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Ehlers-Danlos syndrome (EDS) is a genetically inherited connective tissue disorder which has a high prevalence of sleep conditions, including obstructive sleep apnea, insomnia, fatigue, and hypersomnia., Chronic fatigue is an important factor in the impaired quality of life in patients with EDS. Successful treatment of fatigue and hypersomnia with traditional wake-promoting medications and stimulants is limited, due to the high prevalence of postural tachycardia, orthostatic intolerance, and other cardiac conditions in these patients. We present a case of EDS with underlying cardiac comorbidities, hypersomnia and fatigue who had significant improvement in excessive daytime sleepiness after treatment with flumazenil. Report of Case A 19 yoF with PMH of Ehlers-Danlos syndrome, postural tachycardia syndrome (POTS), autonomic instability and well-controlled depression presented with symptoms of fatigue and excessive daytime sleepiness (ESS of 17/24) despite obtaining 10-15 hours of sleep each day. Polysomnogram followed by MSLT was notable for borderline excessive sleepiness without other abnormalities (PSG: AHI 3/hr, SpO2 nadir 92%; MSLT: 0 SOREMS, mean sleep latency 10 minutes). Prior autonomic and cardiac work-up revealed POTS (maximum HR 180 bpm), orthostatic intolerance and aortic root dilatation. Physical exam and previous laboratory work up for fatigue were unremarkable. A trial of flumazenil 6 mg lozenge every 4-6 hours, as needed for sleepiness, was initiated. On her subsequent visit 6 months later, patient reported 50% improvement in symptoms of fatigue and sleepiness, with decrease in ESS from 17 to 5. No adverse effects to flumazenil were reported. Conclusion Flumazenil is a gamma-aminobutyric acid (GABA)-A receptor antagonist that has been previously documented to provide sustained clinical benefit in treatment-refractory hypersomnolence. This case report highlights a successful alternative treatment option for hypersomnolence in patients with EDS and cardiac comorbidities, in which traditional wake-promoting agents and stimulants may be contraindicated.

1221 Flumazenil for the treatment of hypersomnolence and fatigue in a patient with Ehlers-Danlos syndrome with multiple cardiac comorbidities