Review Article

Sleep-Wake Cycle and Daytime Sleepiness in the Myotonic Dystrophies

Table 2

Sleep disorders and myotonic dystrophy type 2.

AuthorsSleep findingsNumber of patientsMethodology

Shepard et al., 2012 [17]EDS (6/8, 75%)
Insomnia (5/8, 62.5%) excessive fatigue (4/8 50%)
OSA (3/5 60%)
RLS (4/8, 50%)
8 pts (5/8 PSG) Retrospective uncontrolled PSG study in selected DM2 patients sleep complaints

Bhat et al., 2012 [18]EDS (6/6, 100%; 4/6 low MSL 66%, no SOREMPs)
Insomnia (2/6, 33.3%)
Snoring (4/6 66.6%)
OSA (4/6 66.6%)
REM without atonia with dream enacting behavior in OSAS after CPAP (1/6)* 
Paradoxical breathing in REM (2/6 33.3%)
RSWA (1/6, 16.6%)
Low sleep efficiency, alfa-delta sleep (2/6 33%)
6 pts (6/6 PSG)Prospective uncontrolled PSG study in selected DM2 patients sleep complaints

Chokroverty et al., 2012 [57] REM without atonia with dream enacting behavior in OSAS after CPAP*Single Case reportVideo-PSG

Tieleman et al., 2010 [16]EDS 6.9% (DM2) 44.8% (DM1) 6.2%
(Control)
Poor sleep quality 66% (DM2) 45% (DM1) 26% (Control)
29 DM2, 29 DM1, 65 ControlsProspective controlled study with subjective scales

Romigi et al., 2013 [75]Sleep disturbance pain-related (PSQI) (7/12 58%)
EDS (subjective scales 2/12, 66.6%; MSLT 4/12 33% no SOREMPs)
Snoring (2/12 16.6%)
Low sleep efficiency (PSG 100% DM2)
OSA (7/12 58%)
PLMS (PLMI > 15/h 3/12 25%)
RSWA with dream enacting behavior in severe OSAS (8.3% 1/12)
Paradoxical breathing in REM (1/12 8.3%)
RSWA (6/12, 50%)
12 DM2, 18 DM1, and 12 ControlsProspective controlled study with PSG and subjective scales

EDS: excessive daytime somnolence; OSA: obstructive sleep apnea; CSA: central sleep apnea; PLMS: periodic limb movement of sleep; PLMW: periodic limb movement of wake; SOREMPs: sleep onset REM periods; RSWA: REM sleep without atonia
*Probably the same patient.