High Prevalence of Precocious Puberty and Obesity in Childhood Narcolepsy with Cataplexy

Poli, Francesca; Pizza, Fabio; Mignot, Emmanuel; Ferri, Raffaele; Pagotto, Uberto; Taheri, Shahrad; Finotti, Elena; Bernardi, Filippo; Pirazzoli, Piero; Cicognani, Alessandro; Balsamo, Antonio; Nobili, Lino; Bruni, Oliviero; Plazzi, Giuseppe

doi:10.5665/sleep.2366

Cited by 129 publications

(86 citation statements)

References 43 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…It has been previously reported that obesity is very common in narcolepsy [4]. BMI increase was clear also in our study while the majority of the subjects still stayed within the normal range.…”

Section: Discussionsupporting

confidence: 71%

“…The main symptoms of narcolepsy are excessive daytime sleepiness, disturbed nocturnal sleep, cataplexy, sleep paralyses, and hallucinations during sleep-wake transitions. Other common associated features are increased weight, precocious puberty, behavioral problems, and psychiatric comorbidity [4][5][6]. There is a great heterogeneity in symptoms as some subjects are severely affected and disabled, whereas others manage without medication.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Clinical course of H1N1-vaccine-related narcolepsy

2016

View full text Add to dashboard Cite

Objective: To follow and analyze the clinical course and quality of life of Pandemrix H1N1-vaccinerelated narcolepsy (pNT1). Methods: Twenty-six drug-naïve confirmed pNT1 subjects completed Epworth Sleepiness Scale (ESS), Ullanlinna Narcolepsy Scale (UNS), Swiss Narcolepsy Scale (SNS), Rimon's Brief Depression scale (RDS), and WHO-5 Well-being index questionnaires near the disease onset and in a follow-up a minimum of two years later. The number of cataplexies and body mass index (BMI) were recorded. The effects of hypocretin-1 levels and sleep recording results were analyzed. The findings at the follow-up visit were compared with 25 non-vaccine-related type 1 narcolepsy (NT1) subjects. Results: In pNT1, RDS score decreased significantly (mean 10.2, SD 4.7 vs mean 6.7, SD 4.5, p = 0.003). Median of BMI increased from 20.8 kg m −2 to 23.4 kg m −2 , p < 0.001. There were no significant differences in other sleep scores. However, deviation and range in questionnaire scores at the follow-up were wide. Subjects with very low or undetectable hypocretin-1 levels had worse scores in UNS (mean 26.4, SD 6.95 vs mean 19.1, SD 3.83, p = 0.006) and ESS (mean 17.9, SD = 4.29 vs mean 14.1, SD = 3.70, p = 0.047) than those with hypocretin-1 levels of 20-110 pg/mL. Most disabling symptoms were excessive daytime sleepiness and disturbed sleep. There were no significant differences between the scores in pNT1 and NT1. Conclusions: Clinical course of pNT1 is heterogeneous but the evolution of pNT1 seems similar to NT1. Lower hypocretin levels in pNT1 are associated with a more severe phenotype.

show abstract

Section: Discussionsupporting

confidence: 71%

Section: Introductionmentioning

confidence: 99%

Clinical course of H1N1-vaccine-related narcolepsy

2016

View full text Add to dashboard Cite

show abstract

“…OSA is a comorbidity that is related to increased weight and obesity, has a high prevalence in narcolepsy [106][107][108], and can lead to a lack of narcolepsy recognition [27,109]. Central sleep apnea and OSA syndromes should be excluded before prescribing sodium oxybate, and sleep apnea treated prior to initiating sodium oxybate therapy.…”

Section: Treatment-related Exacerbation Of Comorbiditiesmentioning

confidence: 97%

Clinical and practical considerations in the pharmacologic management of narcolepsy

