1391 Sleep Disturbance in Huntington's Disease: A Case Report

Abstract Introduction Huntington's disease (HD) is an autosomal dominant genetic neurodegenerative disorder which often overlaps with various sleep disorders. Research suggests that underlying sleep dysfunction in HD impairs quality of life, can exacerbate symptoms, and may accelerate neurodegeneration. There is limited evidence on managing sleep disorders in patients with HD. We present a challenging case of a patient with HD, chronic insomnia, obstructive sleep apnea, and restless leg syndrome. Report of case(s) A 42-year-old male with HD presented to sleep medicine clinic for evaluation of sleep disturbances. His main concern was chorea during sleep, RLS, and sleep maintenance insomnia. He did snore, especially in supine position. He had a bedtime of 10:30 pm, sleep latency of 10 minutes, waketime 7-8:00 am, 12-night awakenings, total estimated sleep time of 5-6 hours. Medications at bedtime included gabapentin 900mg, quetiapine 50mg, clonazepam 1mg, and melatonin 5mg. In the past, insomnia did not improve with trazodone or doxepin. A sleep study was recommended to evaluate for sleep apnea. Gabapentin dose was increased to 300mg 6 hours prior to bedtime, 900mg 1hour prior to bedtime. A home sleep apnea test (HSAT) showed an AASM (3% desaturation) apnea hypopnea index (AHI) of 30.0/hr, O2 nadir 86%, supine AHI 78.3/hr. The patient started Auto-PAP and had follow-up 3 weeks later. Download showed total usage days of 94%, average usage of 4.75 hours, APAP 6-16cmH2O, residual AHI 8/hr, central apnea index of 6.8/hr, and minimal mask leak. His RLS did improve with adjusting medication timing, but he continued to experience chorea during sleep, which woke him up multiple times per hour. Plan included iron studies, serum ferritin, referral to psychology for cognitive behavioral therapy-insomnia, and follow-up with neurology. PAP titration was recommended to evaluate for treatment emergent central sleep apnea. Conclusion This case demonstrates the complexity of managing an overlap of a progressive neurodegenerative condition, OSA, RLS, and chronic insomnia. A multidisciplinary and multi-system approach is important to address sleep and neuropsychological disorders in HD. Further research is needed to understand optimal management of sleep disorders, especially chorea during sleep in HD. Support (if any)