1256 Narcolepsy Type 2 in a Pediatric Patient with Auto-Immune Disease

Abstract

Abstract Introduction We present a case of Narcolepsy Type 2 in the setting of concomitant auto-immune disease. Report of Case At 9 years of age, an African-American female was referred to a sleep center for fatigue, excessive daytime sleepiness (EDS), and snoring. Polysomnogram (PSG) at an outside facility recorded a total sleep time (TST) of 408 minutes, 92% sleep efficiency (SE), sleep onset latency (SOL) 22 minutes, REM latency 70 minutes, and apnea-hypopnea index (AHI) of 0.3. Multiple Sleep Latency Test (MSLT) was not done at that time. Due to her daytime sleepiness, methylphenidate was initiated empirically twice daily with improvement in daytime sleepiness and sleep maintenance. Over time, methylphenidate was steadily increased due to increasing daytime sleepiness. Between 14 to 15 years of age, she developed increased fatigue, skin changes, arthralgias, myalgias, and presented to our hospital in respiratory distress secondary to severe pulmonary hypertension. Clinical exam and workup indicated scleroderma. Methylphenidate was discontinued due to pulmonary hypertension. At 17 years of age (after receiving treatment for scleroderma and pulmonary hypertension), she had an overnight PSG followed by MSLT. PSG recorded TST 309 minutes, 67% SE, SOL 1.5 minutes, REM latency 48 minutes, AHI 0.2. The MSLT recorded a mean SOL of 2.4 minutes with 2 out of 4 sleep onset REM periods - diagnostic of narcolepsy. She does not endorse cataplexy, hallucinations or sleep paralysis. Conclusion While the link between Narcolepsy Type 1 and an auto-mediated process is more supported given consistent human leukocyte antigen findings, the link between Narcolepsy Type 2 and auto-immune mediated process is less clear. Patients with autoimmune disease may have symptoms of narcolepsy; therefore, they may benefit from screening for EDS.