J. Sleep Res.

(2010) 19, 525–534 Hypersomnia

doi: 10.1111/j.1365-2869.2010.00824.x

Subjective symptoms in idiopathic hypersomnia: beyond

excessive sleepiness
CYRILLE VERNET, SMARANDA LEU-SEMENESCU, MARIE-ANNICK
B U Z A R E and I S A B E L L E A R N U L F
Sleep Disorders Unit, National Reference Center for Narcolepsy and Hypersomnia, Pitié-Salpêtrière Hospital, Inserm UMRS_975, Paris 6
University, France

Accepted in revised form 23 November 2009; received 09 June 2009

SUMMARY Patients with idiopathic hypersomnia never feel fully alert despite a normal or long
sleep night. The spectrum of the symptoms is insufficiently studied. We interviewed 62
consecutive patients with idiopathic hypersomnia (with a mean sleep latency lower than
8 min or a sleep time longer than 11 h) and 50 healthy controls using a questionnaire on
sleep, awakening, sleepiness, alertness and cognitive, psychological and functional
problems during daily life conditions. Patients slept 3 h more on weekends, holidays
and in the sleep unit than on working days. In the morning, the patients needed
somebody to wake them, or to be stressed, while routine, light, alarm clocks and
motivation were inefficient. Three-quarters of the patients did not feel refreshed after
short naps. During the daytime, their alertness was modulated by the same external
conditions as controls, but they felt more sedated in darkness, in a quiet environment,
when listening to music or conversation. Being hyperactive helped them more than
controls to resist sleepiness. They were more frequently evening-type and more alert in
the evening than in the morning. The patients were able to focus only for 1 h (versus 4 h
in the controls). They complained of attention and memory deficit. Half of them had
problems regulating their body temperature and were near-sighted. Mental fatigability,
dependence on other people for awakening them, and a reduced benefit from usually
alerting conditions (except being hyperactive or stressed) seem to be more specific of the
daily problems of patients with idiopathic hypersomnia than daytime sleepiness.
k e y w o r d s fatigue, hypersomnia, long sleep time, mental fatigability, sleep drunk-
enness, sleepiness, tiredness

cases with various clinical forms. Alertness is continuously

INTRODUCTION
Idiopathic hypersomnia is characterized by chronic, daily
excessive daytime sleepiness despite normal sleep. The diag-
nosis is difficult, as more common causes of sleepiness,
including sleep deprivation, the use of drugs or substances,
psychiatric or medical disorders and genuine sleep disorders
(sleep disordered breathing, narcolepsy and periodic leg
movement disorders) had to be first ruled out. Since it has
been distinguished from narcolepsy (Roth, 1957), idiopathic
hypersomnia has been defined according to small series of
Correspondence: Cyrille Vernet, MSc, Unité des Pathologies du
Sommeil, Hôpital Pitié-Salpêtrière, 47-83 Boulevard de lÕHôpital,
75651 Paris Cedex 13, France. Tel.: 142 16 7717; fax: 142 16 77 00;
e-mail: cyrille@hypersomnies.eu
decreased during the daytime, possibly culminating in the
irresistible need for sleeping. Naps are either refreshing or,
more typically, long and unrefreshing (American Academy of
Sleep Medicine, 2005). Patients may also report a prolonged
difficulty waking up with automatic behaviour, confusion and
repeated returns to sleep, a symptom named Ôsleep drunken-
nessÕ. Several clinical forms have been described in the past,
including patients with sleepiness alone (monosymptomatic)
and patients with a prolonged night of sleep and sleep
drunkenness upon awaking (polysymptomatic; Roth, 1981).
Idiopathic hypersomnia is now divided into hypersomnia with
and without a long (>10 h) sleep time (American Academy of
Sleep Medicine., 2005). Hypersomnia is idiopathic when the
symptoms and polygraphic findings cannot be better explained

