No abstract available.
Amyotrophic Lateral Sclerosis ; CADASIL

No abstract available.
Amyotrophic Lateral Sclerosis ; CADASIL

Background: Amyotrophic lateral sclerosis is one of the neurodegenerative disorders with very limited treatment options owing to its progressive course and diverse pathophysiology. Majority of patients succumb to death within three to five years after the onset of symptoms, mostly due to respiratory failure. This study aimed to determine the efficacy and safety of ultra-high dose methylcobalamin versus placebo among patients with early-stage amyotrophic lateral sclerosis in terms of slowing down functional decline. Methods: MEDLINE, CENTRAL, and Google Scholar databases were searched from inception up to September 23, 2023. The impact of treatments was measured by risk ratios with 95% confidence interval. The overall certainty of the evidence was evaluated using GRADE. Results: No significant difference was detected for the outcome median change in the ALSFRS-R score for the whole cohort. Post-hoc analyses showed that ultra-high-dose methylcobalamin decreased ALSFRS-R scores (p=0.003 for 50 mg and p=0.01 for all methylcobalamin groups) in a dose-responsive manner. Mean difference was 1.97 in favor of methylcobalamin (95% CI, 0.44- 3.50; P = .01). Conclusion Ultra-high dose methylcobalamin can reduce ALSFRS-R scores of patients in its early stage but the scarcity of clinical trials makes it difficult to support a robust conclusion. Ultra-high dose methylcobalamin therapy remains to be investigational.
Amyotrophic Lateral Sclerosis ; Systematic Review

No abstract available
Amyotrophic Lateral Sclerosis* ; Apraxias* ; Eyelids* ; Humans

PURPOSE: The purpose of this study was to explore the psychosocial responses and quality of life (QOL) among Amyotrophic Lateral Sclerosis (ALS) patients and their caregivers in South Korea. METHODS: A cross-sectional design was used. Purposive sample of 15 ALS patients and their 14 caregivers were recruited via Korean Amyotrophic Lateral Sclerosis Association (KALSA) website. Demographic characteristics, hopelessness, quality of life, physical function, and caregiver burden were measured. RESULTS: The mean period after being diagnosed with ALS was 57.73 months. The mean score of amyotrophic lateral sclerosis functional rating scale and quality of life was 21.33 (SD=11.97) and 5.70 (SD=1.23) respectively. The mean score of hopelessness was 11.87 (SD=4.72). The caregivers' mean score of McGill quality of life was 4.29 (SD=1.46), and the mean score of McGill quality of life-single item scale was 4.29 (SD=2.02). CONCLUSION: Since the cause of ALS has not been identified and cure is yet to be discovered, supportive care should be provided for not only quality of life but hope of patients. The findings may be used to develop knowledge based nursing intervention for patients diagnosed with ALS and their caregivers.
Amyotrophic Lateral Sclerosis ; Caregivers ; Humans ; Quality of Life

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.
Amyotrophic Lateral Sclerosis ; Dysarthria ; Humans ; Neurodegenerative Diseases* ; Parkinson Disease ; Rehabilitation*

OBJECTIVE: The purpose of this study was to invent a new communication device by utilizing the function of anal sphincter muscles that was another residual motor function to the last in amyotrophic lateral sclerosis (ALS) patients. METHOD: We designed a sensor which worked by changing intensity of the contraction and was enable severely paralyzed patients to communicate by means of self-regulation of the anal sphincter contraction. This device made sentences through combination of selected Korean letters deprived from sphincter contractions. RESULTS: This device was composed of three parts. Anal sensor: the head part of the sensor regulated by sphincters was located in the rectum, signal processing module: the raw signal from the sensor was boosted up and set up on the baseline voltage equal to threshold by threshold detector, and device program: the icon mode which was composed of routine questions and requested to their care-givers. And in user-writing mode, patients could choose syllable elements using the sensor operating by sphincter. CONCLUSION: This system was designed for advanced ALS patients with only the available function of sphincter muscles and could give them another new option in selecting communication devices.
Amyotrophic Lateral Sclerosis ; Anal Canal* ; Head ; Humans ; Muscles ; Rectum

PURPOSE: The purpose of this study was to identify factors influencing psychosocial well-being in family caregivers of patients with amyotrophic lateral sclerosis (ALS). METHODS: A descriptive correlational design was used. The transactional model of stress and coping was used to investigate the psychosocial well-being of 137 family caregivers of patients with ALS. Data were collected through self-reported questionnaires from January to November 2016. Data were analyzed using an independent t-test, one-way ANOVA, Pearson's correlation, and hierarchical multiple regression analysis with the SPSS WIN 21.0 program. RESULTS: The regression model had an adjusted R2 of .49, which indicated that meaning-focused coping, social support, ALS patient-family caregiver relationship (especially a spousal relationship), and tracheostomy were significant predictors of caregivers' psychosocial well-being. CONCLUSION: Meaning-focused coping and social support significantly influenced caregivers' psychosocial well-being. Therefore, interventions to improve caregivers' psychosocial well-being must focus on increasing meaning-focused coping and social support resources.
Adaptation, Psychological ; Amyotrophic Lateral Sclerosis* ; Caregivers* ; Humans ; Social Adjustment ; Tracheostomy

No abstract available.
Amyotrophic Lateral Sclerosis* ; Charcot-Marie-Tooth Disease* ; China* ; Pedigree*

Amyotrophic lateral sclerosis (ALS) is a relentless, progressive, and presently incurable neurodegenerative disease. Its drug development has been hampered by the lack of effective biomarkers for early diagnosis, progression and prognosis. Recently, significant progress has been made for the identification of body fluid biomarkers for ALS, which conferred both theoretical and practical feasibility for the early diagnosis and progression monitoring. Meanwhile, it also facilitated identification of genes and/or pathways for the pathogenesis of ALS. This review summarized biomarkers identified from cerebrospinal fluid, blood and urine of ALS patients and their clinical implications.
Amyotrophic Lateral Sclerosis/genetics* ; Biomarkers ; Humans ; Neurodegenerative Diseases ; Prognosis