title: Polysomnographic Findings and Clinical Correlates in Huntington Disease: A Cross-Sectional Cohort Study.
abstract: OBJECTIVE
  To evaluate the sleep pattern and the motor activity during sleep in a cohort of patients affected by Huntington disease (HD).
  
  
  METHODS
  Cross-sectional cohort study.
  
  
  METHODS
  Sleep laboratory.
  
  
  METHODS
  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).
  
  
  METHODS
  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).
  
  
  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/m(2). 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.
authors.affiliation: Center for Parkinson Disease and Extrapyramidal Disorders, Movement Disorders Unit, Institute of Neurology, Catholic University, Rome, Italy.|IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy; Department of Biomedical and NeuroMotor Sciences (DIBINEM), Alma Mater Studiorum, University of Bologna, Bologna, Italy.
authors.forename: Carla
authors.initials: C
authors.lastname: Piano
authors.affiliation: Sleep Disorders Unit, Institute of Neurology, Rome, Italy.
authors.forename: Anna
authors.initials: A
authors.lastname: Losurdo
authors.affiliation: Sleep Disorders Unit, Institute of Neurology, Rome, Italy.
authors.forename: Giacomo
authors.initials: G
authors.lastname: Della Marca
authors.affiliation: Center for Parkinson Disease and Extrapyramidal Disorders, Movement Disorders Unit, Institute of Neurology, Catholic University, Rome, Italy.
authors.forename: Marcella
authors.initials: M
authors.lastname: Solito
authors.affiliation: IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy; Department of Biomedical and NeuroMotor Sciences (DIBINEM), Alma Mater Studiorum, University of Bologna, Bologna, Italy.
authors.forename: Giovanna
authors.initials: G
authors.lastname: Calandra-Buonaura
authors.affiliation: IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy; Department of Biomedical and NeuroMotor Sciences (DIBINEM), Alma Mater Studiorum, University of Bologna, Bologna, Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Provini
authors.affiliation: Center for Parkinson Disease and Extrapyramidal Disorders, Movement Disorders Unit, Institute of Neurology, Catholic University, Rome, Italy.
authors.forename: Anna Rita
authors.initials: AR
authors.lastname: Bentivoglio
authors.affiliation: IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy; Department of Biomedical and NeuroMotor Sciences (DIBINEM), Alma Mater Studiorum, University of Bologna, Bologna, Italy.
authors.forename: Pietro
authors.initials: P
authors.lastname: Cortelli
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001143:Arousal; D015331:Cohort Studies; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000150:complications* / Q000235:genetics / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D009068:Movement; D017286:Polysomnography; D020187:REM Sleep Behavior Disorder / Q000175:diagnosis / Q000503:physiopathology; D012148:Restless Legs Syndrome / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology; D012890:Sleep / Q000502:physiology*; D012891:Sleep Apnea Syndromes / Q000175:diagnosis / Q000503:physiopathology; D020181:Sleep Apnea, Obstructive / Q000175:diagnosis; D012893:Sleep Wake Disorders / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D012895:Sleep, REM; D014741:Video Recording; D014851:Wakefulness
keywords: Huntington disease; REM eye movement sleep behavior disorder; periodic limb movements; polysomnography; sleep
references.citation: Sleep Med. 2003 Mar;4(2):101-19
references.pmid: 14592341
references.citation: Sleep. 2013 Aug;36(8):1147-52
references.pmid: 23904674
references.citation: Neuropsychologia. 1974 Oct;12(4):487-96
references.pmid: 4437744
references.citation: Exp Neurol. 1976 Oct;53(1):31-50
references.pmid: 183969
references.citation: Exp Brain Res. 1986;62(2):363-72
references.pmid: 3709719
references.citation: J Neurol. 1988 Jan;235(3):177-9
references.pmid: 2966851
references.citation: Eur Arch Psychiatry Clin Neurosci. 1991;240(3):148-52
references.pmid: 1827599
references.citation: J Neurol. 1991 Jul;238(4):203-8
references.pmid: 1832711
references.citation: Hum Genet. 1992 Jun;89(4):365-76
references.pmid: 1535611
references.citation: Cell. 1993 Mar 26;72(6):971-83
references.pmid: 8458085
references.citation: N Engl J Med. 1993 Apr 29;328(17):1230-5
references.pmid: 8464434
references.citation: Br J Psychiatry. 1997 Oct;171:393
references.pmid: 9373439
references.citation: J Neurol Neurosurg Psychiatry. 1999 Jan;66(1):52-6
references.pmid: 9886451
references.citation: Ann Intern Med. 1999 Oct 5;131(7):485-91
references.pmid: 10507956
references.citation: J Neurosci. 2005 Jan 5;25(1):157-63
references.pmid: 15634777
references.citation: Mov Disord. 2005 Mar;20(3):293-7
references.pmid: 15584032
references.citation: J Neurol. 2005 Jun;252(6):642-7
references.pmid: 15742112
references.citation: Sleep Med. 2006 Mar;7(2):175-83
references.pmid: 16459136
references.citation: Sleep. 2006 Jun;29(6):759-69
references.pmid: 16796214
references.citation: Semin Neurol. 2007 Apr;27(2):143-50
references.pmid: 17390259
references.citation: J Clin Sleep Med. 2007 Mar 15;3(2):121-31
references.pmid: 17557422
references.citation: Dialogues Clin Neurosci. 2008;10(4):473-81
references.pmid: 19170404
references.citation: Arch Neurol. 2008 Apr;65(4):482-8
references.pmid: 18413470
references.citation: J Neurosci. 2007 Jul 18;27(29):7869-78
references.pmid: 17634381
references.citation: Brain Res. 2009 Jul 7;1279:90-8
references.pmid: 19450569
references.citation: Parkinsonism Relat Disord. 2009 Jul;15(6):471-4
references.pmid: 19041273
references.citation: Behav Sleep Med. 2009;7(4):245-54
references.pmid: 19787493
references.citation: Curr Neurol Neurosci Rep. 2011 Apr;11(2):211-7
references.pmid: 21103960
references.citation: Brain. 2014 Feb;137(Pt 2):610-20
references.pmid: 24285642
references.citation: Mov Disord. 2007 Dec;22(16):2386-93
references.pmid: 17894337
references.citation: Neurol Sci. 2003 Feb;23(6):295-300
references.pmid: 12624716
references.citation: Mov Disord. 2011 Apr;26(5):877-84
references.pmid: 21370269
references.citation: Clin Neurol Neurosurg. 2004 Sep;106(4):318-29
references.pmid: 15297008