Narcolepsy in childhood

Nevšímalová, Soňa

doi:10.1016/j.smrv.2008.04.007

Cited by 95 publications

(121 citation statements)

References 61 publications

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“…[6][7][8][9] Studies in the 1980s-1990s reported a median delay > 10 years between onset and diagnosis, 10 whereas a more recent report suggests the delay can be only a few years, particularly in children. 11 A focus 271 patients was defined either by the presence of documented hypocretin deficiency (n = 47; CSF hypocretin-1 ≤ 110 pg/ mL) or, if hypocretin deficiency was not documented through CSF studies (CSF hypocretin-1 unavailable), by the presence of clear cataplexy and HLA-DQB1*0602. Using this definition, approximately 98% of cases included are predicted to have hypocretin deficiency 4 if CSF hypocretin-1 had been measured in all subjects.…”

Section: Introductionmentioning

confidence: 99%

Presentations of Primary Hypersomnia in Chinese Children

Han

et al. 2011

Sleep

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627Childhood Narcolepsy/Hypersomnia-Han et al on the pediatric population could help address issues related to puberty, genetic predisposition, and environmental triggers, and possibly shed light on factors that might delay diagnosis. We report on findings from a pediatric cohort of patients who presented with primary hypersomnia to a sleep clinic in Beijing, China over a period of ~10 years. All were evaluated in a standard fashion and classified as narcolepsy with or without cataplexy or idiopathic hypersomnia. Our purpose was to describe age of onset and clinical features of primary hypersomnia among a mainland Chinese cohort of children, and to compare the findings to the literature. METHODS PatientsPatients were children, defined as < 18 years of age, successively presenting with hypersomnia seen over a period of ~10 years (September, 1998 to May, 2009) at People's Hospital, Beijing University, Beijing. A total of 417 children (63%) were drawn from a population of 671 adults and children with hypersomnia. The hospital has a pulmonary medicine departmentbased sleep center, which sees both children and adults, and receives referrals from all of mainland China. It is estimated that 70% of the diagnosed narcolepsy patients in mainland China are seen at this center at People's Hospitals.12 A prior report focused on the impact of a narcolepsy recognition strategy in a pediatric neurology clinic, 13 whereas this report retrospectively describes a cohort with a broader symptom profile, evaluated using a stan- INTRODUCTIONNarcolepsy-cataplexy (NC) occurs worldwide. In China, there is a population prevalence of 0.034% in patients in South China presenting to sleep centers in Hong Kong 1,2 and a prevalence of 0.014% in Korean adolescents with similar clinical and biological presentations.3-5 Studies in North America and Europe estimates are similar. As the origin and mechanisms that produce narcolepsy are not well understood, comparisons of clinical presentations in collections of several characterized patient groups can inform future studies of clinical and genetic epidemiology.Narcolepsy-cataplexy is said to present in the second to third decade of life, with 70% to 80% of patients reporting onset between 10-20 years of age. Large sample size observations from the United States, Canada, Europe, and Japan report that half of adult patients with narcolepsy report narcolepsy onset prior to 15 years old, and only exceptionally at 5 or younger.6-9 Studies in the 1980s-1990s reported a median delay > 10 years between onset and diagnosis, 10 whereas a more recent report suggests the delay can be only a few years, particularly in children.11 A focus Objective: To retrospectively describe childhood presentations of primary hypersomnia with an emphasis on narcolepsy-cataplexy in a Chinese population. Methods: A total of 417 children (< 18 years old) successively presenting with complaints of hypersomnia without anatomic cause or sleep apnea risk were evaluated using the Stanford Sleep Inventory, human leukocyte antigen (HLA) ...

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Section: Introductionmentioning

confidence: 99%

Presentations of Primary Hypersomnia in Chinese Children

Han

et al. 2011

Sleep

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“…In the diagnosis, multiple sleep latency test (MSLT) and CSF hypocretin measurement are needed in addition to appropriate clinical history. Presence of HLA DQB1 0602 allele strongly supports the diagnosis (2,3).…”

Section: Introductionmentioning

confidence: 76%

“…This is thought to be related with accompanying hypothalamic dysfunction, excessive sleepiness during the day, and reduced school attendance (3,8). In the literature, it was reported that a patient who had been followed up because of hypothyroidism-related obesity was diagnosed as having narcolepsy during the follow-up, and lost weight with appropriate treatment (7).…”

Section: Discussionmentioning

confidence: 99%

“…It is thought to be related with disruption in hypocretin secretion as a result of autoimmune damage in cells that secrete hypocretin (orexin) in the lateral hypothalamus. A hypocretin level of less than 110 pg/mL in the cerebrospinal fluid (CSF) is diagnostic (3). In the diagnosis, multiple sleep latency test (MSLT) and CSF hypocretin measurement are needed in addition to appropriate clinical history.…”

Section: Introductionmentioning

confidence: 99%

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Narcolepsy and cataplexy: a pediatric case report

et al. 2016

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Narcolepsy is characterized by excessive sleepiness, cataplexy, hypnagogic hallucinations, and sleep paralysis during the rapid eye movement period of sleep. Herein, we present a boy aged eight years who was diagnosed as having narcolepsy and cataplexy about thirteen months after his first presentation. He was admitted with symptoms of daytime sleepiness. In the follow-up, cataplexy in the form of head dropping attacks developed seven months after the first admission. The patient was investigated for different prediagnoses and was eventually diagnosed as having narcolepsy and cataplexy through polysomnography and multiple sleep latency tests thirteen months after the first presentation. He is being followed up and is under drug therapy; his symptoms have improved substantially. (Turk Pediatri Ars 2016; 51: 221-3)

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“…The described fainting spells consisted of sudden jerking and loss of muscle control, usually not associated with emotion or stress, further suggesting epilepsy rather than cataplexy. The patient has a primary generalized epilepsy with an overlap syndrome between phantom absences and juvenile myoclonic epilepsy (JME 1 RBD, a parasomnia characterized by lack of electromyographic REM sleep atonia and emergence of purposeful complex motor activity associated with vivid dreams, was excluded by the sleep study. 2 The sleep study confirmed UARS.…”

Section: Sectionmentioning

confidence: 99%