Table 2

Case–control polysomnography studies involving patients diagnosed with degenerative movement disorders

DisorderAuthorYearCohortAssessmentOutcome
HDWiegand et al 95 1991 HD (12)
HC (12)
Two nights of PSGPatients had an increased sleep onset latency, reduced sleep efficiency, increased no of awakenings and reduced slow wave sleep compared with controls.
HDWiegand et al 9 6 1991HD (16)
HC (16)
Two nights of PSGPatients with HD had reduced sleep efficiency, increased sleep-onset latency, reduced slow wave sleep and more time spent awake.
HDEmser et al 97 1988HD (10)
HC (12); seven volunteers
Two nights of PSGHD and HC had no differences except for two patients who had a reduction in slow wave sleep. Sleep spindle was also increased during N2 which correlated to duration of disease.
HDArnulf et al 59 2008HD (25), HC (25)
Narcolepsy (25)
Overnight PSG, MSLT, ESS, clinical interviewPatients with HD had insomnia, earlier sleep onset, lower sleep efficiency, increased PLMS and shortened REM sleep, ESS was normal. 12% exhibited RBD.
HDCuturic et al 98 2009HD (12)
HC (9)
One night of PSG, ESSESS score had no significant difference between groups. Sleep latency was significantly longer in patients. Nocturnal SRBD were absent.
HDGoodman et al 99 2011HD (9)
HC (10)
Three nights v-PSG, MSLT, ESS, Actigraphy, FOSQ, MOS, BDI-2FOSQ, ESS and MOS scores did not differ from controls. Sleep architecture (lower percentage of REM sleep) and sleep efficiency differed compared with controls.
HDNeutel et al 100 2015HD (29)
HC (29)
v-PSGPatients had longer total sleep and REM sleep onset latency. Two patients had RWA. Seven patients had giant sleep spindles and one control.
HDLazar et al 101 2015HD (38): premanifest gene carriers
HC (36)
Two nights PSG and MSLT, 2 weeks of ActigraphyGene carriers had more disrupted sleep, characterised by fragmented sleep profile: a decrease in REM sleep.
HDPiano et al 102 2015HD (30)
HC (30)
1 night v-PSG, ESS, IRLSSG, BQ, RBDSQTwo patients reported RLS, 8 had scores ≥9.8 patients had high risk of OSA. 2 had pathological RBD. PSG showed no RWA and RBD. Disease duration correlated with ESS.
SCASeshagiri et al 103 2018SCA1 (6) SCA2 (5)
SCA3 (7) HC (6)
Overnight PSGSleep spindle density significantly decreased in SCA.
SCARodríguez-Labrada et al 64 2019SCA2 (20)
SCA2 preclinical carriers (20),
HC (20)
One whole night of PSGCompared with controls sleep spindle density was significantly reduced in SCA2 and preclinical patients. Reduced spindle activity correlated with reduced N3 sleep in SCA2 patients.
SCARueda et al 66 2016SCA 6 (12)
HC (12)
Overnight PSGSCA6 had higher frequency of snoring, respiratory disorders and slow wave sleep compared with controls.
SCAZanatta et al 67 2019SCA 2 (17)
HC (17)
One night of v-PSGIncreased REM sleep in 70%, increased REM latency in 52%, increased OSA in 82% and absent REM density in 76%. Compared with controls SCA2 had reduced total sleep time, sleep efficiency, sleep latency, sleep N3 latency, REM quantity, N2 and N3 quantity. Disease progression correlated with a reduction in REM density and sleep efficiency
SCAIranzo et al 51 2003SCA3 (9)
HC (9)
Overnight PSG, sleep questionnaire55% of patients has RBD compared with no controls. SCA3 patients had reduced total sleep time, sleep efficiency, REM sleep percentage, increased WASO and PLMI.
SCAPedroso et al 65 2013SCA3 (22) from 15 families
HC (20)
Overnight PSG, RBDSQ, IRLSSGSCA3 patients; 54% had RLS, 77% had PLMS, 73% had RWA and 59% had RBD.
