1353 Management of SARS-CoV-2 Associated Narcolepsy Type 1 in a US Airforce (USAF) Pilot

Abstract Introduction Narcolepsy Type 1 (NT1) is associated with immune-mediated loss of lateral hypothalamus hypocretin neurons. Several cases of NT1 diagnoses related to autoimmune diseases, H1N1 infection, and influenza vaccination are reported. One published case of NT1 associated with SARS-CoV-2 infection reports partial symptomatic improvement on modafinil and venlafaxine. This case describes the diagnosis and treatment of the first published CSF-confirmed case of NT1 with severe cataplexy following SARS-CoV-2 infection. Report of case(s) A 30-year-old M Pilot with no previous history of excessive daytime sleepiness (EDS) presented with URI symptoms and tested positive for SARS-CoV-2 infection. Over the subsequent 7 months, despite sleeping 7-8 hours nightly, he developed rapidly progressive EDS including falling asleep while flying, frequent (5-10 episodes/day)cataplexy, sleep paralysis, and hypnagogic/hypnopompic hallucinations. Hypersomnia evaluation demonstrated an apnea-hypopnea index of 6.2 on overnight polysomnogram with a next day mean sleep latency of 3.6 minutes with 4/5 sleep onset REM periods. Human leukocyte antigen haplotype showed heterozygosity for DQB1*06:02. CSF analysis revealed Orexin-A at < 50pg/mL. Trials of modafinil 400 mg, armodafinil 150 mg, venlafaxine 75 mg, and as-needed methylphenidate resulted in mild improvement in EDS and improvement of cataplexy but caused anxiety and palpitations. Weaning medications to achieve monotherapy with pitolisant 36.5mg caused status cataplecticus (15-20 episodes/day) with no improvement in EDS. Addition of venlafaxine significantly reduced cataplexy. Long acting (LA) sodium oxybate 7.5 mg, venlafaxine 150 mg po bid, and LA methylphenidate 30 mg ultimately controlled EDS and reduced cataplexy to an acceptable level. Conclusion This case describes the onset and therapeutic course of a patient with SARS-CoV-2 associated NT1. It is difficult to generalize from a single case, particularly given the atypical severity of patient’s cataplexy. Thus, future studies will be needed to confirm if pitolisant is ineffective in SARS-CoV-2 associated NT1 cases and if multimodal therapy is always required. Support (if any) The views expressed herein are those of the authors and do not reflect the official policy of the Department of War or U.S. Government.