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Introduction Creating a guideline for the management of patients with narcolepsy is complex due to the number of potential mechanisms that could account for the increased risk of perioperative complications: aggravation of narcolepsy itself, dysautonomia, narcolepsy-related medications, anesthesia interactions, etc. Report of case(s) An 80-year-old woman with a history of narcolepsy type 1, depression, restless leg syndrome, obstructive sleep apnea, and atrial fibrillation presented to our sleep clinic to establish care. Her narcolepsy was managed with strategic naps, but no medications. She had previously experienced rare episodes of cataplexy, described as the inability to talk, and syncope. While on anticoagulation for her atrial fibrillation, she sustained a fall during an episode of cataplexy resulting in brain bleed and eye loss, which prompted her to start pitolisant. Subsequent polysomnography showed an apnea-hypopnea index of 72/hr with nadir oxygen of 74%, requiring Adaptive Servo-Ventilation (ASV), which she did not tolerate. She noted cognitive decline and extreme weakness resulting in hospitalization, during which neurology reported frequent myoclonic jerks and excessive sleepiness. Given her untreated sleep apnea, we recommended re-initiation of ASV during the inpatient hospital stay, which improved her symptoms significantly. She subsequently developed an unrelated need for urgent bowel surgery, necessitating cessation of oral intake, which was temporally related to debilitating and refractory cataplexy. Following recovery from this acute hospitalization, she recovered and was again seen in the clinic, with no further episodes of cataplexy and improved sleepiness in the setting of ASV compliance and resumption of her pitolisant. Conclusion This case emphasizes the importance of controlling even rare cataplexy in the setting of potentially catastrophic outcomes. It also raises concerns about the management of narcolepsy with cataplexy in the perioperative period, which currently lacks quality data and guidelines. Support (if any)
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Buntak et al. (2024) studied this question.
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