Screening Angelman Patients for Sleep Disorders Can Help Manage Problems
Screening patients with Angelman syndrome and other neurodevelopemental disorders for sleep disturbances can enable multidisciplinary management, and diagnosing and treating these problems may improve patient quality of life, a review that looked at eight neurogenetic diseases reported.
The study, “Sleep Disorders in Childhood Neurogenetic Disorders,” was recently published in the journal Children.
Compared to the general population, children with neurodevelopmental disorders have a higher prevalence of sleep-related problems (25 percent vs. 80 percent, respectively, in preschool-age children). This difference is somewhat expected, given that sleep is a multifaceted function of the central nervous system.
In addition to the physiological elements of the disorders themselves, causes underlying sleep disturbances can be related to effects of medication and troubled breathing.
As many as 80 percent of Angelman syndrome patients suffer from sleep problems, which seem to be more severe between the ages of 2 and 6 but may diminish or disappear by late childhood.
Among the problems experienced by Angelman patients are difficulty initiating and maintaining sleep, reduced total sleep time, sleep-wake rhythm disorders, involuntary urination at night (enuresis), involuntary grinding of teeth (bruxism), sleep walking, sleep paralysis, excessive daytime sleepiness, and sleep-related breathing difficulties.
Some of the sleep disturbances observed in Angelman patients are thought to be the result of the condition itself, while others may be the effects of anti-epileptic drugs used in children experiencing recurrent seizures consistent with epilepsy.
Studies have found that Angelman patients have a lower level of nocturnal serum melatonin, a hormone that plays a key role in sleep regulation. Low doses of melatonin increased total sleep time for six days in one study, but the maintenance of this effect was not studied. Patient numbers in these studies were small, and additional research is needed to determine whether melatonin supplementation can be a valid and safe option for sleep management in Angelman children.
Sleep disturbances can impact cognitive development, social interactions, and behavior. Parents of Angelman children have been reported to experience more depression, stress, and anxiety compared with parents of children with other rare genetic conditions.
Stabilization of sleep and wake patterns by limiting stimulating factors and a consistent schedule are ways to help manage sleep-related problems in patients with neurogenetic disorders.
“The bedroom environment should focus on prevention of injury associated with sleep walking by limiting the height of the bed, obstructing access to windows, and removing features that may cause injury,” the researchers said. Patients may also have seizures at night and require higher doses of anticonvulsants.
Behavioral management of sleep-related problems is also beneficial and can involve encouraging independent sleep initiation and empowering parents to have a sleep-compatible environment for the child.
Screening patients with neurodevelopmental disorders for sleep disturbances during routine medical examinations is important since “treatment of sleep related disorders in patients with these complex and often multi-system diseases are often best managed in a multidisciplinary setting,” the researchers said.