Thorpy¹,

Dauvilliers²

2015

Sleep Medicine

109

110

View full text Add to dashboard Cite

Despite published treatment recommendations and the availability of approved and off-label pharmacologic therapies for narcolepsy, the clinical management of this incurable, chronic neurologic disorder remains challenging. While treatment is generally symptomatically driven, decisions regarding which drug(s) to use need to take into account a variety of factors that may affect adherence, efficacy, and tolerability. Type 1 narcolepsy (predominantly excessive daytime sleepiness with cataplexy) or type 2 narcolepsy (excessive daytime sleepiness without cataplexy) may drive treatment decisions, with consideration given either to a single drug that targets multiple symptoms or to multiple drugs that each treat a specific symptom. Other drug-related characteristics that affect drug choice are dosing regimens, tolerability, and potential drug-drug interactions. Additionally, the patient should be an active participant in treatment decisions, and the main symptomatic complaints, treatment goals, psychosocial setting, and use of lifestyle substances (ie, alcohol, nicotine, caffeine, and cannabis) need to be discussed with respect to treatment decisions. Although there is a lack of narcolepsy-specific instruments for monitoring therapeutic effects, clinically relevant subjective and objective measures of daytime sleepiness (eg, Epworth Sleepiness Scale and Maintenance of Wakefulness Test) can be used to provide guidance on whether treatment goals are being met. These considerations are discussed with the objective of providing clinically relevant recommendations for making treatment decisions that can enhance the effective management of patients with narcolepsy.

show abstract

“…It should be noted that both these targets of pattern A autoantibodies (MCH and POMC neurons) are also involved in the regulation of food intake [20,21]. This is highly relevant in the context of narcolepsy, where a rapid increase in body weight often occurs [22], and could be associated with an increased risk of cardiovascular disease as well as a significantly impaired quality of life [23]. Thus, it could be hypothesized that patients with autoantibodies against MCH/POMC neurons would have a higher body mass index than those without such autoantibodies.…”

Section: Narcolepsy: Genetic and Immunohistochemical Studiesmentioning

confidence: 99%

A coordinated cross‐disciplinary research initiative to address an increased incidence of narcolepsy following the 2009–2010 Pandemrix vaccination programme in Sweden

et al. 2015

View full text Add to dashboard Cite

In response to the 2009-2010 influenza A(H1N1) pdm09 pandemic, a mass vaccination programme with the AS03-adjuvanted influenza A(H1N1) vaccine Pandemrix was initiated in Sweden. Unexpectedly, there were a number of narcolepsy cases amongst vaccinated children and adolescents reported. In this review, we summarize the results of a joint cross-disciplinary national research effort to investigate the adverse reaction signal from the spontaneous reporting system and to better understand possible causative mechanisms. A three-to fourfold increased risk of narcolepsy in vaccinated children and adolescents was verified by epidemiological studies. Of importance, no risk increase was observed for the other neurological and autoimmune diseases studied. Genetic studies confirmed the association with the allele HLA-DQB1*06:02, which is known to be related to sporadic narcolepsy. Furthermore, a number of studies using cellular and molecular experimental models investigated possible links between influenza vaccination and narcolepsy. Serum analysis, using a peptide microarray platform, showed that individuals who received Pandemrix exhibited a different epitope reactivity pattern to neuraminidase and haemagglutinin, as compared to individuals who were infected with H1N1. Patients with narcolepsy were also found to have increased levels of interferongamma production in response to streptococcusassociated antigens. The chain of patient-related events and the study results emerging over time were subjected to intense nationwide media attention. The importance of transparent communication and collaboration with patient representatives to maintain public trust in vaccination programmes is also discussed in the review. Organizational challenges due to this unexpected event delayed the initiation of some of the research projects, still the main objectives of this joint, cross-disciplinary research effort were reached, and important insights were acquired for future, similar situations in which a fast and effective task force may be required to evaluate vaccination-related adverse events.

show abstract

High Prevalence of Precocious Puberty and Obesity in Childhood Narcolepsy with Cataplexy

Cited by 129 publications

References 43 publications

Clinical course of H1N1-vaccine-related narcolepsy

Clinical course of H1N1-vaccine-related narcolepsy

Clinical and practical considerations in the pharmacologic management of narcolepsy

A coordinated cross‐disciplinary research initiative to address an increased incidence of narcolepsy following the 2009–2010 Pandemrix vaccination programme in Sweden

Contact Info

Product

Resources

About