An 18-month-old girl with genetically confirmed Smith-Magenis syndrome (SMS) presented to the pediatric sleep clinic with excessive behavioral problems and a poor sleep pattern. She would start feeling drowsy between 5-6 PM, followed by multiple awakenings lasting 5-8 minutes, requiring being bottle fed or rocked. She would wake up at 2 AM and remain active and playful thereafter. She was hyperactive and restless throughout the day, associated with temper tantrums and head banging. A 24-hour polysomnography showed decrease in total sleep time (6 hours), delayed sleep latency (22 min), delayed REM latency (132 min) and multiple night awakenings. There were no features of obstructive sleep apnea.

Sleep coaching was initiated by setting a regular sleep routine at night. A time gap of one hour between feeding or play and sleep was maintained. All sleep associations in the form of rocking and feeding as well as co-sleeping were stopped with graduated extinction. Her night time sleep was delayed by 15 minutes each day, till she was able to sleep by 9 PM. The bed room was darkened and all access to multimedia screens was removed. Within one month, she was able to sleep by 9 PM and wake up at 6:30 AM, with no night awakenings. Her behavioral symptoms and tantrums during the day resolved. She was maintaining this schedule at the 6-month follow-up.

SMS is characterized by infantile hypotonia, expressive speech delay, mental retardation, short stature, scoliosis, characteristic craniofacial features and self-injurious behavior [1]. Sleep issues in children with SMS commonly include early sleep onset, frequent nocturnal awakenings, early morning arousal and daytime sleepiness [2]. There is increasing evidence of an inverted melatonin rhythm in SMS with low levels of melatonin at night, and significantly high levels during the day [3]. Behavioral problems increase in children when the levels peak and sleep attacks are noted when levels drop. Administering melatonin; however, only enables a patient to sleep earlier and does not affect the early morning awakening or behavioral changes. Sleep coaching has shown to produce reliable and durable changes in infant sleep patterns [4]. This report demonstrates that sleep issues in children with SMS can be managed with sleep coaching alone.

1. Greenberg F, Lewis RA, Potocki L, et al. Multidisciplinary clinical study of Smith-Magenis syndrome (deletion 17p11.2).　Am J Med Genet. 1996;62:247-54.

2. De Leersnyder H, De Blois MC, Claustrat B, et al. Inversion of the circadian rhythm of melatonin in the Smith-Magenis syndrome. J Pediatr. 2001;139:111-6.

3. Potocki L, Glaze D, Tan DX, et al. Circadian rhythm abnormalities of melatonin in Smith-Magenis syndrome. J Med Genet. 2000;37:428-33.

4. Mindell JA, Kuhn B, Lewin DS, Meltzer LJ, Sadeh A. Behavioural treatment of bedtime problems and night wakings in infants and young children. Sleep. 2006; 29:1263-76.