SCAVelázquez-Pérez et al 104 2011SCA2 (32)
HC (32)
Two nights of v-PSG, ESS, sleep interviewsReduced REM sleep percentage and REM density, an increase in RWA, PLMS in 37.5% of patients. 21.8% complained of insomnia vs 3% in HC and RLS diagnosed in 25% of patients compared with 3% in HC. No difference in ESS scores.
SCASilva et al 65 2016SCA3 (47)
HC (47)
Overnight PSG, IRLSSG, RBDSQ,SCA3 had higher frequency of arousals from slow wave sleep, parasomnia complaints, RWA, PLMI, percentage of N1 and N3 sleep.
SCAChi et al 105 2013SCA 3 (15)
HC (16)
Overnight PSG, ESSSCA3 patients had reduced sleep efficiency and percentage of REM sleep which negatively correlated with severity of ataxia. ESS was normal to controls.
SCAReimold et al 68 2006SCA1 (10), SCA2 (4)
SCA3 (2), HC (9)
Overnight PSG, ESSRLS present in 25% of SCA1 and SCA2 patients, 100% of SCA3 patients. All RLS patients had abnormal PLMS score. One RLS had OSA. 1 SCA2 and SCA3 patient had EDS.
SCALondon et al 67 2018SCA 10 (23)
HC (23)
One night of PSG, ESSSCA10 patients had longer REM sleep and more REM arousal than controls. REM sleep onset correlated with disease duration.
WDNetto et al 106 2010WD (25)
HC (25)
PSGPatients had reduced total sleep time, sleep efficiency, percentage of N3 sleep, and REM sleep, prolonged sleep-onset latency and latency to N2.
WDTrindade et al 72 2017WD (42)
HC (42)
v-PSG, IRLSSG, ESS, PSQI, BDISleep quality was worse compared with HC. WD patients showed lower sleep efficiency, less N2 sleep and more WASO and arousal compared with HC. WD with RLS showed significantly more PLM, more N1 sleep and a longer REM sleep latency.
WDTribl et al 70 2015WD (41)
HC (41)
v-PSG, RBDSQ, PSQI, ESS, MSQ, clinical and sleep interviews5 WD patients fulfilled the RBD criteria and had significantly higher values in RWA. RWA in WD patients without RBD was still significantly increased compared with controls.
WDNevsimalova et al 71 2011WD (55)
HC (55)
PSG (24 WD and HC), ESS, RBDSQ,13 WD patients fulfilled RLS criteria. WD patients were more prone to daytime napping, EDS and poor nocturnal sleep. Mean ESS and RBDSQ were higher than controls. TST was lower, with decreased sleep efficiency and increased wakefulness. WD had lower latency of N1 and N2 sleep. 14% had MSLT <8 min.
NPCVankova et al 74 2003Juvenile NPC (5)
HC (12)
At least one night of PSG, MSLTIn all patients, sleep was fragmented and disorganised. Total sleep time and sleep efficiency was lower, shorter sleep latency and increased WASO.
  • BDI/−2, Beck’s depression inventory; BQ, Berlin Questionnaire; EDS, excessive daytime sleepiness; ESS, Epworth Sleepiness Scale; FOSQ, Functional Outcomes of Sleep Questionnaire; HC, healthy control; HD, Huntington’s disease; HDSS, Hungtinton’s Disease Sleepiness Scale; IRLSSG, International Restless Leg Syndrome Study Group; MSLT, Multiple Sleep Latency Test; MSQ, Mayo Sleep Questionnaire; NPC, Niemann-Pick disease Type C; OSA, obstructive sleep apneoa; PLMI, Periodic Limb Movement Index; PLMS, periodic limb movement during Sleep; PSG, polysomnography; PSQI, Pittsburgh Sleep Quality Index; RBD, rapid eye movement sleep behaviour disorder; RBDSQ, Rapid Eye Movement sleep Behaviour Disorder Screening Questionnaire; REM, rapid eye movement sleep; RLS, Restless leg syndrome; RWA, rapid eye movement sleep without atonia; SCA, spinocerebellar ataxia; SRBD, sleep-related breathing disorder; v-PSG, video polysomnography; WASO, wake after sleep onset; WD, Wilson’s disease.