Sclerosis lateralis amyotrophica
Journal Title: Postępy Nauk Medycznych - Year 2010, Vol 23, Issue 6
Abstract
Sclerosis lateralis amyotrophica (ALS) is a progressive neurodegenerative disorder, of unknown etiology and pathogenesis leading to upper and lower motor neuron degeneration. The prevalence of ALS is approximately 7/100,000. The average age of onset of ALS is 65 years, 20% cases of the SALS begins over the age of 70 years, which means ALS is also the neurodegenerative disorder of elderly people. The diagnosis of ALS id based on correlation of clinical symptoms, neurophysiologic studies, neuroimaging and some serological tests. The direct cause of ALS is unknown, although there are genetic risk factors of this disease. In ALS there is a special vulnerability of motor neurons to glutaminergic excitotoxicity. Axonal transport defects, abnormal mitochondrial function and oxidative stress are also involved in the pathogenesis of ALS. The majority of authors' agree with the hypothesis of complex etiology for SLA which is a result of interaction of genetic and environmental factors. Despite many clinical trials and the progress in understanding of molecular pathology of ALS, there are no medicines effectively inhibiting the disease progress. Riluzole is the only drug registered by FDA/EMEA with proved small effectiveness in life prolongation of ALS patients. Treatment of ALS changed considerably during the last years, currently the emphasis is placed on comprehensive mulitispecialist care. Despite, that disease is incurable, many of ALS symptoms can be alleviated, the aim of management should be the improvement of quality of life and supporting the patient's independence as long as possible
Authors and Affiliations
Justyna Zajączkowska, Hubert Kwieciński
Keywords
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