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Abstract
To review the clinical and polysomnographic characteristics of idiopathic hypersomnia
as well as the long-term response to treatment.
The Respiratory Support and Sleep Centre at Papworth Hospital, Cambridge, UK.
A large database of more than 6000 patients with sleep disorders was reviewed. A retrospective
study of the clinical and polysomnographic characteristics of 77 patients with idiopathic
hypersomnia was performed. Comparison with a similar group of patients with narcolepsy
was performed. The response to drug treatment was assessed in 61 patients over a mean
follow-up of 3.8 years.
Idiopathic hypersomnia was 60% as prevalent as narcolepsy. Comparison with a similar
group of patients with narcolepsy showed that those with idiopathic hypersomnia were
more likely to have prolonged unrefreshing daytime naps, a positive family history,
increased slow-wave sleep, and a longer sleep latency on the Multiple Sleep Latency
Test. The results of the Multiple Sleep Latency Test were not helpful in predicting
disease severity or treatment response. The clinical features were heterogeneous and
of variable severity. The majority of patients with idiopathic hypersomnia had symptoms
that remained stable over many years, but 11% had spontaneous remission, which was
never seen in narcolepsy. Two thirds of patients with idiopathic hypersomnolence had
a sustained improvement in daytime somnolence with medication, although a third needed
high doses or combinations of drugs.
Idiopathic hypersomnolence has characteristic clinical and polysomnographic features
but the prolonged latency on the Multiple Sleep Latency Test raises doubt about the
validity of this test within the current diagnostic criteria. The disease often responds
well to treatment and a substantial minority of patients appear to spontaneously improve.