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• W4387247965 abstract "SESSION TITLE: Challenging Cases in Sleep Medicine SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/09/2023 02:10 pm - 02:55 pm INTRODUCTION: Narcolepsy presents as excessive daytime sleepiness with or without cataplexy, hypnagogic hallucinations and sleep paralysis. With an overall prevalence of 25-50 per 100,000 people, it manifests as a rare disease and often goes undiagnosed. Diagnosis of narcolepsy requires daily periods of irrepressible urge to sleep or sleep attacks for at least 3 months. For narcolepsy type 1, the patient will have cataplexy with a mean sleep latency test (MSLT) of ≤ 8 minutes with ≥2 sleep onset of rapid eye movement periods (SOREMPs) during the MSLT and/or a low CSF orexin-A level. Narcolepsy type 2 will present with similar MSLT findings without cataplexy along with normal/no CSF orexin-A levels and hypersomnolence not better explained by another cause. This case details a patient presenting with recurrent seizures since childhood who, at the age of 58, had resolution of seizures with diagnosis and treatment for narcolepsy type I. CASE PRESENTATION: A 59-year-old female presented to her cardiologist for supraventricular tachycardia and autonomic dysfunction treated with propranolol and midodrine with complaints of persistent syncope. At this office visit, an event recorder showed her syncope is not related to arrhythmia, and she was noted to have excessive daytime sleepiness (EDS). She subsequently was referred to a sleep physician and diagnosed with mild obstructive sleep apnea (OSA) with an apnea/hypopnea index of 5 on PSG followed by an MSLT. Her PSG revealed a SOREMP, and her MSLT revealed 3 out of 4 nap tests positive for SOREMPs, mean sleep latency of 4.8 minutes. She additionally noted that her history of seizures was brought on by laughter, sadness, fear, or people yelling at her. She was diagnosed with narcolepsy with cataplexy and subsequently started on sodium oxybate and armodafinil with complete resolution of her cataplexy. Mixed amphetamine salts were later added for persistent hypersomnolence. DISCUSSION: The diagnosis of narcolepsy requires a clinician to first think of it in the differential. Generally, workup includes a PSG followed by an MSLT. To meet criteria for diagnosis of narcolepsy with cataplexy, the patient must have at least 3 months of symptoms. A SOREMP noted on a PSG does count toward MSLT results, and MSLT must show at least 1 additional SOREMP (2+ total). If negative, a low CSF orexin-A can satisfy the second requirement toward narcolepsy type I. First line therapy includes scheduled naps with wake-promoting medications based on severity of EDS. Generally, modafinil is considered first line with addition of oxybate or other stimulant-type medications for wakefulness promoting agents if EDS persists. CONCLUSIONS: This 59-year-old female presented with recurrent syncope in the setting of SVT and hypotension. The patient endorsed a history of chronic hypersomnolence, and her astute physician referred the patient to sleep medicine for further evaluation. Her childhood seizure disorder turned out to be a classic tale of narcolepsy with cataplexy, undiagnosed and untreated for 58 years. The patient had excellent response to her medications with complete resolution of her cataplexy. This case exemplifies the importance of keeping a broad differential with high index of suspicion for sleep disorders. REFERENCE #1: Dauvilliers Y, Arnulf I, Mignot E. Narcolepsy with cataplexy. The Lancet. 2007;369(9560):499-511. doi:https://doi.org/10.1016/s0140-6736(07)60237-2 REFERENCE #2: Longstreth WT, Koepsell TD, Ton TG, Hendrickson AF, van Belle G. The Epidemiology of Narcolepsy. Sleep. 2007;30(1):13-26. doi:https://doi.org/10.1093/sleep/30.1.13 REFERENCE #3: Practice Guidelines - American Academy of Sleep Medicine – Association for Sleep Clinicians and Researchers. American Academy of Sleep Medicine – Association for Sleep Clinicians and Researchers. Published 2017. https://aasm.org/clinical-resources/practice-standards/practice-guidelines/ DISCLOSURES: No disclosure on file for Donald DeCoy No relevant relationships by David Vavrinak-Davis" @default.
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• W4387247965 date "2023-10-01" @default.
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• W4387247965 title "NARCOLEPSY WITH CATAPLEXY PRESENTING AS LONGSTANDING SEIZURES" @default.
• W4387247965 doi "https://doi.org/10.1016/j.chest.2023.07.4091" @default.
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