Sleep

  NECDIN is a gene that is deleted or inactivated in those with Prader-Willi syndrome , as it is found on chromosome 15 in the 15q11-q13 region. The gene product, Necdin, can be knocked out in mice, and a model for PWS thus emerges that displays some of the same respiratory and abnormal sleep breathing patterns that are often seen in humans with PWS. Necdin acts to stop cell death during the normal process of cell death in cells. The authors hypothesize that Necdin deficiency in mice (and perhaps even in humans) may result from deficits in the maturation and proper functioning of networks in the brain and set out to examine the relationship between Necdin and the neurotransmitter serotonin (5-HT) to better describe how 5-HT may affect Necdin-modulated breathing problems (Necdin Plays a Role in the Serotonergic Modulation of the Mouse Respiratory Network: Implication for PWS , Zanella et al., The Jl of Neurosci, 2008).
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Obstructive sleep apnea (OSA) and other sleep disturbances are common in people with Prader-Willi syndrome. Many studies in the medical literature suggest that the oxygen deprivation and fragmented sleep associated with OSA are associated with deficits in learning. A new study looks at just how damaging untreated OSA can be in children.[ Read more . . . ]

Modafinil (Provigil) has been suggested as a potential wake-promoting drug that might be helpful in PWS. Interestingly, this article (although a very small study) showed that individuals taking modafinil consumed less food. Compared to amphetamine, which also increases wakefulness and decreases appetite, modafinil did not cause increased heart rate at high doses.
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Here is a new study is just out in the journal “Pediatrics” using the new formulation of modafinil for ADHD in children (age 6-17) [ Read more . . . ]

You may remember that orexin is a small protein (peptide) released in the brain, which was originally identified because it stimulates feeding. In addition to a role in feeding/energy regulation, it is also critical in wakefulness. People with narcolepsy have very low (to undetectable) levels of orexin in their cerebral-spinal fluid (CSF). Notably, the only other disorder that has been associated with low orexin in the CSF to date (and it has only been tested in a couple of individuals) is PWS.[ Read more . . . ]

Here is a recent study (link to abstract below) looking at the use of modafinil, a wake-promoting drug, in children with excessive daytime sleepiness (in this study, primarily narcolepsy).[ Read more . . . ]