SLEEP 2011 Abstract Supplement

B. Clinical Sleep Science XI. Pediatrics
estimated using 2003 NIH workshop criteria for RLS in children and the
Pediatric Sleep Questionnaire.
Results: Fifty-six of 102 surveys were returned, 48 of which were
evaluable. Eleven respondents (23%) had probable or definite RLS.
Nineteen (40%) had survey answers consistent with PLMS. All subjects
who met criteria for RLS also had responses consistent with PLMS. Five
of 11 respondents with RLS (46%) reported using medication for TS
(SSRI’s/SNRI’s, stimulants, or alpha-2 agonists), with 3/ 11 (27%) reporting
polypharmacy. Thirteen of 19 respondents with probable PLMS
(68%) reported using medication (SSRI’s/SNRI’s, stimulants, neuroleptics,
alpha-2 agonists, benzodiazepines), with 10/ 19 (53%) reporting
polypharmacy.
Conclusion: Based upon this cohort, it appears that children with TS
have higher rates of both RLS (23%) and PLMS (40%) than the general
population (2.0%). Although this association is made ambiguous
by concurrent medication use, even after removing patients with current
medication exposure, an estimated 12.5% (6/48) of TS patients endorsed
RLS/PLMS. Future studies examining RLS and PLMS in larger groups
are needed to clarify the relationship of TS and RLS/PLMS and its neuropharmacology.
0875
HEART RATE RESPONSES TO SPONTANEOUS AROUSALS
FROM SLEEP IN CHILDREN WITH CHROMOSOME 15Q11-
13 DUPLICATION SYNDROME: HYPOAROUSAL AS A
CONTRIBUTOR TO SUDDEN DEATH?
Maski K 1 , Kothare SV 1 , Wood C 3 , Katz E 2
1
Neurology, Children’s Hospital Boston, Boston, MA, USA,
2
Pulmonology, Children’s Hospital Boston, Boston, MA, USA, 3 Sleep
Medicine, Beth Israel Deaconess Hospital, Boston, MA, USA
Introduction: Chromosome 15q11-13 contains imprinted genes essential
for normal mammalian neurodevelopment. Over-expression of
maternally expressed imprinted genes is associated with a neurodevelopmental
disorder with autistic features. There is an increased risk of
sudden unexpected death during sleep among children and adults with
chromosome 15q duplication syndrome, estimated at 1% per year. The
etiology of the sudden death has not been established, even after autopsy.
We tested the hypothesis that hypoarousal may be present in children
with 15q duplication syndrome as evidenced by a reduced heart rate
(HR) response to spontaneous arousal from sleep.
Methods: Five children with chromosome 15q11-13 duplication who
had overnight polysomnograms (PSGs) for suspicion of sleep-disordered
breathing were compared to 5 age/gender matched healthy controls with
normal PSGs. A total of 30 spontaneous arousals were identified during
NREM sleep based on guidelines of the ASDA criteria (an abrupt shift
in EEG frequency lasting >3 seconds). The arousal length, baseline heart
rate, and heart rate response to arousal were compared using an unpaired
t-test with statistical significance at p 2 or > 5 obstructive events per hour. Events were
either an apnea, or hypopnea with 3% desaturation.
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SLEEP, Volume 34, Abstract Supplement, 2011