PT  - JOURNAL ARTICLE
AU  - Hélène De Leersnyder
AU  - Marie-Christine de Blois
AU  - Michel Vekemans
AU  - Daniel Sidi
AU  - Elisabeth Villain
AU  - Catherine Kindermans
AU  - Arnold Munnich
TI  - β<sub>1</sub>-adrenergic antagonists improve sleep and behavioural disturbances in a circadian disorder, Smith-Magenis syndrome
AID  - 10.1136/jmg.38.9.586
DP  - 2001 Sep 01
TA  - Journal of Medical Genetics
PG  - 586--590
VI  - 38
IP  - 9
4099  - http://jmg.bmj.com/content/38/9/586.short
4100  - http://jmg.bmj.com/content/38/9/586.full
SO  - J Med Genet2001 Sep 01; 38
AB  - Smith-Magenis syndrome (SMS) is a clinically recognisable contiguous gene syndrome ascribed to interstitial deletions of chromosome 17p11.2. Patients have a phase shift of their circadian rhythm of melatonin with a paradoxical diurnal secretion of the hormone. Serum melatonin levels and day-night behaviour were studied in nine SMS children (aged 4 to 17 years) given acebutolol, a selective β1-adrenergic antagonist (10 mg/kg early in the morning). Cardiac examination, serum melatonin, motor activity recordings, and sleep diaries were monitored before and after drug administration. The present study shows that a single morning dose of acebutolol suppressed the inappropriate secretion of melatonin in SMS. A significant improvement of inappropriate behaviour with increased concentration, delayed sleep onset, increased hours of sleep, and delayed waking were also noted. These results suggest that β1-adrenergic antagonists help to manage hyperactivity, enhance cognitive performance, and reduce sleep disorders in SMS.