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Death represents the main hallmark of amyotrophic lateral sclerosis (ALS). Despite its importance in clinical care and phase III trials, many uncertainties remain on the cause of death due to the lack of post‐mortem verifications. To provide a more robust approach to these causes, we performed a retrospective pathological study on a large cohort of patients.

100 ALS patients referred for a deterioration of their clinical condition and who died in the ALS clinic of Salpétrière had a complete macroscopic and microscopic post‐mortem analysis. The clinical causes of death reported on medical records were compared to the results of autopsy. The concordance between clinical and pathological conclusions was insufficient (20%) to consider clinical assessment as a reliable marker of causes of death. At autopsy, broncho‐pneumonia and pneumonia were the main causes of death. Heart failure, representing 10% of deaths …