“Am I locked-in?” Illness Trajectories, Medical Terminology, and the Experience of the Locked-in State in Japan and Europe

Abstract

Locked-in syndrome consists of anarthria and quadriplegia with preservation of consciousness and cognitive faculties. It results mostly from brainstem stroke. In its late stages, amyotrophic lateral sclerosis (ALS) can lead to a locked-in state. What is it like to be locked-in? To investigate this question in a comparative perspective, we distributed semi-structured open-ended questionnaires to persons in the locked-in state/with locked-in syndrome (PLIS) in Japan, Spain and France. Most Japanese were in LIS due to ALS (PLIS-ALS); most Europeans, in LIS of vascular origin (PLIS-stroke). We report here on responses to the questions “What do you think about the official name of the diagnosis (Locked-In Syndrome)?” and “Sometimes people with LIS are described as ‘a mind trapped in a body.’ What do you think about it? Why?” PLIS-stroke said “locked-in syndrome” corresponded to their situation. Some accepted the trapped mind image; others did not or only for certain circumstances. In contrast, most PLIS-ALS rejected both as inaccurate because they could communicate. The difference between the two groups reflects diverging illness trajectories. PLIS-stroke experienced a sudden entrance into LIS and a period of quasi absolute “captivity,” from which they emerged gradually. PLIS-ALS experience the opposite, since their illness can gradually take them to an increasingly locked-in state. In light of the research presented here, we recommend placing less emphasis on the locked-in terminology, and eventually envisaging a more purely descriptive name.