Locked-In Syndrome: Case Report and Discussion of Decisional Capacity

Locked-in syndrome (LIS) is a rare neurologic disorder rendering an individual quadriplegic and anarthric with preserved self-awareness and normal if not near-normal cognition. A lesion to the ventral pons causes the classic form of LIS, and patients can typically interact with their environment with eye/eyelid movements. LIS patients may live for years with preserved quality of life (QoL) and cognitive function, but with severe disability. However, medical providers and family often underestimate the patient's QoL, and choose less aggressive care. Prompt assessment of decisionality in LIS patients is challenging, but it must be done to allow these patients to participate in their care. We present the case of a 54-year-old man with LIS. The medical team recommended comfort measures, but the family advocated involving the patient in goals of care discussions. The patient was determined to be decisional during the acute hospitalization, and he elected for life-prolonging care. This case emphasizes the importance of unbiased shared decision making, but also the importance of utilizing a practical framework to assess the decision-making capacity in these patients. We provide a suggested approach to determining decision-making capacity in similar cases or conditions.