11 April 2011

An article in the journal Sleep, dated October 2007, makes a comparison between Narcolepsy and Idiopathic Hypersomnia patients. The clinical and polysomnographic presentations of both groups are reviewed. How well each group responds to medication is also discussed.

Here are some brief excerpts from the article:

The clinical and polysomnographic features of the patients with idiopathic hypersomnia
were compared with the features of the patients with narcolepsy to see if there were
any statistically significant differences. Comparisons are summarized in Table 2. A
number of predictive factors made a diagnosis of idiopathic hypersomnia more likely, and
these are summarized in Table 3; the sensitivity and specificity of the key predictive
factors are also shown. Patients with idiopathic hypersomnia were significantly more
likely to be sleep drunk and to have a positive family history, nighttime sleep of 9 or more
hours, and a daytime nap duration of 60 or more minutes. They were also more likely to
have a body mass index of less than 30 and, without treatment, to have an ESS score of
less than 20. When we reviewed the polysomnography data, we found that patients with
idiopathic hypersomnia were significantly more likely to have more than 20% of slow-wave
sleep, a sleep efficiency of greater than 90%, and a sleep latency on MSLT of longer than
8 minutes…

Of the factors in Table 3, the single most useful indentifier in the clinical history to reliably
distinguish idiopathic hypersomnia from narcolepsy was daytime napping of greater than
an hour, which had both a sensitivity and a specificity of 87%…

Of 54 patients starting modafinil (mean dose 400 mg, range 100–1000 mg), 39 remained
on this alone, 8 switched to dexamphetamine because of side effects, and 7 reported
lack of efficacy, with 3 adding caffeine (50–100 mg) and 3 adding dexamphetamine. Of the
7 remaining treated patients, 2 patients used dexamphetamine then modafinil, 2 used
alternative stimulants (pemoline and methylphenidate), and 3 patients were treated
with sleep hygiene.

Modafinil alone helped 62%, with the respondents showing a drop of more than 4 points
on their ESS scores and reporting decreased daytime napping. Nineteen of these patients
had a normal ESS score (<11) on medication. Two patients had transient benefit from
Modafinil with decreased efficacy after a few weeks, and they were counted as

Of the patients who used another drug or combinations of drugs, 57% had a significant
drop in ESS their score, with 6 of these patients having an ESS score less than 11…

There have been no epidemiologic studies, and variable criteria have been used to
diagnose idiopathic hypersomnia. The prevalence of the disease remains unclear.
Narcolepsy is said to affect up to 0.05% to 0.1% of American and European populations.
Over time, the ratio of idiopathic hypersomnia to those patients with narcolepsy appears
to have decreased, with the most recent studies showing the condition to be 10% to
15% as common as narcolepsy when studying referrals to sleep centers….

There were a number of patients (IH) who felt that they had functional benefit and
decreased daytime napping with stimulant medication but did not have a significant drop
in ESS scores. They still chose to remain on medication. The ESS is the most widely
used questionnaire to assess EDS, but it may be that other measures of alertness may
have shown benefit…

SLEEP, vol. 30 (01 October 2007)


Links to this article can be found below.
Journal Sleep
PubMed (read full report here)


11 April 2011

An article on PlosOne, dated 05 March 2010, reports that there appears to be a connection between Narcolepsy and the immune / autoimmune system. The research described in this article also mentions their findings in regards to Idiopathic Hypersomnia.

Here are some brief excerpts from this article:

Our study is the first to determine IgG abnormalities in Narcolepsy and Idiopathic
Hypersomnia by measuring the serum IgG levels in a large number of hypersomnia
patients. The observed IgG abnormalities indicate humoral immune alterations in
Narcolepsy and Idiopathic Hypersomnia. Different IgG profiles suggest immunological
differences between Narcolepsy and Idiopathic Hypersomnia.

PLoS ONE 5(3): e9555. doi:10.1371/journal.pone.0009555


Links to this article can be found below.


11 April 2011

An article in Brain (A Journal of Neurology), dated August 2001, addresses the subject of Excessive Daytime Sleepiness (EDS) and the diagnostic and treatment problems it can cause for Neurologists.

Here are some brief excerpts from the article:

According to the National Sleep Foundation 2000 Omnibus Sleep in America Poll: ‘a
sizable proportion of adults (43%) report that they are so sleepy during the day that
it interferes with their daily activities a few days per month or more; and, one out of
five (20%) experience this level of daytime sleepiness at least a few days a week or
more’. A recent study (Powell et al., 1999) demonstrated that decreased performance
due to sleepiness may be worse than that associated with alcohol intoxication.

