BackgroundImproved knowledge of the quality of life (QoL) of locked-in syndrome (LIS) patients have implications for managing their care, and assists clinicians in choosing the most appropriate interventions. We performed a survey of a population of LIS patients to describe the course of the QoL of LIS patients over a 6-year period and to determine the potential predictive factors of QoL changes over time.MethodThis is a study performed over a 6-year period in patients with a LIS diagnosis. Questionnaires were sent in 2007 and 2013. The following data were recorded: i) sociodemographic data; ii) clinical data related to LIS, physical/handicap status, psychological status; iii) self-reported QoL: Anamnestic Comparative Self-Assessment (ACSA); iv) Integration in life: French Reintegration to Normal Living Index (RNLI).ResultsAmong the 67 patients included in 2007, 39 (58 %) patients returned their questionnaire in 2013. The LIS etiology was stroke in 51 individuals. The QoL of the patients was relatively satisfactory compared to populations in other severe conditions. Twenty-one (70 %) individuals reported a stable/improved QoL between 2007 and 2013. The physical/handicap statuses in 2007 and 2013 were not related to the QoL 6 years later, with the exception of one communication parameter: the individuals who used yes-no code reported significantly lower QoL levels than those who did not in 2013.DiscussionIn opposition to a widespread opinion, LIS persons report a relatively satisfactory QoL level that stays stable over time, suggesting that life with LIS is worth living. Preservation of autonomy and communication may help them to live as normal life as possible.

There are very few studies where quality of life (QOL) is assessed in patients with complete physical and functional disability and dependence to invasive mechanical ventilation (IV). We compared QOL of amyotrophic lateral sclerosis (ALS) and locked-in-syndrome (LIS) patients with invasive mechanical ventilation to ALS and LIS patients without mechanical invasive ventilation. Thirty-four patients, 27 with ALS and seven with LIS (vascular or tumoral aetiology) were included in the study. Twelve had invasive ventilation, 22 had non-invasive ventilation, and in the non-invasive ventilation group, five of them had ventilation via mask. The following scales were used for patients: ALS Functional Rating Scale (ALSFRS), McGILL, Short-Form 36 (SF36), Beck Depression Inventory-II, the Toronto Alexithymia Scale and the anxiety inventory of Spielberger. Mean ALSFRS scores were significantly lower in the invasive ventilation group (IV) than in the non-invasive ventilation group. McGILL and SF36 were not significantly different between the IV group and the non-invasive ventilation group; there were no significant differences between the two groups for others scales either. Comparison between IV group and LIS without invasive mechanical ventilation revealed no significant difference for SF36 and McGILL QOL scores. QOL was not significantly different between the IV and not invasively ventilated patients, but ALSFRS was significantly lower in the IV group, and comparison of QOL scores between non-ventilated LIS patients who had the same score of dependence that invasively ventilated patients did not show any difference. Invasive mechanical ventilation for patients who accept tracheotomy allows life prolongation and their QOL is not affected; medical teams should be aware of that.