Abstract
The amyotrophic lateral sclerosis (ALS) is a degenerative disease that compromises the motor neurons of the cortex, cerebral stem and spinal cord. Its annual incidence fluctuates among 0.6-2.6 for 1000, with a prevalence de1.6-8.5 by
100.000. ALS occurs in adult men, the majority of times without family antecedents and has a survival, in average, of two years once diagnosis is confirmed. A bulbar form of clinical presentation with faster evolution exists.
This paper presents the recommendations for management, of amyotrophic lateral sclerosis, according to evidence levels. Every patient should be well informed on its disease, its evolution and the forecast. The medical management includes measures to sialorrea control, to avoid complications of dysphagia with a gastrostomy that is indicated when the vital capacity is smaller of 50 percent. Tracheosthomy or invasive ventilation must use according to the degree of breath shortness. Pain management can be done in the framework of WHO recommendations. The pseudobulbar mode can be treating with amitryptilin or fluvoxacine
In the terminal phase physician should be accepted the prior decisions of the patient and its family and must permit a worthy decease.
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