BrainWaves #168 (Mis)understanding the locked-in state

MedLink Neurology Podcast is delighted to feature selected episodes from BrainWaves, courtesy of James E Siegler MD, its originator and host. BrainWaves is an academic audio podcast whose mission is to educate medical providers through clinical cases and topical reviews in neurology, medicine, and the humanities, and episodes originally aired from 2016 to 2021.

Originally released: July 23, 2020

Locked-in syndrome is a rare clinical consequence following many types of neurologic injury. In general, the locked-in patient is fully paralyzed, with perhaps minimal function of the fingers, eyes, or mouth. What's more, gross motor dysfunction is enormously disproportional to the cognitive function of the individual. The locked-in patient is conscious and completely aware of their surroundings. They can often hear, see, smell, and feel just as any other person would. But because of their profound physical disability, they have a very limited means of communicating even the simplest thoughts. "I feel hot." "My head hurts." "My cheek itches."

It may surprise you that the patient's perspective of their condition is wholly different from how the healthcare provider or caregiver imagines it to be. This week on the program, Dr. Lauren Elman (Pennsylvania Medical Center ALS Multi-disiplinary Clinic) reviews this discrepancy. Dr. Elman also shares her experience managing this inevitable consequence of amyotrophic lateral sclerosis when all life-sustaining measures are desired.

REFERENCES

Kuzma-Kozakiewicz M, Andersen PM, Ciecwierska K, et al. An observational study on quality of life and preferences to sustain life in locked-in state. Neurology 2019;93(10):e938-45. PMID 31391247

Niedermeyer S, Murn M, Choi PJ. Respiratory failure in amyotrophic lateral sclerosis. Chest 2019;155(2):401-8. PMID 29990478

Patterson JR, Grabois M. Locked-in syndrome: a review of 139 cases. Stroke 1986;17(4):758-64. PMID 3738962

Rousseau MC, Baumstarck K, Alessandrini M, Blandin V, Billette de Villemeur T, Auquier P. Quality of life in patients with locked-in syndrome: evolution over a 6-year period. Orphanet J Rare Dis 2015;10:88. PMID 26187655

Trail M, Nelson ND, Van JN, Appel SH, Lai EC. A study comparing patients with amyotrophic lateral sclerosis and their caregivers on measures of quality of life, depression, and their attitudes toward treatment options. J Neurol Sci 2003;209(1-2):79-85. PMID 12686407

We believe that the principles expressed or implied in the podcast remain valid, but certain details may be superseded by evolving knowledge since the episode’s original release date.