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526 C. Vernet et al.
by medical or psychiatric (mostly depression) disorders. Based
on these evolving definitions, several series of patients have
been published (Aldrich, 1996; Anderson et al., 2007; Baker
et al., 1986; Billiard et al., 1998; Bove et al., 1994; Bruck and
Parkes, 1996; Coleman et al., 1982; Komada et al., 2005; Roth
et al., 1972; Sforza et al., 2000; Vernet and Arnulf, 2009;
Vgontzas et al., 2000); they contain 10–77 patients. These
series suggest that idiopathic hypersomnia is a rare disease,
representing 8 : 10 to 1 : 10 patients with narcolepsy, with a
prevalence of approximately 0.005% (Billiard and Dauvilliers,
2001). Most patients are young at disease onset. This is usually
a life-long disease, although recent series suggest that hyper-
somnia may spontaneously disappear in 14–25% of patients
(Anderson et al., 2007; Billiard, 1996; Bruck and Parkes, 1996).
These limited case series mainly evaluate the night-time sleep
and the narcolepsy-associated features (sleepiness, hallucina-
tions and sleep paralysis) but, more rarely, some problems
associated with the autonomic nervous system, such as
Raynaud-like syndrome, migraine and orthostatic syncope
(Bassetti and Aldrich, 1997; Matsunaga, 1987; Roth, 1981;
Roth et al., 1972). They frequently lack healthy controls, as
idiopathic hypersomnia is usually compared with narcolepsy.
The spectrum of problems linked to idiopathic hypersomnia,
however, is much larger in clinical practice and daily life. For
example, there are a lack of data on how long patients with
hypersomnia usually sleep when working (versus during the
weekend or on holidays), what is their most efficacious means
to wake up, how their alertness changes with environmental
(types of light, sounds and company) factors, how long they
can focus, and the numerous daytime problems they can
experience. In our experience, many patients with idiopathic
hypersomnia complain of fatigue rather than of sleepiness, and
have difficulties completing the classical Epworth sleepiness
score, suggesting that their complaint is not exactly (or simply)
sleepiness. We took the opportunity of a large prospective
cohort of patients with idiopathic hypersomnia studied over
4 years and systematically interviewed the patients on their
subjective symptoms using a formal questionnaire.
MATERIALS AND METHODS
Subjects
Between 2005 and 2008, all patients being monitored for 48 h
on the suspicion of idiopathic hypersomnia completed a face-
to-face interview and a standardized questionnaire. The
patients who completed the whole questionnaire and later
received a final diagnosis of idiopathic hypersomnia by an
experienced sleep neurologist were included in this study. The
patients with idiopathic hypersomnia met the following
inclusion criteria: (1) complaints of excessive daytime sleepi-
ness occurring daily for at least 3 months; (2) no improvement
of sleepiness with an increase of the night-time length for
15 days (excluding the diagnosis of behaviourally induced
insufficient sleep syndrome); (3) a mean sleep latency (MSL)
during multiple sleep latency tests (MSLT) lower than 8 min;
or (4) a total sleep time >11 h on long-term (24 h) sleep
monitoring following a habituation night (Vernet and Arnulf,
2009). We excluded the patients with: (1) sleep-disordered
breathing, defined by a respiratory disturbance index greater
than 10 events h)1 (this index included apnoea, hypopnoea
and respiratory effort-related arousal events, with the flow
limitation being measured with a nasal cannula); (2) narco-
lepsy defined as the presence of a definite cataplexy or MSL
lower than 8 min and multiple sleep-onset rapid eye movement
(REM) periods during the MSLT; (3) hypersomnia because of
a medical or psychiatric condition (e.g. ParkinsonÕs disease,
hypothyroidism, genetic disease or depression); (4) hypersom-
nia because of a drug or substance and (5) circadian sleep
disorders. In addition, the patients with a REM sleep latency
lower than 20 min during the night-time or during the daytime
monitoring (at more than one nap) were excluded, as they may
be a form of atypical narcolepsy. Thus, 62 patients (43 women,
of whom nine were postmenopausal and 19 men) were
included in the study.
Fifty paid subjects volunteered to take part as controls after
recruitment by advertisement and selection by the physician.
They were matched by age and sex with the patients. The
subjects were selected after a medical interview and had no
complaints regarding their sleep, no excessive daytime sleep-
iness (defined as the absence of spontaneous or elicited
complaint, and also having a score on the Epworth sleepiness
scale lower than 10), no chronic sleep deprivation (determined
using a questionnaire on sleep habits), no shift or night work,
no severe medical illness and no use of medications known to
modify sleep and wakefulness. Thus, 50 healthy subjects (27
women, of whom 6 were postmenopausal and 23 men)
completed the study. In addition, 30 of these 50 controls
completed a 48-h sleep monitoring. All subjects signed an
informed consent to take part. The local ethics committee
accepted the protocol.
Investigations
A large, systematic questionnaire was developed after inter-
viewing sleep neurologists experienced in treating narcolepsy
and hypersomnia (I. Arnulf., S. Leu, M. A. Buzare and M.
Minz), and prospectively interviewing a panel of 30 patients
with idiopathic hypersomnia already diagnosed in the sleep
disorders unit. New participants underwent a face-to-face
interview about classical sleep symptoms (cataplexy, sleep
drunkenness, sleep paralysis and restorative naps) and com-
pleted the sleep questionnaire. This auto-questionnaire con-
tained five sections (Appendix S1). The sleep section included
questions on the sleep duration at the time of the study and
when the subject was 10-years old (as we wanted to determine
if sleep excess was permanent or developed with time), the
usual sleep times of the parents, the self-estimated sleep time
need and naps (refreshing type, duration and frequency). In
the section on morning and post-nap awakening, the subjects
evaluated their difficulties on waking up and looked at the
effects on awakening of a series of stimuli (e.g. alarm clock,
 2010 European Sleep Research Society, J. Sleep Res., 19, 525–534
 Subjective symptoms in idiopathic hypersomnia 527

