Psychiatry and Clinical Neurosciences (2000), 54, 323–325

Hypersomnia
Relationship between hypersomnia and respiratory
disorder during sleep in Prader–Willi syndrome
YU HIROE, md, YUICHI INOUE, md, SHIGERU HIGAMI, md, YUJI SUTO, md
AND RYUZO KAWAHARA, md
Department of Neuropsychiatry, Department of Otorhinolaryngology, Department of Radiology, Faculty of
Medicine,Tottori University, Japan

Abstract To assess whether hypersomnia in Prader–Willi syndrome (PWS) patients is related to the respi-
ratory disorder during sleep (RDDS), we made a systematic evaluation regarding the relation-
ship between the two disorders in three patients. All patients showed hypersomnia manifested
as the long duration of night sleep and shortened sleep latencies of multiple sleep latency test.
Although magnetic resonance imaging and laboratory studies revealed obstruction of the upper
airway and mild increase of esophageal pressure during sleep, the number of other apneic
episodes or awakenings was not as frequent. From the above results, we speculate that the mech-
anism of excessive daytime sleepiness in PWS is not caused by RDDS and quite resembles that
of essential hypersomnia.

Key words esophageal pressure, excessive daytime sleepiness, Prader–Willi syndrome, respiratory disorder
during sleep, upper airway magnetic resonance imaging.

INTRODUCTION 3, a female 24 year old, BMI 78.2) who were geneti-

cally proved to have the presence of a small deletion
Prader–Willi syndrome (PWS), due to the deletion of
within the long arm of chromosome 15. From
the proximal part of long arm of chromosome 15, is
enquiries with their family members, it was apparent
characterized by muscle hypotonia, obesity, hypogo-
that all three patients have been markedly obese and
nadism and mental retardation.1 Moreover, PWS has
showed daytime sleepiness since early childhood. On
been known to frequently accompany both excessive
the above subjects, we carried out polysomnographic
daytime sleepiness and respiratory disorder during
recordings and multiple sleep latency test (MSLT) as
sleep (RDDS).2 However, causative relation between
well as both measurements of esophageal pressure
these two sleep disorders is not well investigated.
and ultrafast MRI3 during daytime nap. Regarding
In order to clarify the above issue, we evaluated
esophageal pressure, the difference between maximal
the characteristics of sleep structure on nocturnal
and minimal negative value during the recording
polysomnogram and correlated them to RDDS vari-
(delta Pes)4 was employed as an index. Moreover, in
ables and the level of daytime sleepiness in PWS
one case who underwent uvulopalatopharyngoplasty
patients.
(UPPP), comparison of the above variables were
made between before and 2 years after the surgery.
SUBJECTS
Our study comprised three PWS patients (case 1, RESULTS
a female 15 year old, body mass index (BMI)
The nocturnal polysomnograms showed the value
35.7 kg/m2; case 2, a male 17 year old, BMI 35.7; case
of total sleep time more than 9 h. No remarkable
abnormalities of sleep stage distributions were
found in all subjects (Table 1). With respect to RDDS
Correspondence address: Yu Hiroe, Department of Neuropsy- variables, paradoxical thoracoabdominal movements
chiatry, Faculty of Medicine, Tottori University, 36-1 Nishimachi, indicating the narrowing of the upper airway were
Yonago 683-8504, Japan. Email: TQJ00444@nifty.ne.jp recognized all through the nights, and the fall of
324 Y. Hiroe et al.

Table 1. Sleep architecture of subject cases

Case 1
Before UPPP After UPPP Case 2 Case 3

Total sleep time (min) 492 507 493 579

Sleep efficiency (%) 85.7 97.7 89.8 87.7
Stage 1 (%) 44.1 18.5 22.4 36.2
Stage 2 (%) 28.1 29.5 27.5 19.2
Stage 3 + 4 (%) 4.7 29.9 20.2 3.5
Stage REM (%) 14.8 19.8 19.7 28.2
Number in appearance of Stage REM 3 4 4 4
Stage awake (%) 8.3 2.3 10.2 12.3
Number of awakenings 82 46 60 55
Awakenings index (/h) 8.8 5.3 6.6 5
Apnea index (/h) 5.5 0.4 2.8 1
Mean duration of apnea (s) 21.2 21.3 13.6 13.8
Hypopnea index (/h) 1.3 0.8 0.5 2.8
Mean duration of hypopnea (s) 17.8 13.6 12.5 12.7
Lowest value of SaO2 (%) 72 88 79 72
Duration of periods in which
SaO2 fell 90% or below (min) 63 0 52 80

UPPP, uvulopalatopharyngoplasty.

oxygen saturation to the value of 80% or less was
frequently observed in all three cases. However, only
case 1 fulfilled the criteria of sleep apnea syndrome
(SAS) with mild degree, and the other two cases did
not fulfil the criteria of SAS nor upper airway resis-
tance syndrome (UARS) due to the small numbers of
both apneic episodes and awakenings (Table 1). On
upper airway MRI during daytime nap, the narrowing
of velopharynx was recognized in the subject cases.
However, values of delta Pes showed only a small
increase (case 1, –17 cmH2O; case2: –10 cmH2O; case
3: –12 cmH2O). Concerning MSLT, the values of sleep
latency in all subjects were abnormally short (case 1,
4.9 min; case 2, 6.4 min; case 3, 4.8 min). In case 1, who
underwent UPPP, MSLT did not show the prolonga-
tion of average sleep latency 2 years after the surgery
despite the remarkable reduction in the number of
apneic episodes and the increase in the percentage
of stages 3 and 4 on nocturnal polysomnograms as
well as the decrease of oxygen desaturation while
asleep.
DISCUSSION
In our results, it was confirmed that definite hyper-
somnia exists in patients with PWS as previously
reported.2,5 Concerning RDDS measures, apneic
events while asleep were few despite severe obesity,
and only one case fulfilled the criteria of SAS. This
finding is consistent with several previous reports.5,6
However, mild increase of delta pes, velopharyngeal
narrowing and frequent falls of SaO2 were recognized
during sleep in PWS cases. These findings indicate the
increase of upper airway resistance in PWS patients.
However, arousal index did not fulfil the criteria of
UARS. Moreover, case 1 who underwent UPPP did
not show the decrease in excessive daytime sleepiness
despite both the improvement of RDDS and sleep
architecture. Accordingly, we can conclude that exces-
sive daytime sleepiness in PWS did not relate to
RDDS as previously reported by Harris and Allen,7
and the mechanism of excessive daytime sleepiness
in the disorder quite resembles that of essential
hypersomnia.
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