EDS exacts a significant toll on individuals and on society. On the individual level, the
symptom itself not only reduces personal effectiveness at school or work, but it also
leads to problems with concentration, memory and mood, which have further negative
impacts on performance. On the societal level, the negative impacts of sleepiness are
likewise significant…

Various tools have been developed to assess sleepiness more objectively, but each of
these has shortcomings. These instruments explore different aspects of sleepiness, and
several tools may be needed to evaluate a given patient. Investigators must recognize
the limitations of the available methods and select them according to the clinical
problem being addressed…

Idiopathic CNS Hypersomnia
This disorder is characterized by EDS, but without cataplexy or nocturnal sleep disruption
(Billiard et al., 1998). It is believed to be less common than Narcolepsy, but prevalence
is diffiucult to determine, because strict diagnostic criteria are lacking, and no specific
diagnostic marker is currently available…No amount of sleep ameliorates the EDS, and
naps are generally not refreshing. Occupational and social functioning may be severely
impacted by sleepiness. Polysomnography usually reveals shortened initial sleep latency,
increased total sleep time and normal sleep architecture. Mean sleep latency on MSLT is
usually reduced, often in the 8-10 min range, but SOREMP’s are not seen. Treatment is
often less than satisfactory and includes stimulant medication (see Table 1) and lifestyle

Brain, August 2001


Links to this article can be found below.
Brain (read full article here)
UK PubMed Central


11 April 2011

The Journal of Clinical Sleep Medicine, in an article dated 15 December 2009, reports on the clinical presentation of Idiopathic Hypersomnia and its response to treatment. This article was in response to a call from the American Academy of Sleep Medicine for more research on Idiopathic Hypersomnia.
Here is a brief excerpt from that article:

A retrospective review of our database initially identified 997 patients, utilizing
“Idiopathic Hypersomnia”, “Hypersomnia NOS”, and “Primary Hypersomnia” as
keywords. The charts of eligible patients were examined in detail, and data
were abstracted and analyzed. Response to treatment was graded utilizing an
internally developed scale…

Eighty-five patients were ultimately identified (65% female)…65% of patients
demonstrated a “complete response” to pharmacotherapy as assessed by the
authors’ grading schema…At the last recorded follow-up visit, 92% of patients
were on monotherapy, with greater representation of Methylphenidate versus
Modafinil (51% vs. 32%). Among these patients, methylphenidate produced a
higher percentage of “complete” or “partial” responses than Modafinil, although
statistical significance was not reached…

The majority of patients with idiopathic hypersomnia respond well to treatment.
Methylphenidate is chosen more often than modafinil as final monotherapy in the
treatment of idiopathic hypersomnia, despite the fact that it is less commonly
used initially. Further prospective comparisons of medications should be explored.

Journal of Clinical Sleep Medicine (15 Dec 2009)


Links to this article can be found below.
Journal of Clinical Sleep Medicine
PubMed #2


11 April 2011

An article posted in the Journal for Psychosomatic and Psychotherapeutic Medicine (PsychoSom), dated March-April 2000, reports that being able to distinguish between Idiopathic and Psychiatric Hypersomnia is tricky. The following study was undertaken in order to see if polysomnography could help.

Here are some brief excerpts from the study:

During nocturnal sleep, patients with psychiatric hypersomnia showed significantly
higher sleep latency, wake time after sleep onset, and total wake time and a
significantly lower percentage of sleep time than patients with primary hypersomnia
and control subjects (p < .05). In addition, the daytime sleep of patients with
psychiatric hypersomnia was significantly higher in terms of sleep latency, total
wake time, and percentage of light (stage 1) sleep and lower in terms of percentage
of sleep time and stage 2 sleep than in patients with primary hypersomnia and
control subjects (p < .05). The daytime sleep of patients with primary hypersomnia
as compared with that of control subjects was characterized by lower sleep latency
and total wake time and a higher percentage of sleep time (p < .05). Finally, a
sleep latency of less than 10 minutes or a sleep time percentage greater than 70%
in either of the two daytime naps was associated with a sensitivity of 78.0% and
a specificity of 95.7%.

