Polysomnographic findings and clinical correlates in Huntington disease: A cross-sectional cohort study

Carla Piano, Anna Losurdo, Giacomo Della Marca, Marcella Solito, Giovanna Calandra-Buonaura, Federica Provini, Anna Rita Bentivoglio, Pietro Cortelli

Research output: Contribution to journalArticlepeer-review

Abstract

Study Objectives: To evaluate the sleep pattern and the motor activity during sleep in a cohort of patients affected by Huntington disease (HD). Design: Cross-sectional cohort study. Setting: Sleep laboratory. Patients: Thirty HD patients, 16 women and 14 men (mean age 57.3 ± 12.2 y); 30 matched healthy controls (mean age 56.5 ± 11.8 y). Interventions: Subjective sleep evaluation: Epworth Sleepiness Scale (ESS); Berlin's Questionnaire, interview for restless legs syndrome (RLS), questionnaire for REM sleep behavior disorder (RBD). Clinical evaluation: disease duration, clinical severity (unified Huntington disease motor rating scale [UHDMRS]), genetic tests. Laboratory-based full-night attended video-polysomnography (V-PSG). Measurements and Results: The duration of the disease was 9.4 ± 4.4 y, UHMDRS score was 55.5 ± 23.4, CAG repeats were 44.3 ± 4.1. Body mass index was 21.9 ± 4.0 kg/m2. No patients or caregivers reported poor sleep quality. Two patients reported symptoms of RLS. Eight patients had an ESS score ≥ 9. Eight patients had high risk of obstructive sleep apnea. At the RBD questionnaire, two patients had a pathological score. HD patients, compared to controls, showed shorter sleep, reduced sleep efficiency index, and increased arousals and awakenings. Four patients presented with sleep disordered breathing (SDB). Periodic limb movements (PLMs) during wake and sleep were observed in all patients. No episode of RBD was observed in the V-PSG recordings, and no patients showed rapid eye movement (REM) sleep without atonia. The disease duration correlated with ESS score (P <0.02). UHMDRS correlated positively with the ESS score (P <0.005), and negatively with the percentage of REM sleep. Conclusions: Patients with Huntington disease showed a severe sleep disruption and a high prevalence of periodic limb movements, but no evidence of sleep disordered breathing or REM sleep behavior disorder.

Original languageEnglish
Pages (from-to)1489-1495
Number of pages7
JournalSleep
Volume38
Issue number9
DOIs
Publication statusPublished - Sep 1 2015

Keywords

  • Huntington disease
  • Periodic limb movements
  • Polysomnography
  • REM eye movement sleep behavior disorder
  • Sleep

ASJC Scopus subject areas

  • Physiology (medical)
  • Clinical Neurology

Fingerprint Dive into the research topics of 'Polysomnographic findings and clinical correlates in Huntington disease: A cross-sectional cohort study'. Together they form a unique fingerprint.

Cite this