The burden of narcolepsy with cataplexy: How disease history and clinical features influence socio-economic outcomes

Ingravallo, Francesca; Gnucci, Valentina; Pizza, Fabio; Vignatelli, Luca; Govi, Annamaria; Dormi, Ada; Pelotti, Susi; Cicognani, A; Dauvilliers, Yves; Plazzi, Giuseppe

doi:10.1016/j.sleep.2012.08.002

Cited by 70 publications

(47 citation statements)

References 29 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…This analysis focused on the patient perspective and highlights four main domains: (1) diagnostic delay, (2) symptom burden, (3) comorbidities associated with narcolepsy, and (4) general treatment efficacy. Given that the literature suggests duration of narcolepsy symptoms affects disease burden, 4,14 we also assessed the effect of pediatric onset (symptoms before 18 years of age) on time to diagnosis and presence of self-reported depression. We hypothesized that pediatric onset of symptoms would be associated with a diagnostic delay of greater than 1 year given the unique presentation of cataplexy symptoms children can experience and challenges in obtaining information on symptoms from children.…”

Section: Introductionmentioning

confidence: 99%

Listening to the Patient Voice in Narcolepsy: Diagnostic Delay, Disease Burden, and Treatment Efficacy

Maski

Steinhart

Williams

et al. 2017

Journal of Clinical Sleep Medicine

105

View full text Add to dashboard Cite

Study Objectives: Describe common symptoms, comorbidities, functional limitations, and treatment responsiveness among patients with narcolepsy. Investigate the effect of pediatric onset of narcolepsy symptoms on time to diagnosis of narcolepsy and presence of comorbid depression. Methods: Cross-sectional survey of 1,699 people in the United States with self-reported diagnosis of narcolepsy. We utilized mixed-methods data analyses to report study findings. Results: Most participants reported receiving a diagnosis of narcolepsy more than 1 y after symptom onset. We found that the strongest predictor of this delayed diagnosis was pediatric onset of symptoms (odds ratio = 2.4, p < 0.0005). Depression was the most common comorbidity but we detected no association with pediatric onset of narcolepsy symptoms. Overall, participants reported that fatigue and cognitive difficulties were their most burdensome symptoms in addition to sleepiness and cataplexy. The majority of participants reported residual daytime fatigue and/or sleepiness despite treatment. Most participants reported they could not perform at work or school as well as they would like because of narcolepsy symptoms. Conclusions: This study provides unique insight into the narcolepsy disease experience. The study quantifies the problem of diagnostic delay for narcolepsy patients in the United States and highlights that symptoms are more likely to be missed if they develop before 18 y of age. These results suggest that narcolepsy awareness efforts should be aimed at parents, pediatric health care providers, school professionals, and children/adolescents themselves. Disease burden is high because of problems with fatigue, cognition, and persistence of residual symptoms despite treatment.

show abstract

Section: Introductionmentioning

confidence: 99%

Listening to the Patient Voice in Narcolepsy: Diagnostic Delay, Disease Burden, and Treatment Efficacy

Maski

Steinhart

Williams

et al. 2017

Journal of Clinical Sleep Medicine

105

View full text Add to dashboard Cite

show abstract

“…Considering the heterogeneous disease course as described above, determining the long-term (or even short-term) handicap caused by narcolepsy is challenging, and the suitable monetary compensation is difficult to determine if it is based on individual consideration. Nonetheless, narcolepsy causes marked social and economical burden for patients and their close relatives, in addition to deleterious effects which may occur from health-related reductions in quality of life [16,17].…”

Section: Introductionmentioning

confidence: 99%

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

“…Excessive daytime sleepiness (EDS) is a remarkable finding in patients with narcolepsy that determines occupational and personal impact [1]. The current literature shows that sleep disturbances have negative effects on attention, vigilance, learning, and memory.…”

Section: To the Editormentioning

confidence: 99%

Deficits of attention and cognition in narcoleptic patients – is it hypocretin dependent?

Iatallese¹,

Cremaschi²,

Carvalho³

et al. 2015

Sleep Medicine

View full text Add to dashboard Cite

The burden of narcolepsy with cataplexy: How disease history and clinical features influence socio-economic outcomes

Cited by 70 publications

References 29 publications

Listening to the Patient Voice in Narcolepsy: Diagnostic Delay, Disease Burden, and Treatment Efficacy

Listening to the Patient Voice in Narcolepsy: Diagnostic Delay, Disease Burden, and Treatment Efficacy

Clinical course of H1N1-vaccine-related narcolepsy

Deficits of attention and cognition in narcoleptic patients – is it hypocretin dependent?

Contact Info

Product

Resources

About