Eduard Cesari, Amaia Muñoz-Lopetegi, Joan Santamaria, Alex Iranzo, Carles Gaig
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Brunner syndrome is a recessive X-linked disorder characterized by intellectual disability and impulsive aggressiveness associated with monoamine oxidase A (MAOA) deficiency leading to increased monoaminergic activity. We report the presence of rapid eye movement (REM) sleep behavior disorder in a 46-year-old patient with Brunner syndrome due to a c.1438A > G/iVS14-2 A > G mutation of the MAOA gene. He has mild intellectual disability and psychotic disturbances. He presented a 15-year history of nightmares (chase, attacks, and fights), sleep-related vocalizations, and motor behaviors characterized by talking, screaming, crying, gesturing, punching, and kicking. Video polysomnography showed REM sleep behavior disorder characterized by excessive tonic and phasic muscle activity in the mentalis and limb muscles with dream-enacting behaviors during REM sleep. Clonazepam achieved a significant reduction of REM sleep behavior disorder symptomatology. We conclude that REM sleep behavior disorder can be a manifestation of Brunner syndrome, probably due to an increased monoaminergic neurotransmission occurring in this rare genetic disorder.
Citation: Cesari E, Muñoz-Lopetegi A, Santamaria J, Iranzo A, Gaig C. REM sleep behavior disorder in Brunner syndrome. J Clin Sleep Med. 2024;20(9):1557-1560.
期刊介绍:
Journal of Clinical Sleep Medicine focuses on clinical sleep medicine. Its emphasis is publication of papers with direct applicability and/or relevance to the clinical practice of sleep medicine. This includes clinical trials, clinical reviews, clinical commentary and debate, medical economic/practice perspectives, case series and novel/interesting case reports. In addition, the journal will publish proceedings from conferences, workshops and symposia sponsored by the American Academy of Sleep Medicine or other organizations related to improving the practice of sleep medicine.