title: Sleep patterns in congenital dopamine beta-hydroxylase deficiency.
abstract: Sleep patterns of two young female patients with congenital dopamine beta-hydroxylase deficiency are described. In this orthostatic syndrome central and peripheral noradrenergic failure occurs as a result of impaired beta-hydroxylation of dopamine. Consequently, the levels of dopamine and its metabolites are elevated. The relative importance of noradrenaline deficit in the face of dopamine excess for sleep-regulatory mechanisms can be inferred from the sleep pattern of these patients. No subjective sleep complaints were reported. The sleep patterns showed a high percentage of slow-wave sleep in both patients (29% and 34% of sleep period time) and a relatively low to normal percentage of REM sleep (18% and 21%). A normal cyclic REM sleep pattern was observed. Alpha-delta sleep occurred during light sleep (15% and 8%); consequently, the amount of stage 2 sleep was reduced. These results indicate that functional insufficiency of the noradrenergic system in two patients with dopamine beta-hydroxylase deficiency is not associated with profound changes in the (REM) sleep pattern. This supports a modulatory or permissive role for noradrenaline in REM sleep mechanisms.
authors.affiliation: Department of Psychiatry, University Hospital Dijkzigt, Rotterdam, The Netherlands.
authors.forename: J H
authors.initials: JH
authors.lastname: Tulen
authors.forename: A J
authors.initials: AJ
authors.lastname: Man in't Veld
authors.forename: K
authors.initials: K
authors.lastname: Mechelse
authors.forename: F
authors.initials: F
authors.lastname: Boomsma
mesh_terms: D000328:Adult; D001794:Blood Pressure / Q000502:physiology; D004298:Dopamine / Q000378:metabolism; D004299:Dopamine beta-Hydroxylase / Q000172:deficiency*; D005260:Female; D006339:Heart Rate / Q000502:physiology; D006801:Humans; D009638:Norepinephrine / Q000378:metabolism; D012890:Sleep; D012895:Sleep, REM
chemical_list: D004299:Dopamine beta-Hydroxylase; D004298:Dopamine; D009638:Norepinephrine