The clinical features, pathology, the autopsy studies, EEG correlation, and the cause of death were discussed. The difference between the locked-in syndrome and classical akinetic mutism was discussed. The importance of reaching a correct diagnosis was stressed to allow a different nursing care program for the conscious "locked-in" patient. Two of our cases were short-term survival, two were long-term survival, one of whom (Case 4) is still living and is the longest survival reported in the literature.