Features of narcolepsy course in children
https://doi.org/10.31146/2949-4664-apps-3-1-61-67
Abstract
Narcolepsy is a severe chronic disease characterized by disorders of sleep and wakefulness centers, with a predominant violation of the REM sleep phase. Until the end of the 20th century, narcolepsy had been considered as a complex clinical and neurophysiological phenomenon with an unclear etiology, affecting almost all areas of regulation of the sleep/wake cycle. Over the past decades, the advancement of imaging and instrumental diagnostics methods has improved our knowledge of the etiology, pathogenesis, and clinical pattern of this condition. Currently, two major types of the disease are distinguished. In children’s practice, type 1 (NT1) is more common. This type is characterized by excessive daytime sleepiness, cataplexy, sleep paralysis, as well as the appearance of hallucinations and fragmentation of night sleep. Recent years have seen an increase in cases of this pathology, largely due to an improved awareness of physicians about the disease. Children diagnosed with type 1 narcolepsy exhibit a peculiar cataplexy phenotype characterized by persistent hypotension with marked changes in facial expression (cataplectic emotions) and a complex mosaic of hyperkinetic movements that are amplified during emotional exertion. These features gradually disappear as the disease progresses, leading to a typical cataplexy pattern. Children with such a diagnosis also show behavioral abnormalities and mental disorders, hyperactive/aggressive behavior, up to psychotic features. Researchers also note that type 1 narcolepsy, which occurs in children during the prepubertal period, is associated with obesity and premature sexual development, which may reflect a hypothalamic disorder secondary to a decrease in the level of the neurotransmitter hypocretin. The complexity of the phenotype of pediatric NT1 requires an interdisciplinary assessment and selection of therapy, taking the behavioral and endocrinological characteristics of the growing organism into account. The presented review article discusses the clinical pattern, diagnosis and treatment of this pathology in children’s practice.
About the Authors
R. R. Khaertdinova
Saint-Petersburg State Pediatric Medical University
Russian Federation
Russian Federation
A. Yu. Trapeznikova
Saint-Petersburg State Pediatric Medical University
Russian Federation
Russian Federation
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Review
For citations:
Khaertdinova R.R., Trapeznikova A.Yu. Features of narcolepsy course in children. Archives of Pediatrics and Pediatric Surgery. 2025;3(1):62-68. (In Russ.) https://doi.org/10.31146/2949-4664-apps-3-1-61-67
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