Locked-in syndrome in Sweden, an explorative study of persons who underwent rehabilitation: a cohort study

OBJECTIVE: Locked-in syndrome (LiS) is a rare condition, characterised by a complete paresis except for vertical eye movements and blinking with cognitive functions intact, commonly caused by ischaemia in the ventral pons. Previous studies have indicated that persons with LiS can live on for many years and have a good quality of life (QoL). To our knowledge, LiS has never been studied in Sweden. The aim was to explore LiS in Sweden; describing population characteristics, living situation, mortality/cause of death and health-related QoL/impact on participation. DESIGN: Explorative, nationwide study with two parts (quantitative and qualitative). Persons registered in the national quality register WebRehab during 2007–2014 were eligible. PARTICIPANTS: Ten persons were identified in part 1, four participated in part 2. During part 1, data were collected from WebRehab, medical charts and registers, while questionnaires and interviews were used during part 2. RESULTS: Seven out of 10 were men, median age at onset was 49 years and the cause of LiS was in all cases stroke, 70% of which ischaemic. Three were deceased with a median time of survival of 1.9 years. Seven were still alive, with a median time elapsed since onset of 5.8 years. Three participants experienced good QoL. Information, respect from professionals and more specialised technical devices were three areas containing unfulfilled needs. CONCLUSION: This was the first study conducted in Sweden and the characteristics of this population were like those studied abroad. In this study, the persons with LiS who were interviewed expressed the need for proper care, appropriate technical aids and a supportive environment in order to have QoL. However, there is still much room for improvements.