Objectives‐ To investigate if degeneration of motor neurons in amyotrophic lateral sclerosis (ALS) is related to altered levels of the apoptosis regulating proteins Bcl‐2 and Bax. In addition, immuno‐reactivity of the cysteine protease ICH‐1_L and detection of motor neurons with DNA fragmentation, indicative of apoptosis, was also studied. Material and methods‐ The immunoreactivity of Bcl‐2, Bax and ICH‐1_L were compared in ALS and control spinal cord motor neurons by immunohistochemical analysis and motor neurons with DNA fragmentation were identified by the TUNEL‐method. Results‐ The results demonstrate an increased expression of Bax in the ALS material as compared to controls but no change in Bcl‐2 and ICH‐1_L expressions. Moreover, a larger proportion of motor neurons stained positive for TUNEL in ALS spinal cords. Conclusion‐ Present study suggest an upregulation of the cell death promoting protein Bax and increased DNA degradation, indicative of apoptosis, in spinal motor neurons of ALS patients.