1231 Slurred Speech- Unusual Presentation of Cataplexy in Childhood Narcolepsy

Abstract

Abstract Introduction Narcolepsy, a chronic neurological disorder of excessive daytime sleepiness, cataplexy, sleep paralysis, and hypnagogic/hypnopompic hallucinations, frequently presents in late childhood/early adolescence. Cataplexy, the most specific symptom, presents as transient loss of muscle tone causing weakness. Approximately 80% of children with narcolepsy present with cataplexy1. Report of Case Eight year old African American boy presented to sleep clinic with concerns for excessive daytime sleepiness and slurred speech. Teachers initially referred to speech therapy because of slurred speech. There, speech got noticeably worse with frustration. Subsequently, sleepiness developed, and his pediatrician referred him for polysomnogram (PSG) and Mean Sleep Latency test (MSLT). After unremarkable PSG, MSLT demonstrated mean sleep latency of 10 minutes with no SOREM sleep. Patient came for evaluation in Sleep Medicine clinic where family reported worsening speech with extreme emotions and profound sleepiness, Epworth of 20. Because of the presentation and lack of REM sleep on MSLT, brain MRI was ordered and was normal. HLA typing for narcolepsy positively showed HLA haplotype associated with narcolepsy. Patient was diagnosed with narcolepsy with cataplexy and started on Modafinil 50 mg. Modafinil was progressively increased to 200 mg in a.m. and 100 mg at noon. To address cataplexy symptoms, he started Venlafaxine 12.5 mg and increased to 25 mg. Epworth score improved on Modafinil, his cataplexy symptoms, including speech difficulties, subsided on Venlafaxine. Patient’s communication improved, as did school performance and social life, and patient is currently thriving in 10th grade. Current Epworth is 5 and current medications include Modafinil 200 mg in the morning, 100 mg at noon and Venlaflaxine 25 mg at night. Conclusion This case report highlights the importance of recognizing unusual presentations of cataplexy and the impact of treatment. These symptoms can be easily missed and erroneously attributed to developmental delay or behavioral issues.