نتایج جستجو برای: sporadic amyotrophic lateral sclerosis sals
تعداد نتایج: 198646 فیلتر نتایج به سال:
background: the rapidity of progression of amyotrophic lateral sclerosis (als) to death or respiratory failure impacts patients, clinicians, and clinical investigators. the aim of this study is to evaluate of the pulmonary function tests (pfts) in patients with als and the association between these pfts and survival methods: a total of 36 als patients who pfts, including vital capacity (vc), ma...
Neurodegenerative diseases are the hereditary and sporadic diseases which are characterized by progressive neuronal loss of the nervous system and are emerging as the leading cause of death, disabilities, and a socioeconomic burden due to an increase in life expectancy. There are many neurodegenerative diseases including Alzheimer’s disease, Parkinson’s, disease, amyotrophic lateral...
background: as a disease of motor nervous system (motor neuron disease), amyotrophic lateral sclerosis (als) has a great impact on several aspects of quality of life (qol). generic questionnaires of qol do not address all the especial features of als and therefore translation and validation of disease specific questionnaires such as amyotrophic lateral sclerosis assessment qu...
BACKGROUND Mutations in the valosin-containing protein (VCP) gene were first found to cause inclusion- body myopathy with early-onset Paget disease and frontotemporal dementia (IBMPFD). Mutations in the VCP gene were later reported to occur in familial amyotrophic lateral sclerosis (ALS). But the role of VCP in the neurodegenerative processes that occur in ALS remains unknown. The purpose of th...
Objective. To illustrate that sporadic amyotrophic lateral sclerosis (ALS) may be caused by ischemia in the intraparenchymal territory of the anterior spinal artery (ASA) and/or anterior-ventral spinal arteries (AVSAs). Case report. A 56-year-old woman presented clinical data of spinal and bulbar forms of ALS. Previously she was attended in several neurological centers. Results. In 2002 the pat...
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized clinically by rapidly progressive paralysis leading ultimately to death from respiratory failure. There is substantial evidence suggesting that ALS is a heritable disease, and a number of genes have been identified as being causative in familial ALS. In contrast, the genetics of the much commoner sporadic for...
Sporadic amyotrophic lateral sclerosis (sALS) is a progressive degenerative motor neuron disorder lacking specific treatment. Riluzole is the only drug able to modestly slow down the course of the disease. Respiratory insufficiency is the main cause of death; non invasive ventilation (NIV) has shown to improve survival. Our aim was to evaluate the effect of NIV and riluzole on survival. Ninety ...
Autophagy plays a major role in the elimination of cellular waste components, the renewal of intracellular proteins and the prevention of the build-up of redundant or defective material. It is fundamental for the maintenance of homeostasis and especially important in post-mitotic neuronal cells, which, without competent autophagy, accumulate protein aggregates and degenerate. Many neurodegenera...
Amyotrophic lateral sclerosis (ALS) is a fatal disease characterized by progressive motor neuron degeneration and neurofilament aggregate formation. Spinal motor neurons in ALS also show a selective suppression in the levels of low molecular weight neurofilament (NEFL) mRNA. We have been interested in investigating the role of microRNAs (miRNAs) in NEFL transcript stability. MiRNAs are small, 2...
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