light) or events (e.g. scheduled appointment). The section on
circadian variation of daytime sleepiness and fatigue across the
daytime included the morningness–eveningness questionnaire
(Horne and Ostberg, 1976), and questions such as Ôwhat is your
level of alertness?Õ for five times of the day, or Ôat what time of
the day are you the most tired and the most awake?Õ. The
section on sleepiness and alertness contained the Epworth
sleepiness scale (Johns, 1991), the Pichot fatigue scale (Pichot
and Brun, 1984) and the Fatigue Severity Score (Krupp et al.,
1989) and evaluated the fluctuation of sleepiness and alertness
in the presence of environmental conditions or stimuli (light,
sounds) or internal stimuli (sports, motivation). The section on
non-sleep problems included memory, automatic behaviours,
ability to focus, the hospital anxiety and depression (HAD)
rating scale (Zigmond and Snaith, 1983) and somatic com-
plaints. Each section contained mostly multiple-choice ques-
tions and some open, more descriptive questions, in order to
catch more adequately the exact complaints of the patients.
The sleep- and wake-monitoring procedures included: (1) a
habituation night with sleep and respiratory monitoring from
11:00 to 06:30 hours; followed the next day by (2) five standard
MSLT at 08:00, 10:00, 12:00, 14:00 and 16:00 hours that were
terminated after 20 min if no sleep occurred and after 15 min
asleep if sleep occurred (Carskadon et al., 1986); and followed
the next evening by (3) a long-term (24 h) sleep monitoring. All
of the patients were monitored, while 30 ⁄ 50 (60%) of the
healthy controls underwent the full 48-h sleep-monitoring
procedure.
Statistical analyses
After having checked the Gaussian distribution of the
variables, we analysed the between-groups dichotomous vari-
ables using a chi-square test and analysed our continuous
variables using an analysis of variance (Statistica 7.1, Stat Soft,
Tulsa, OK, USA). A P-value less than 0.01 was considered to
be significant (with corrections for repeated measures). The
values are presented as mean ± SD (unless otherwise speci-
fied).
RESULTS
There were 62 patients with idiopathic hypersomnia and 50
controls. The sleep measures in both groups are displayed in
Table S1. Among the patients with idiopathic hypersomnia, 25
(40%) had a long night-time sleep time (>10 h) and 37 (60%)
had a normal night-time sleep time. As many patients as
controls regularly practice a sport. The 30 controls who
underwent the sleep monitoring were not different with respect
to demography and answers to the sleep questionnaires from
the 20 controls who did not (data not shown). The disease
occurred when 21.2 ± 13.0 years old. Before the disease onset,
23% of the patients had insomnia and 38% had a major
change in sleep habits. Half of the patients reported an
important personal event before the disease onset (death of a
loved one, divorce, pneumopathy, end of military service or
end of a high level of sport practice).
Usual sleep duration during the night and during naps
Patients and controls rarely had one parent (at least) who
sleeps more than 9.5 h (12.5% versus 5.1%, P = 0.40). Their
usual sleep times as a child, and during work days, weekends
and holidays, are indicated in Fig. 1. The patients and
controls slept the same amount of time when they were
10-years old. While the patients slept as long as controls
during the working days, they slept much longer during the
weekend and holidays. As a result, they extended their usual

Usual sleep time when 10

years old

Self estimated sleep time

need

Usual sleep time during

working days

Usual sleep time during the

weekend

Usual sleep time during

holidays

Maximum time asleep per

night

Total sleep time during the

long term sleep monitoring

Figure 1. Sleep time in patients with idio-

0:00 2:00 4:00 6:00 8:00 10:00 12:00 14:00
pathic hypersomnia (dark grey columns) and
Sleep duration (h)
in healthy controls (light grey columns) in
*
various conditions. P < 0.01 for a difference between the patients and the controls.

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528 C. Vernet et al.

