Prognosis in early-onset ALS

Neurology has a paper which gives some very general prognostic guidance regarding survival in early-onset ALS.   The study (based on a single Italian institution's 20 year experience) compares the natural history of 57 patients diagnosed with ALS before the age of 40 with 450 older patients. 

Overall median survival was 48 months:  44 in older patients and 71 months in younger patients.  Younger patients with upper-motor neuron predominant symptoms did better than those with lower-motor neuron predominant symptoms (56 vs 74 months).  No patients with LMN-predominant symptoms lived longer than 10 years.