Review of: Kuzma-Kozakiewicz M et al, Neurology 2019;93(10):e938–e945

Study Type: Observational cross-sectional

Imagine being completely alert and aware but unable to move or speak. Patients with locked-in syndrome, a catastrophic result of neurologic disorders such as stroke or amyotrophic lateral sclerosis (ALS), live this way for years. Better off dead? Not according to this observational study that examined quality of life and preferences to sustain life in the locked-in state.

The authors of this exploratory cross-sectional study contacted 103 caregivers of ALS patients with locked-in syndrome. 25 caregivers responded, and 19 patients were evaluated with questionnaires that assessed psychosocial adaptation by multiple measures, including subjective quality of life, depression, wish for hastened death, and satisfaction with life-sustaining care such as assisted ventilation and tube feedings. Caregivers were also asked how they thought patients would judge their own quality of life. With only eye movement, these patients answered questions through blink responses or a visual keyboard with laser eye tracking technology.

Surprisingly, only 7 of 19 patients reported poor quality of life; the rest deemed it satisfactory. Even the 5 patients with no residual physical function reported positive quality of life and no depression. Overall, patients did not want to die, and almost all (17 of 19) reported they would select invasive life-sustaining measures if given the option again. About half the patients were dysthymic, a quarter were clinically depressed, and the rest were not depressed at all. Caregivers tended to underestimate patients’ sense of well-being.

Notably, these were long-term patients who had time to adapt to their circumstances. The fact that they had caregivers who lived in the home and made the effort to respond to the research team’s recruitment email could indicate a skewed sample with unusually strong psychosocial support. Other limitations include the small size, low response rate, and observational nature. However, the results are consistent with earlier studies that found a higher-than-expected quality of life in patients with locked-in syndrome. This study was the first to show that severity of disability did not predict a poorer quality of life.

TCPR’s Take
Life can be meaningful even in the face of formidable symptoms. When we encounter patients with severe physical limitations, we can offer hope that successful adaptation is possible. Depression is not an inevitable result of locked-in syndrome and it needs to be treated when encountered in this population.

To learn more, listen to our 6/1/20 podcast, “What Locked-In Syndrome Teaches Us About Happiness.” Search for “Carlat” on your podcast store.