sleep by +3.00 ± 2.21 h on the weekend and holidays, for breakfast, versus 78% of the controls (P = 0.03). As many
versus +1.12 ± 1.22 h in the controls (P < 0.001). Their as 78% of patients had difficulties waking up in the morning
maximum time asleep per night (range 10–20 h of sleep in and could not sometimes hear the alarm clock, a much larger
patients versus 10–13 h in controls, P < 0.001) and their self- percentage than in the controls. Difficulties with waking up
estimated necessary sleep time were also longer than in the were associated with more frequent automatic behaviours
controls. The time asleep during holidays was greater than (P = 0.006). To specifically study the sleep drunkenness, we
the sleep needs in both the patients (P = 0.009) and the isolated the patients with frank, genuine sleep drunkenness (i.e.
controls (P = 0.002). Almost all (90%) of the patients need three alarm clocks to wake up, need to put the alarm
reported to sometimes sleep longer than 10 h consecutively, clock on a stack of plates or need to be pulled out of the bed by
versus 27% of controls. The patients slept more than 10 h parents and remaining sleepiness for at least 1 h and having
consecutively for a mean of 7.4 ± 7.2 nights ⁄ month versus this symptom almost every day). There were 18 patients in this
0.9 ± 1.9 nights ⁄ months in the controls (P < 0.0001). The case. We contrasted them with the other extreme of the group,
total sleep time measured in the sleep lab during the i.e. the 32 patients with an easy time waking up (Table S2). We
continuous 24-h-long monitoring was similar in both groups removed from the analysis the patients Ôin-betweenÕ, i.e. with
to the usual sleep time declared during the weekend or the unclear difficulties in waking up (i.e. mild sleep inertia, able to
holidays (P < 0.001). As for naps, their frequency (2.7 ± 2.6 wake up, but mildly groggy for 1 h).
per week in patients versus 1.5 ± 1.9 per week in controls, The 18 patients with genuine sleep drunkenness had a total
P = 0.01), average duration (1.21 ± 0.51 h in patients sleep time similar to the 32 patients with no sleep drunken-
versus 0.50 ± 0.49 h in controls, P = 0.018) and maximum ness at all, a similar sleep efficacy and fragmentation. They
duration (2.47 ± 1.45 h in patients versus 1.11 ± 0.50 h in had a period of slow-wave sleep after 06:00 hours as often as
controls, P < 0.001) were higher in patients than in controls. the others. In contrast, the patients with sleep drunkenness
tended to be more frequently of the evening type, as indicated
by a lower Horne–Ostberg score (41.0 ± 12.6 versus
Waking up
52.6 ± 12.3, P = 0.02). The other scores or measures were
In the hypersomnia group, there were less patients feeling not different.
refreshed after a usual night than in the control group For the question ÔWhat helps you with waking up?Õ, the
(Table 1). The patients felt as hungry on awakening as presence of a human (not an animal) helping the subject to
controls, as 58% of the patients felt a normal or great hunger wake up and the stress of being late were more helpful for
obtaining a full awakening in the patients than in the controls
(Fig. 2). The alarm clock, motivation, sounds, a new activity
and hunger or thirst were chosen as often in the two groups,
Table 1 Demographical and clinical characteristics of the patients but a bright light and habit helped less frequently in the
with idiopathic hypersomnia and the healthy controls patients than the controls. Some patients changed the tone and
Idiopathic Healthy music of their alarm every other day, so that they would not
Patients hypersomnia controls get used to it, or tried an alarm clock set for a hearing-
impaired person, with a vibrating pillow, or coupled their
Number 62 50
Age, years 34.9 ± 13.2 38.3 ± 16.9
alarm clock to a distributor that vapourized cold water onto
Body mass index, kg m)2 24.5 ± 4.9 23.9 ± 4.4 the face, but none of these helped. A patient used his stereo (set
Women, % 69 54 at a very high volume) as an alarm, which was located in a far
Refreshing night, % 19* 89 corner of the room, combined with obstacles on the floor, so
Difficulties with morning 78* 26 that he would need to stand up, walk, avoid the obstacles and
awakening, %
Sleep drunkenness, % 36* 0
then shut-off the sound. He would manage to wake up with
Do not hear the alarm clock, % 78* 32 this system.
Normal or great hunger 58 78 An almost similar pattern was found in patients after
for breakfast, % daytime naps, with two-thirds of them having difficulties
Daytime naps, % yes waking from naps versus one-third of the controls. Of interest,
Awakening spontaneously 48* 80
from a nap
short naps were not felt to be as refreshing in 75% of the
Difficulties awakening from 67 35 hypersomniacs (while two-thirds of controls felt them to be
a nap refreshing). The patients with short refreshing naps had
Are short naps (<30 min) 25* 65 shorter usual sleep times (9.36 ± 1.33 h versus
refreshing 11.49 ± 2.40 h, P = 0.003). As for long naps, the perception
Are long naps (> 30 min) 48 35
refreshing
of not being refreshed afterwards was similar in both groups
Feels an urgent need to lie 56* 9 (48% versus 35%). Eventually, as many as 56% of the
down hypersomniacs reported the occasional, urgent need to lie
* down, while this symptom was exceptional (9%) in the
P < 0.01 for a difference between the patients and the controls.
controls.

