Objective: Characterize sleep architecture and cognitive performance in people with early Huntington’s disease (HD) and explore their associations.

Background: Cognitive impairment is a devastating feature of HD, which can start prior to motor symptoms and worsens with disease progression.[1] Sleep dysfunction occurs in up to 88% of individuals and appears prior to motor symptoms.[2] Slow wave sleep (SWS) is critical for memory and has been associated with better cognitive performance in other neurodegenerative diseases.[3-5] Limited studies suggest that individuals with HD experience alternation in sleep architecture using the gold standard polysomnography (PSG) [6], however these studies often do not focus on the early stage of disease when potential interventions may have the greatest impact. Given the importance of sleep in cognition, evaluation of sleep architecture and its relationship to cognitive performance in early HD is needed.

Method: PSG and cognitive assessments were performed on four Stage 2 and one early Stage 3 participants, >18 years of age. Cognitive performance was measured using the Montreal Cognitive Assessment (MoCA), the Verbal Fluency Test (VF), the Symbol Digit Modalities Test (SDMT), and the Stroop Test. Descriptive analyses were performed, and Spearman rank correlations were assessed for associations.

Results: Five individuals with early HD were included in this exploratory analysis. Mean age was 43.8 years, mean time since diagnosis 13.5 months, mean CAG repeats 41.6 (+/- 1.3, range 40-43), with one female and four males. Mean MoCA score: 26.4 (+/- 2.8, range 23-29), Verbal fluency: 36 (+/- 8.6, range 8-41), and SDMT: 34.4 (+/- 9.2, range 23-46). Mean Epworth Sleepiness Scale was 7.4 (+/- 4.2, range 4-14). Please see Table 1 for descriptions of sleep architecture. No clinically significant associations were found between measures of cognitive performance and sleep architecture (Table 2). There was a trend toward significance, indicating a possible association of limb movements with cognitive performance on some tests, but this did not meet the significance threshold.

Conclusion: This pilot study describes sleep architecture and cognition in individuals with early HD. Understanding sleep patterns and their association with cognitive performance in early HD is a critical area of importance, however a larger cohort with longitudinal data is required to clearly characterize associations.

Table 2

Table 1

References: 1. Paulsen JS, Miller AC, Hayes T, Shaw E. Chapter 6 – Cognitive and behavioral changes in Huntington disease before diagnosis. In: Feigin AS, Anderson KE, eds. Handbook of Clinical Neurology. Vol 144. Huntington Disease. Elsevier; 2017:69-91. doi:10.1016/B978-0-12-801893-4.00006-7
2. Taylor N, Bramble D. Sleep disturbance and Huntingdon’s disease. Br J Psychiatry. 1997;171(4):393-393. doi:10.1192/bjp.171.4.393c
3. Spira AP, Chen-Edinboro LP, Wu MN, Yaffe K. Impact of Sleep on the Risk of Cognitive Decline and Dementia. Curr Opin Psychiatry. 2014;27(6):478-483. doi:10.1097/YCO.0000000000000106
4. Lee YF, Gerashchenko D, Timofeev I, Bacskai BJ, Kastanenka KV. Slow Wave Sleep Is a Promising Intervention Target for Alzheimer’s Disease. Front Neurosci. 2020;14:705. doi:10.3389/fnins.2020.00705
5. Wood KH, Memon AA, Memon RA, et al. Slow Wave Sleep and EEG Delta Spectral Power are Associated with Cognitive Function in Parkinson’s Disease. J Park Dis. 2021;11(2):703-714. doi:10.3233/JPD-202215
6. Zhang Y, Ren R, Yang L, Zhou J, Li Y, Shi J, Lu L, Sanford LD, Tang X. Sleep in Huntington’s disease: a systematic review and meta-analysis of polysomongraphic findings. Sleep. 2019 Oct 9;42(10):zsz154. doi: 10.1093/sleep/zsz154. PMID: 31328779; PMCID: PMC6783889.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/sleep-architecture-and-cognitive-performance-in-early-huntingtons-disease-a-pilot-study/