Using a cohort of 99 patients with ALS who were representative of all patients with ALS diagnosed during 2015 to 2018 in Stockholm, Sweden, we found that higher levels of TC, LDL-C, LDL-C/HDL-C ratio, ApoB, and ApoB/ApoAI ratio were statistically significantly associated with a lower risk of mortality, after adjustment for all other known prognostic indicators of ALS. Here, APOB is linked to amyotrophic lateral sclerosis.