ALS causes a consistent, predictable deterioration in motor function, leading to tracheostomy in most patients within 2 to 4 years. 8 ALS leads to complete respiratory failure in 80 percent and complete limb paralysis in 45 percent of patients. Although early diagnosis and aggressive management have increased the median survival of sALS, 50 percent of patients die within 3 years of diagnosis, and only 10 percent survive beyond 10 years. Patient diagnosed prior to age 40 may live up to four times longer than those diagnosed after age 60, especially if swallowing and airway control are spared. Survival in fALS is shorter, with a median survival of 2 years and a 23 percent 5-year survival rate. Long-term outcome is currently being followed in a longitudinal European survey termed the European ALS Health Profile Study.
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