Brain, Vol 120, Issue 8 1423-1435, Copyright © 1997 by Oxford University Press
C Bassetti and MS Aldrich
The features of idiopathic hypersomnia are not well defined. We reviewed
clinical and laboratory information on 42 subjects with idiopathic
hypersomnia and obtained detailed follow-up evaluations on 28 of them. Only
29% of subjects had 'classic' idiopathic hypersomnia with non-imperative
sleepiness, long unrefreshing naps, prolonged night- time sleep, difficult
awakening with sleep drunkenness and prominent mood disturbances.
Thirty-two percent had clinical features similar to narcolepsy, i.e.
irresistible sleepiness, short and refreshing naps, few problems with
awakening and good response to stimulants, without cataplexy or any
indication of abnormal REM (rapid eye movement) sleep. The other 39% had
intermediate clinical characteristics. We found no increase in the
frequency of the human leucocyte antigens associated with narcolepsy.
Overall, response to stimulants was good in three- quarters of the patients
and spontaneous improvement of sleepiness occurred in one-quarter. Possible
aetiologies identified in 10 patients included viral illness, head trauma
and primary mood disorder. Idiopathic hypersomnia is a rare syndrome in
which clinical heterogeneity suggests a variable or multifactoral
pathogenesis. Only a minority of cases correspond to classical
descriptions. Stimulants are often beneficial and spontaneous improvement
appears to be more common than in narcolepsy.
ARTICLES
Idiopathic hypersomnia. A series of 42 patients
Department of Neurology, University of Michigan Medical Center, Ann Arbor, USA.
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