 2010 European Sleep Research Society, J. Sleep Res., 19, 525–534


60
Percent of positive answers
40
20
0
Figure 2. Conditions that help waking up in
the morning in hypersomniacs (dark grey
*
columns) and controls (light grey columns).
Circadian fluctuations of alertness
The Horne–Ostberg score was lower in patients (48.3 ± 12.3)
than in controls (56.7 ± 9.1, P < 0.001), indicating that more
patients than controls were evening type. Fig. 3 displays how
patients and controls scored on a five-point scale for alert-
ness ⁄ tiredness. The patients always scored lower than the
controls, but their changes throughout the day (better alertness
at 11:00 hours and 18:00 hours, lower at 13:00 hours) paral-
leled those of the controls. However, when they were asked to
designate the clock time when they felt the most tired, it was at
the time of awakening in 85% of the patients (versus 48% of
the controls, P = 0.003) and, in contrast, the time was at
22:00 hours in 83% of the controls versus 41% of the
hypersomniacs (P < 0.001).
Daytime alertness
During the MSLT, the patients had lower sleep onset latencies
than the controls, whatever the time test. As expected, the
score on the Epworth sleepiness scale was higher in the 62
patients than in the 50 controls (15.9 ± 3.9 versus 6.5 ± 3.1,
P < 0.001). Similarly, when daytime functioning was
expressed in terms of fatigue, the severity of fatigue was
scored as higher in the patients than in the controls, whether
on the fatigue severity scale (49.7 ± 11.2 versus 32.7 ± 11.2,
P < 0.001) or on the Pichot fatigue scale (25.9 ± 7.9 versus
12.0 ± 4.6, P < 0.001). Most patients were able to differen-
Tiredness/wakefulness score
Figure 3. Temporal fluctuations of self-eval-
uated alertness ⁄ tiredness level in hypersom-
niacs (plain, black line with diamonds) and in
*
controls (dotted grey line with crosses).
 2010 European Sleep Research Society, J. Sleep Res., 19, 525–534
Subjective symptoms in idiopathic hypersomnia 529
* *
* *
Alarm clock Bright light A person Motivation Habit Sounds Stress to be A new Hunger or
helps late activity thirst
P < 0.01 for a difference between the patients and the controls.
tiate sleepiness from tiredness. The first one was described as
an ability (and a need) to fall asleep soon and could be
alleviated with modafinil. In contrast, tiredness was not
necessarily associated with a need and an ability to sleep
(and sometimes even with an inability to fall asleep), but was
expressed as a Ôcognitive fatigueÕ or Ôloss of vital energyÕ (not
physical). A patient reported: ÔI feel sleepy only once a day
(after lunch), but I feel tired all the timeÕ. Another patient said:
Ômodafinil is like botulium toxin, it keeps my eyes open, but my
brain is still asleepÕ. We then duplicated all questions below
using first the terms sleepy ⁄ awake and then the terms tired ⁄
alert, but we obtained similar answers, regardless of the pair of
adjectives that was used.
The conditions considered as equally sedative in both groups
were Ôwaiting ⁄ being boredÕ and routine condition. A quiet
activity was felt as sedative in 64% of the patients versus 16%
in the controls (P < 0.001). Unexpectedly, the need to focus
on a task (whether manual or intellectual) was felt as sedative
by more (29%) patients than controls (7%, P = 0.006).
The conditions able to fight sleepiness (drinking caffeine,
being stressed, performing a sport, being active or hyperactive,
doing something really interesting, thinking too much and
being hungry or thirsty) were reported as often by the patients
and the controls. Stress was considered as the most alerting
condition in the controls (chosen by 73% of them). Being
hyperactive was the most alerting condition in the patients
(chosen by 65% of them). Some patients reported that they
would be more attentive if they were standing up rather than
Morning awakening 11:00 am 1:00 pm 6:00 pm 10:00 pm
P < 0.01 for a difference between the patients and the controls.
530 C. Vernet et al.

sitting, and they would learn or rehearse their class easier while
Table 2 Cognitive, psychological and functional problems in the
walking. A patient wrote down lists of consecutive, useless patients with idiopathic hypersomnia and the healthy controls
numbers on a notebook while attending a meeting so that she
felt more attentive. Another would draw or fill a cross-word Idiopathic Healthy
Patients hypersomnia controls
while waiting for the train and travelling. Several patients
spoke continuously, with a rapid flow, especially when tired. Number 62 50
We tested the effects of various environmental stimuli on the Cognitive problems
ability to adequately function during the daytime (Fig. 4). As Memory problems, % 79* 43
Attention deficit, % 55* 18
for the lighting conditions, darkness had a much more sedative Maximum concentration 1.01 ± 1.15* 3.43 ± 3.30
effect in the patients than in the controls, while both groups duration
felt a similar benefit when exposed to the sun light and an Difficulty focusing in loud 69 38
unchanged (halogen neon or incandescent light, rainy day) or environment, %
decreased (flashing light) alertness when exposed to the other Frequently forgetting 47* 5
something, %
various types of lights. In contrast, the sound conditions, such Mislaying objects, % 55* 18
as a quiet environment, music or listening passively to other Automatic behaviours, %
people, were clearly alerting in controls but not in hypersom- Mind going blank 58* 13
niacs. A loud environment was tiring and sedative in both Automatic activity 87 70
groups. Being on holidays and watching a nice landscape were Not remembering the 41* 11
beginning of an activity
stimulants in both groups, but slightly less so in the patients Being lost in thought 95 84
than in the controls. Stress, workload and frustration had Telling something inappropriate 50* 16
similar tiring effects in both groups. As for interactions with in a conversation
other people, being with friends was a stimulant in both Inappropriate mistake during a 61* 30
groups, but more so in the controls than in the hypersomniacs. usual activity
Psychological problems
Being alone or with strangers did not change the level of HAD anxiety (0–21) 8.7 ± 3.9* 6.4 ± 3.6
alertness in the controls but decreased it slightly, but signif- HAD depression (0–21) 7.0 ± 4.7* 3.9 ± 3.2
icantly, in the hypersomniacs. Functional problems, %
Temperature dysregulation 25* 2
Cold extremities 46* 20
Cognitive, psychological and somatic problems Feeling of faintness 32* 9
Digestive problems 22* 6
The patients reported attention deficit more frequently than Palpitations 23* 5
the controls (Table 2). They felt able to focus for only 1 h in a Eye problems 67 60
row versus almost 4 h in controls. As many as 70% of them Short-sighted 51* 23
had difficulties focusing on their task in a loud environment Allergy 38* 14
versus 38% of controls (P = 0.03). A young student with HAD, hospital anxiety and depression rating scale.
idiopathic hypersomnia noticed: ÔWhen I must rehearse and *P < 0.01 for a difference between the patients and the controls.
learn by heart my lessons, I cannot stand my girlfriend

Figure 4. Effect of different stimuli on the

daytime alertness in hypersomniacs (dark
diamond shape) and in the controls (light
*
P < 0.01 for a difference between the patients and the controls. crosses).

