![]() ![]() Secondary narcolepsy can occur due to structural lesions affecting the hypothalamus and/or brainstem, perhaps via disruption of orexin signaling. Genetic susceptibility and immune dysregulation likely play essential roles in narcolepsy type 1. There are no studies that have examined the relative proportions of type 1 and type 2 narcolepsy in children.ĮTIOLOGY - In the majority of cases, narcolepsy in children is sporadic and of unknown etiology. The overall prevalence of narcolepsy in Western countries has been estimated at 20 to 50 cases per 100,000. (See "Management and prognosis of narcolepsy in children" and "Clinical features and diagnosis of narcolepsy in adults" and "Treatment of narcolepsy in adults".)ĮPIDEMIOLOGY - The epidemiology of narcolepsy in children is not well characterized.Ī European study estimated a pooled incidence rate of 0.83 per 100,000 person-years in children aged 5 to 19 years. Management in children, and diagnosis and treatment of narcolepsy in adults, are reviewed separately. This topic will review the clinical features, diagnosis, and management of narcolepsy in children. ![]() When narcolepsy starts in childhood, which it commonly can, it can have several unique features, including atypical cataplexy characterized by facial hypotonia or positive motor signs, precocious puberty, rapid unexplained weight gain, and daytime sleepiness manifesting primarily as habitual napping or irritability and hyperactivity. ![]() In children, narcolepsy is often overlooked as a cause of disabling sleepiness, and delays in the diagnosis are common. Narcolepsy type 2, or narcolepsy without cataplexy, shares all of the features of narcolepsy type 1 except cataplexy and low CSF orexin. Narcolepsy type 1, previously called narcolepsy with cataplexy, includes cataplexy as one of the earliest symptoms and is associated with low cerebrospinal fluid (CSF) orexin (also called hypocretin) levels. Narcolepsy has two forms in both children and adults, depending on whether cataplexy is present. INTRODUCTION - Narcolepsy is a chronic neurologic disorder characterized by excessive and irresistible sleepiness, cataplexy (episodes of muscle weakness during periods of strong emotion), hypnagogic hallucinations (vivid dreams at sleep onset), and sleep paralysis (a momentary inability to move the body as one is drifting off to sleep or upon waking up). ![]()
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