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    • 제16권2호
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    • pp.159-169
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    • 2005
  • Amyotrophic lateral sclerosis (ALS) is characterised by progressive impairment of motor functions up to a complete loss of autonomy, and most of ALS patients are associated with the total preservation of mental state like depression. A patient in this case report had ALS and during progress of ALS had undergone depression and Hwabyung's Symptoms. This patient was treated with herb medication, acupuncture, psychotherapy(relaxation & councelling) primarily. During the 18 days of treatment from admission, the patient experienced much improvement of depression and Hwabyung's Symptoms. and ALS's symptom like weakness & numbness of lower limb had improved considerably, too.

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근위축성 측삭경화증 환자의 삶의 질 (Quality of Life in Patients with Amyotrophic Lateral Sclerosis)

  • 허현숙;최스미
    • 재활간호학회지
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    • 제16권2호
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    • pp.122-132
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    • 2013
  • Purpose: This study measured the quality of life in patients with amyotrophic lateral sclerosis (ALS). Methods: The participants consisted of consecutive patients with ALS who visited the neurology outpatient department from January to July, 2008. To collect the data, face-to-face interviews were applied at S national university hospital in Seoul. Using multivariate analysis, factors to predict QOL in ALS patients including demographic factors, functional independence, depression, anxiety, social support and quality of life were analyzed. Results: The final analysis included 69 patients. The mean score for the physical health and mental health components was $34.4{\pm}21.3$ and $44.7{\pm}20.6$, respectively. The mean score for the ALS functional rating scale was $24.3{\pm}10.8$ out of 40. Anxious and depressed patients accounted for 44.9% and 71%, respectively. Quality of life in ALS patients was significantly affected by functional independence, depression and anxiety level. Conclusion: To improve the quality of life in ALS patients with declining functional independence, comprehensive interventions are necessary to manage depression and anxiety.

Pain in amyotrophic lateral sclerosis: a narrative review

  • Kwak, Soyoung
    • Journal of Yeungnam Medical Science
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    • 제39권3호
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    • pp.181-189
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    • 2022
  • Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative condition characterized by loss of motor neurons, resulting in motor weakness of the limbs and/or bulbar muscles. Pain is a prevalent but neglected symptom of ALS, and it has a significant negative impact on the quality of life of patients and their caregivers. This review outlines the epidemiology, clinical characteristics, underlying mechanisms, and management strategies of pain in ALS to improve clinical practice and patient outcomes related to pain. Pain is a prevalent symptom among patients with ALS, with a variable reported prevalence. It may occur at any stage of the disease and can involve any part of the body without a specific pattern. Primary pain includes neuropathic pain and pain from spasticity or cramps, while secondary pain is mainly nociceptive, occurring with the progression of muscle weakness and atrophy, prolonged immobility causing degenerative changes in joints and connective tissue, and long-term home mechanical ventilation. Prior to treatment, the exact patterns and causes of pain must first be identified, and the treatment should be tailored to each patient. Treatment options can be classified into pharmacological treatments, including nonsteroidal anti-inflammatory drugs, antiepileptic drugs, drugs for cramps or spasticity, and opioid; and nonpharmacological treatments, including positioning, splints, joint injections, and physical therapy. The development of standardized and specific assessment tools for pain-specific to ALS is required, as are further studies on treatments to reduce pain, diminish suffering, and improve the quality of life of patients with ALS.

하시모토 갑상선염을 동반한 근위축성 측삭경화증 1예 (Amyotrophic lateral sclerosis combined with Hashimoto's thyroiditis : A case report)

  • 고영채;강성수;이영배;박현미;신동진
    • Annals of Clinical Neurophysiology
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    • 제3권1호
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    • pp.37-39
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    • 2001
  • Although the etiology and pathogenesis of amyotrophic lateral sclerosis(ALS) is unknown, increasing evidence supports a role for autoimmune mechanisms in motor neuron degeneration. The coexistence of immune disease in ALS supports that an altered immune system may contribute to disease pathogenesis. A 55-year-old woman was admitted to our department due to dysarthria and gait disturbance. On physical and neurologic examination, she showed thyroid enlargement, tongue atrophy, muscle weakness, fasciculation, and increased deep tendon reflex. The electrophysiological studies are compatible with motor neuron disease. Cytological findings of thyroid were compatible with Hashimoto's thyroiditis. Thus, we report a case of ALS combined with Hashimoto's thyroiditis. And the simultaneous presentation with ALS and Hashimoto's thyroiditis led us to consider whether this was simply a chance association or not.

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감각신경전도 이상을 동반한 근위축성측색경화증 (Amyotrophic Lateral Sclerosis Combined with Abnormal Sensory Nerve Conduction)

  • 서상혁;안재영;이택준;서연림;신경진;김병준
    • Annals of Clinical Neurophysiology
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    • 제7권2호
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    • pp.133-137
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    • 2005
  • Underlying neuropathies combined with amyotrophic lateral sclerosis (ALS) cast doubt on the diagnosis of ALS when present. Abnormal sural nerve conductions were found in 3 patients with clinically definite ALS. Pathologically demyelinating, axonal, or vasculitic neuropathy was suggested respectively. High dose oral corticosteroid had no effect and clinical courses were deteriorating in all the patients. The causes of combined neuropathies were unclear. Possibility of direct consequence of ALS, concomitant neuropathies, or rare variants of ALS should be considered in these cases.

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