 2010 European Sleep Research Society, J. Sleep Res., 19, 525–534


speaking to me, or any background soundÕ. Patients reported
more frequent memory problems, forgot something more often
(an appointment, a thing to do) and lost their belongings more
frequently.
Automatic behaviours and being lost in thought were
reported in the patients as often as in the controls. But mind
going blank, not remembering the beginning of an activity,
telling something inappropriate in a conversation and a
significant inappropriate life mistake were more frequent in
the patients than in the controls (Table 2). Examples of
automatic behaviours included putting oneÕs cell phone in the
fridge, bringing the garbage bag to the dentist appointment
instead of leaving it in the garage, putting clothes in the
dishwasher, using a hairbrush to wash oneÕs teeth, putting
oneÕs glasses in a videotape case (and not finding them for
3 months) and forgetting to pick up the children at school.
As for psychological aspects, the patients as a group scored
higher both on the anxiety and depression scales (Table 2).
The frequency of subjects with a score higher than the
threshold of 11 (suggesting either an anxiety disorder or
depression) was similar in both groups.
As for somatic complaints, more patients than controls
experienced problems in regulating their body temperature
(heavy sweating, feeling colder or, on the contrary, warmer,
than the other people in the same room) and cold extremities
(Table 2). One-third of them would occasionally faint versus
9% of the controls. Similarly, about one-fifth of the patients
had digestive problems or palpitations, while it was rare in the
controls. Half of the patients were short-sighted (and wore
glasses) versus only 23% of the controls. The other eye
problems (astigmatism, far-sightedness and needing sun-
glasses) were similarly frequent in both groups. Headache,
tinnitus and losing oneÕs hair were equally frequent in both
groups (data not shown). One-third of the patients reported an
allergy versus only 14% in the controls.
DISCUSSION
In this controlled study of the subjective sleep and alertness in
idiopathic hypersomnia, patients have a night-time sleep time
as short as controls during the working days, but much longer
during the weekend, holidays and in the sleep laboratory. As
many as 78% of patients could hardly wake up in the morning
or from a daytime nap, with no specific benefit of alarm clocks,
bright light, motivation, routine and sounds, except if some-
body wakes them up or if they are stressed. Daytime naps are
more frequent and longer than in the controls. Short naps are
refreshing in the controls, but not in 75% of the patients.
During the daytime, the alertness is modulated by the same
external conditions (e.g. higher during a sunny day than a grey
day) in the controls and in the patients, but the patients feel
more sedated in darkness, in a quiet environment, when
listening to music or a conversation, when alone or not. Being
hyperactive, helps them to resist sleepiness more than the
controls. In contrast to the controls, the patients are more
evening time and more alert in the evening than in the morning.
 2010 European Sleep Research Society, J. Sleep Res., 19, 525–534
Subjective symptoms in idiopathic hypersomnia 531
The patients are able to focus only for 1 h (versus almost four
consecutive hours in controls). They complain of attention and
memory deficit, with frequently mislaid objects. Half of them
complain of cold extremities and are near-sighted.
Sleep excess
The Greek-origin word ÔhypersomniaÕ means Ôexcess of sleepÕ,
which captures one of the main essences of the disease. One may
regret that the word hypersomnia has progressively changed
meaning to designate many conditions associated with excessive
daytime sleepiness, but not with a real excess of sleep (Billiard,
1994). In idiopathic hypersomnia, the sleep excess is best
expressed in unrestricted conditions, such as during the
weekend, on holidays and in the sleep laboratory, with an
average of three additional hours slept. One may notice in this
study that the sleep time obtained during long-term monitoring
in the sleep laboratory is very similar to the usual sleep time
during holidays and on weekends in the patients, suggesting it is
not a completely artificial measure, disconnected from true life.
Controls also sleep longer in these conditions, only an extra
hour here, but even more in epidemiological surveys (http://
www.sleepfoundation.org, poll 2006). Hypersomniacs and
controls sleep less during the working days, suggesting that an
actimetry, or a sleep agenda, in these forced life conditions is
poorly sensitive, except if one pays attention to large sleep
differences between working days (more than 7 h) and weekend
days (more than 10–12 h). In addition, the sleep debt caused by
the constraint of working is probably much higher in hyper-
somniacs. Controls can sleep a maximum of 10–13 h in a row
(versus 10–20 h in hypersomniacs), suggesting that being able to
sleep occasionally more than 13 h in a row (without previous
sleep debt) is specific to hypersomniacs. Notably, the patients
and the controls reported the same amount of sleep when they
were 10 years old and the same frequency of a Ôlong-sleeperÕ
phenotype in one or both parents. These results suggest that the
disease is acquired and does not result from an additional sleep
load on an already ÔsleepyÕ phenotype.
Sleep drunkenness
Sleep drunkenness is another symptom of hypersomnia and
constitutes an important disability in the daily life of the
patients. Seventy-eight percent of the patients had difficulties
with morning awakening, and one-third had sleep drunken-
ness, paralleling the percentages (21 and 52%) reported in
other series (Anderson et al., 2007; Billiard and Dauvilliers,
2001). However, this last symptom is highly specific, as clear-
cut sleep drunkenness is not found in controls. Compared with
hypersomniacs without any sleep drunkenness, those with
sleep drunkenness are more frequently evening types on the
Horne–Ostberg score. In this article, hypersomniacs are more
frequently evening types than controls, and more alert in the
evening than in the morning. These data suggest that they have
a delayed shift in their circadian rhythm and a longer circadian
period. To support this hypothesis, one had to perform a
532 C. Vernet et al.
continuous measure of the body core temperature and the
