Cognition and Behavior in Pre-Pubertal Children With Prader-Willi Syndrome and Associations With Sleep-Related Breathing Disorders

摘要: Prader-Willi syndrome (PWS) is characterized by hypotonia, hypogonadism, obesity, and short stature. Neurobehavioral abnormalities, cognitive impairment, sleep-related breathing disorders (SRBD) are common. In the general population associations between neurobehavioral abnormalities SRBD have been found. We investigated cognition, behavior, in children with PWS. Thirty-one pre-pubertal PWS were evaluated (5 paternal deletion, 14 maternal disomy, 4 imprinting-center mutation, 8 defect was not specified). Cognition assessed Wechsler scale subtests, behavior parent-questionnaires. Polysomnography performed. Cognition, evaluated. All subtests significantly below O SDS, lowest median (interquartile range) scores for Block design subtest (-2.7 SDS (-3.0 to -0.3)). 60%, verbal less affected than performance subtests. Parents reported problem related "emotions/behavior adapted social situation" "insensitivity information." had SRBD, an Apnea Hypopnea Index of 4.1/hr (2.6-7.9). One score higher better sleep efficiency, daytime sleepiness associated more autistic-like impairment. contrast our expectations, worse breathing. children, cognition impaired. common, particularly Sleep efficiency on one sleepiness. contrast, we could confirm a positive association