Rare Diseases Symptoms Automatic Extraction

Fragmentation of slow wave sleep after onset of complete locked-in state.

[locked-in syndrome]

Locked-in syndrome (LIS) as a result of brainstem lesions or progressive neurodegenerative disorders, such as amyotrophic lateral sclerosis (ALS), is a severe medical condition in which a person is fully conscious but unable to move or talk. LIS can transition into complete locked-in syndrome (CLIS) in which residual abilities to communicate through muscle twitches are entirely lost. It is unknown how CLIS affects circadian rhythm and sleep/wake patterns. Here we report a 39-year-old ALS patient who transitioned from LIS to CLIS while brain activity was continuously recorded using electrocorticography (ECoG) over one month. While we found no circadian rhythm in heart rate and body temperature, transition into CLIS was associated with increased fragmentation of slow wave sleep (SWS) across the day. Total time in SWS did not change. SWS fragmentation might reflect progressive circadian system impairment and should be considered as a factor further limiting communication capabilities in these patients.