melatonin secretion (ideally during a constant routine to avoid
the masking effect of a long sleep on the temperature) in these
two groups. In our series, the patients with sleep drunkenness
did not have a longer sleep time. The fact that sleep
drunkenness is not correlated with sleep duration argues
against an extreme form of sleep inertia (a normal period of
hypovigilance and impaired cognitive and behavioural perfor-
mances following awakening from naps, increasing with the
duration of the earlier sleep in healthy subjects). The same
observation can be made for naps, as not only long but also
short naps (which had to limit the sleep inertia) are felt as non-
refreshing by 75% of hypersomniacs. Eventually, one may
imagine that forced awakening during slow-wave sleep at the
end of the night would promote disorientation, diminished
mentation and blunted responses to questions. In our study,
however, slow-wave sleep is equally frequent at the end of the
night in the patients with and without sleep drunkenness,
suggesting that the presence of late slow-wave sleep is not
causing the sleep drunkenness. However, this conclusion is
limited by the fact that we do not inquire about sleep
drunkenness the very morning of night 2, but as a general,
frequent symptom. Notably, Roth et al. (1972) observed that
sleep drunkenness is infrequent in the settings of the sleep
laboratory, possibly as a consequence of a lighter, more
fragmented sleep before awakening.
The methods that make awakening in the morning easier are
different in the patients and controls. Of interest, the habit of
waking up at a certain time and the presence of a bright, sunny
light are quite efficient in the controls but not in the
hypersomniacs. The regulation of sleep termination has been
thought to be embedded in a daily circadian rhythm (Czeisler
et al., 1980), controlling in parallel the release of pituitary and
adrenal hormones. A routine, predicted time of sleep offset is
preceded by a gradual increase of adrenocorticotrophin 90–
180 min before the final awakening (Born et al., 1999).
Whether hypersomniacs have delayed pituitary hormone
secretion in the morning (with a cortisol phase delay as
observed by Nevsimalova et al., 2000 in 15 hypersomniacs) or
have become resistant to these strong internal circadian signals
is partly unknown. In this study, the intervention of someone
helps them to wake up, but makes them quite dependent on
others. Notably, a human voice calling someone by his first
name is better processed by the sleeping brain than a sound
during non-REM and REM sleep (Bastuji et al., 2002). A
subject can not only call but also touch and even shake the
sleeping hypersomniac, leading to a multimodal arousal. As a
practical consequence, one may advise hypersomniacs to live
in a student community or with a family (a parent or a
caregiver) with someone responsible for waking them up.
Daytime alertness
Although patients insist on differentiating between sleepiness
(as estimated by their ability to fall asleep in passive condi-
tions, e.g. using the MSLT or the Epworth score) and
tiredness ⁄ decreased alertness, we could not find stimuli that
preferentially affect the sleepiness rather than the tired-
ness ⁄ alertness, they always go in the same direction. Billiard
(1994) previously noticed that most patients with idiopathic
hypersomnia never feel fully awake during the daytime, even if
they can resist sleep easier than narcoleptics (Komada et al.,
2005). During the daytime, alertness is modulated by the same
external conditions (e.g. higher during a sunny day than a grey
or rainy day or than when exposed to artificial lighting,
regardless of whether it is neon, halogen or incandescent
lighting) in the controls and patients, but the patients feel more
sedated than the controls in darkness. Bright light (e.g. 8–
10 000 lux, closer to sunlight) has already been demonstrated
to increase alertness in workers (Santhi et al., 2008), but we are
not aware of studies showing an alertness change reported
during a grey or rainy day, so one may encourage hypersom-
niacs to use sun-like bright lights when working. Noises and
loud environments are perceived as sedative by the controls
and hypersomniacs, possibly because of the increase load to
focus oneÕs attention. In contrast, a quiet environment, music
and conversation help the controls (but not the hypersomni-
acs) to feel alert and focused. However, being with friends (and
not with strangers) is perceived as stimulating in the hyper-
somniacs, even if it is to a lesser degree than the controls.
Taken together, it seems that hypersomnia narrows the
spectrum of conditions associated with full alertness, given
that the patients feel tired in the presence of over-stimulating
conditions (a loud environment, strangers and flashing light),
and feel sleepy in under-stimulating conditions (darkness, left
alone or listening to a conversation). Basically, it appears in
this study that the patients would feel all right only during
holidays, in a nice landscape with sun and friends. One may
wonder if they use, in this case, the motivation ⁄ mood system
to stay awake rather than the usual arousal systems.
In addition, being hyperactive helps hypersomniacs to resist
sleepiness more than controls. They use this term to describe
both any increased motor activity (such as standing up rather
than sitting, walking while learning or speaking continuously)
and doing several tasks at the same time (such as writing while
listening). Hyperactivity is a symptom of attention def-
icit ⁄ hyperactivity disorder. In this case, excessive motor
activity can be viewed as a strategy to stay awake and alert,
while decreased attention could be the consequence of the
hypoarousal (Lecendreux et al., 2000). We suspect that the
hypersomniacs use the motor arousal to supplement their
cognitive arousal, and the stress of multi-tasks to increase their
level of alertness by fighting monotony. As a consequence,
they could get more tired. Hence, we wonder if the feeling of
tiredness that the patients described as different from sleepi-
ness is a general lack of mental energy, as a consequence of
using multi-modal systems to fight sleepiness.
Cognitive and somatic symptoms
A deficit of attention has been previously described in
patients with idiopathic hypersomnia (Oosterloo et al., 2006).
 2010 European Sleep Research Society, J. Sleep Res., 19, 525–534