Conclusions: Our findings indicate that psychiatric hypersomnia is a disorder of
hyperarousal, whereas primary hypersomnia is a disorder of hypoarousal.
Polysomnographic measures may provide useful information in the differential diagnosis
and treatment of these two disorders.

Psychosom Med. 2000 Mar-Apr;62(2):220-6


Links to this article can be found below.
Psychosomatic Medicine


11 April 2011

The World Health Organization, in an article dated 08 February 2011, reports that there appears to be a connection between a vaccine and an increase of cases of Narcolepsy.

Here is a brief excerpt from that article:

Since August 2010, following widespread use of vaccines against influenza
(H1N1) 2009, cases of narcolepsy, especially in children and adolescents,
have been reported from at least 12 countries. Narcolepsy is a rare sleep
disorder that causes a person to fall asleep suddenly and unexpectedly.
The rates reported from Sweden, Finland and Iceland have been notably
higher than those from other countries. The National Institute for Health
and Welfare of Finland issued a preliminary statement on 1 February 2011
following an investigation into the cases in Finland. A systematic
retrospective registry-based review was conducted of all new narcolepsy
cases diagnosed during 2006-2010 and cases in 2009-2010, born in 1990
or later, were reviewed using newly developed Brighton collaboration
criteria for the disease. During 2009-2010 they found a higher risk of
narcolepsy among those aged 4-19 years old who had received the
vaccination against influenza (H1N1) 2009 compared with those who had
not been vaccinated. The only pandemic vaccine used in Finland was
Pandemrix, an adjuvanted influenza (H1N1) 2009 monovalent vaccine
manufactured by GlaxoSmithKline. Pandemrix vaccine was used in 47
countries worldwide during the 2009-2010 season. Studies are ongoing to
determine if the apparent increased risk of narcolepsy reported in Sweden
is higher in vaccinated persons.

The National Institute in Finland (on the advice of the Finnish National
Narcolepsy Task Force) has concluded that the risk of developing narcolepsy
among those vaccinated aged between 4 and 19 years is about nine times
greater than those unvaccinated in the same age group, corresponding to
a risk of about 1 case of narcolepsy per 12,000 vaccinated in this age
group. The increased risk has not been seen in younger or older age groups.
Narcolepsy is a condition that has a strong genetic linkage, being almost
uniquely seen in persons who have the (HLA) DQB1*0602 genotype. Of the
cases of narcolepsy tested so far in Finland (n=22), diagnosed during
2009-2010, all have that genotype. The National Institute considers it
probable that the Pandemrix vaccine was a contributing factor to this
observed increase, and has called for further investigation of other co-factors
that may be associated with the increased risk. They consider it most likely
that the Pandemrix vaccine increased the risk of narcolepsy in a joint effect
in those genetically disposed with some other, still unknown, genetic and/or
environmental factor. The final report from the Finnish National Narcolepsy
Task Force is expected by 31 August 2011…

Links to this article can be found below.
World Health Organization (WHO)
National Institute for Health and Welfare of Finland


11 April 2011

The Journal of Sleep Research (01 December 2010) has an interesting study on Idiopathic Hypersomnia (IH). This study attempts to summarize the various symptoms sufferers of IH have in common. This is an article worth reading for anyone interested in the study of Idiopathic Hypersomnia.

Here is a brief summary of the article:

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 hours) 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 hours 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-types and more alert in the evening than in the
morning. The patients were able to focus only for 1 hour (versus 4 hours 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.

Journal of Sleep Research, Volume 19, Number 4 (01 December 2010)


Links to this article can be found below.
Wiley Online Library
Ingenta Connect
Journal of Sleep Research


11 April 2011

The journal Sleep (01 June 2005) reports on the risks associated with high doses of stimulants, when treating excessive somnolence. Among the risks listed in the article are tachycardia, weight loss, anorexia, psychosis and substance abuse.

Here is a brief excerpt from this article:

116 patients with narcolepsy or idiopathic hypersomnia were individually matched by
sex, diagnosis, age of onset, and duration of follow-up from both onset and diagnosis.
Members of the high-dose group had received at least 1 stimulant at a
dosage > or = 120% of the maximum recommended by the American Academy of Sleep
Medicine Standards of Practice Committee. The standard-dose control group had
received stimulants at a dosage < or = 100% of the American Academy of Sleep
Medicine guidelines…

Sleep. 2005 June 01; 28(6): 663.


Links to this article can be found below.