• 근관절건강학회지
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• 제13권2호
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• pp.130-139
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• 2006

Purpose: This study was designed to present preliminary data for the development of appropriate nursing care system for the patients with ALS by analyzing their physical function. Method: The clinical data of 36 ALS patients, who visited ALS Clinic of H University Hospital in Seoul, were collected from January, 2006 to August, 2006. To determine the physical function, Norris ALS scale and Appel ALS Rating Scale were used. The data were analyzed by frequency, percentage, mean, standard deviation, range, t-test, ANOVA, using SPSS PC program. Results: The mean score of physical activity, muscle strength, upper extremity function, lower extremity function was 18.08, 27.72, 25.94, 25.19 respectively. There were significant differences in physical activity, muscle strength, and upper extremity function according to sender and comorbid disease(diabetes). Although sites of symptom onset were not statistically significant with all physical function, patients with bulbar onset showed relatively severe physical disabilities. Conclusion: The preliminary data on physical function of patients with ALS would be helpful for the development of ALS nursing guideline system.

• 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.

• The Journal of Korean Physical Therapy
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• 제19권5호
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• pp.77-85
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• 2007

Purpose: This study was to investigated the effect of physical function and quality of life through physical therapy and occupational therapy by Bobath approach to patient with ALS. Methods: The data of 1 patient with ALS, who visited D hospital in Busan, was collected from June 5, 2006 to August 19, 2007. To determine the physical function and quality of life, Berg Balance Scale (BBS), Gait, Skate. Purdue Pegboard, WHOQOL-BREF, COPM were used. Results: BBS score was increased 0 to 33 in the pre and post test. Gait distance was increased 0m to 500m in pre and post test. Skate score was increased 13 to 22 in the pre and post test, Purdue Pegboard score was increased 0 to 3 in the pre and post test, WHOQOL-BREF was increased 1.92 to 2.46 in the pre and post test. Average COPM score of performance and satisfaction grade of pre test score was 1 then Post test score of performance score increased to 5.7 and satisfaction increased to 4. Conclusion: Physical therapy and Occupational therapy increase physical function and quality of life in ALS patients. Physical therapy and occupational therapy should study more about patients with ALS.

• 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.

• Annals of Clinical Neurophysiology
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• 제15권2호
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• pp.53-58
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• 2013

Background: The autophagy is the major route for lysosomal degradation of misfolded protein aggregates and oxidative cell components. We hypothesized that rapamycin (autophagy enhancer) would prolong the survival of motor neuron and suppress the disease progression in amyotrophic lateral sclerosis (ALS). Methods: A total of 24 transgenic mice harboring the human G93A mutated SOD1 gene were used. The clinical status involving rotarod test and survival, and biochemical study of ALS mice model were evaluated. Results: The onset of symptoms was significantly delayed in the rapamycin administration group compared with the control group. However, after the clinical symptom developed, the rapamycin exacerbated the disease progression and shortened the survival of ALS mice model, and apoptosis signals were up-regulated compared with control group. Conclusions: Even though further detailed studies on the relevancy between autophagy and ALS will be needed, our results revealed that the rapamycin administration was not effective for being novel promising therapeutic strategy in ALS transgenic mice and exacerbated the apoptosis.

• Annals of Clinical Neurophysiology
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• 제7권2호
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• pp.101-106
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• 2005

Background: There is no currently effective treatment for amyotrophic lateral sclerosis (ALS), although this disorder is a progressive neurodegenerative disease resulting in death within several years. Because recent evidence suggests that homocysteine (HC) is highly related to neurodegenerative disorders with aging, we tried to elucidate the effects of multi-vitamin therapy on G93A SOD1 transgenic mice. Methods: We treated this murine model of ALS with multi-vitamin (folic acid 1.97 mg/day, pyridoxine 0.98 mg/day, cyanocobalamin 0.1 mg/day) from 45 days of age, per oral. We performed the rotarod test from postnatal $10^{th}$ week, weekly. Results: We found that multi-vitamin reinforcement significantly prolonged average lifespan and delayed disease onset with improvement of motor performance. However, it did not significantly slow disease progression and statistical differences of weight loss were not observed between in transgenic mice and controls. Conclusions: These results suggest that multi-vitamin can be a potent therapeutic strategy for familial forms of ALS.

• Annals of Clinical Neurophysiology
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• 제13권2호
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• pp.71-79
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• 2011

Geographical differences in the incidence of amyotrophic lateral sclerosis (ALS) have been reported, and there are still many unresolved problems. The incidence as well as epidemiologic data of ALS is not known in Korea. Web-based multicenter registry of ALS, the Korean ALS registry, was established at January, 2011. The aim of "the Korean ALS registry" is the following: (1) to establish a database for the prospective collection of epidemiological information; (2) to assess the incidence and prevalence; (3) to find the temporal and geographic trends in the disease; (4) to define the full clinical spectrum of the disease; (5) to develop treatment guideline based on the database.

• 사상체질의학회지
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• 제26권2호
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• pp.156-164
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• 2014

Objectives The purpose of this study was to analyze the Sasang constitution and psychological traits of amyotrophic lateral sclerosis (ALS) patients and to utilize the results in treatment and management. Methods The differences of Sasang Constitution distribution, psychological characteristics, comorbid disorders between ALS group of 26 patients and general group of 1132 persons were investigated and analyzed. Results There was no significant different distribution of Sasang Constitution between ALS group and general group but there was Yang-like personality tendency in ALS group than general group. In overall constitution, ALS group had the high prevalence rate of musculoskeletal disorders and diabetes than general group. In Soyangin constitution, ALS group had a high prevalence rate of musculoskeletal disorders than genral group. In Taeeumin constitution, ALS group had a high prevalence rate of diabetes and hypertension than general group. Conclusion ALS patients have the Yang-like personality. It is due to the adaptation of personality on the environmental change or coping strategy on diseases rather than the characteristics of congenital Sasang Constitution.