Executive dysfunction is a consequence of many disorders
with excessive daytime sleepiness, including narcolepsy, sleep
apnoea syndrome and sleep deprivation (Anderson et al.,
2009; Lis et al., 2008; Naumann et al., 2006); it had to be
improved using stimulants. We could not find any previous
report of defective memory in idiopathic hypersomnia.
Whether the patients have a long-term memory deficit (i.e.
in absence of attention and working memory deficit) is
unlikely, but had to be formally assessed. In the present
study, the patients notably reported that they could not
sustain attention for more than 1 h, versus almost 4 h in the
controls. This limited mental endurance suggests that patients
with idiopathic hypersomnia have a cognitive fatigability, as
it was described in patients with chronic fatigue syndrome
(Capuron et al., 2006); this aspect could be tested on
sustained attention tests. Automatic behaviours are found
both in patients and in controls, but the severity is different.
Telling something inappropriate in a conversation and
making a mistake during a usual activity are more frequently
experienced by patients than by healthy controls (except if
they are sleep deprived). Hence, one may interview patients
about the frequency of these behaviours. When the patients
mix two different activities during routine tasks, they seem
only partly able to shift from an action (bringing the garbage
to the cellar) to another action (going to the dentist). As this
sort of shifting (and focusing) is seen as a frontal lobe
function, it suggests that their automatic programs are no
longer driven by the frontal lobe. Although they are not
monitored, they do not seem to be drowsy or in stage one
during these behaviours. Rather, they are unfocused. The
automatic behaviours can be partly responsible for the
ÔmemoryÕ problems reported by the patients. For example, a
patient lost her glasses for 3 months because she had put
them into a videotape case. She spontaneously reports this
incident as a memory problem.
Some somatic problems regarding the autonomic nervous
system (Raynaud-like syndrome, migraine and orthostatic
syncope) have already been described in hypersomnia
(Bassetti and Aldrich, 1997; Matsunaga, 1987; Roth et al.,
1972). In this study, some functional equivalents (cold
extremities and fainting) are indeed more frequent in
hypersomniacs than in controls, while there is no more
headache in the patient group. In addition, half of the
hypersomniacs are near-sighted, 38% are allergic and 25%
have problems regulating their temperature. The mechanism
of these symptoms is unknown, they could contribute to the
burden of the disease.
The symptoms reported in this article are subjective, which
constitutes a limitation of this work. There is no formal testing
of the cognitive status by a neuropsychologist, no measure of
supine hypotension, no systematic sight assessment in the
controls, so that a bias towards over-reporting in patients is
still possible. The questionnaire is, however, the same for any
subject, whether hypersomniac or not. On the contrary, we try
to catch the somatic problems of the patients, which are by
definition subjective.
 2010 European Sleep Research Society, J. Sleep Res., 19, 525–534
Subjective symptoms in idiopathic hypersomnia 533
ACKNOWLEDGEMENTS
Cyrille Vernet received four unrestricted grants, ANTADIR
AO2006, UCB-Pharma Ltd AO2007, CARDIF AO2007 and
ANC 2008. Part of this work was financed by the PHRC-
P070138.
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SUPPORTING INFORMATION
Additional Supporting Information may be found in the online
version of this article:
Table S1. Polysomnographic characteristics of 62 patients
with idiopathic hypersomnia and 30 matched controls, during
48-h sleep monitoring.
Table S2. Sleep determinant of morning inertia in 18
hypersomniacs with a frank sleep drunkenness versus 32
hypersomniacs without any sleep drunkenness.
Appendix S1. Questionnaire.
Please note: Wiley-Blackwell are not responsible for the
content or functionality of any supporting materials supplied
by the authors. Any queries (other than missing material)
should be directed to the corresponding author for the article.
 2010 European Sleep Research Society, J. Sleep Res., 19, 525–534