The origin of excessive daytime sleepiness in the Prader-Willi syndrome.
The polygraphically recorded sleep-wake continuum of 21 Prader-Willi syndrome (PWS) patients was compared with that of 19 normal people. In the Prader-Willi group, excessive daytime sleepiness (EDS) is found in 95% of subjects, and rapid eye movement (REM) sleep disorders occur in 52%. These two features were significantly different from the normal group of subjects. No indications were found for the presence of the apnoea syndrome. The REM sleep disorders are: sleep onset rapid eye movements (SOREM), REM sleep in naps, many arousals during REM sleep, and a significant decrease in total REM sleep. These disturbances in the Prader-Willi group, combined with the presence of EDS and sometimes of cataplexy, are likely to be expressions of a narcoleptic syndrome although this was not sustained by the HLA-DR2 expression above normal. The quality of life of PWS subjects can be improved in some cases by treating them as narcoleptic patients.
Helbing-Zwanenburg
B
B
Pameijer Foundation, Rotterdam, The Netherlands.
Kamphuisen
H A
HA
Mourtazaev
M S
MS
D000293:Adolescent; D000328:Adult; D002375:Catalepsy / Q000175:diagnosis / Q000188:drug therapy; D002997:Clomipramine / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007040:Hypoventilation / Q000175:diagnosis; D007099:Imipramine / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009290:Narcolepsy / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D017286:Polysomnography; D011218:Prader-Willi Syndrome / Q000150:complications*; D012891:Sleep Apnea Syndromes / Q000175:diagnosis; D012895:Sleep, REM / Q000502:physiology; D012978:Sodium Oxybate / Q000627:therapeutic use
D012978:Sodium Oxybate; D002997:Clomipramine; D007099:Imipramine