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F47 Sleep quality and related clinical manifestations in Huntington disease
  1. Sabrina Maffi1,
  2. Eugenia Scaricamazza1,
  3. Simone Migliore1,
  4. Melissa Casella2,
  5. Consuelo Ceccarelli2,
  6. Ferdinando Squitieri1,2
  1. 1IRCCS Casa Sollievo della Sofferenza Research Hospital, San Giovanni Rotondo, Italy
  2. 2LIRH Foundation, Rome, Italy


Background Sleep patterns are frequently disrupted in neurodegenerative disorders such as Huntington Disease (HD), yet in the latter they are still poorly understood, especially their association with clinic features.

Aim Our study aimed to explore potential relationships between sleep features and motor, cognitive, behavioural and functional changes in manifest HD subjects.

Methods We enrolled 42 patients who were assessed by Pittsburgh Sleep Quality Index (PSQI) and Insomnia Severity Index (ISI) questionnaires; clinical features were evaluated by the validated ENROLL-HD platform assay including the Unified Huntington’s Disease Rating Scale (UHDRS) and the Problem Behaviours Assessment Short Form (PBA-s).

Results We found a significant association between the patients’ perception of sleep abnormalities and scores of impaired independence, cognitive and motor performances. We found a strong association between depression, both self-reported by the patients and reported by the raters, and several sleep scores, i.e. including the sleep disturbance (PSQI – C5 subscore). Our analysis showed that the score of self-reported depression also correlates with insomnia (ISI scores), poor quality of sleep (PSQI – C1 subscore) and impact on daily life activities (PSQI – C7 subscore). Specifically, the sleep efficiency (PSQI - C4 subscores) and the use of sleep medications (PSQI - C6 subscores) seem to be more frequently associated with the severity of the disease progression.

Conclusion Sleep abnormalities represent an important part of the HD clinical profile and can impair patients’ quality of life by affecting their level of independence, cognition performance and mental well-being.

  • Sleep in Huntington Disease
  • PSQI
  • ISI
  • Depression
  • Cognitive Functions

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