The Natural History of Health and Symptoms in Narcolepsy: A to-year Longitudinal Study

Bruck, Dorothy; Costa, Andréa Suzana Vieira

doi:10.1071/py03007

Cited by 10 publications

(6 citation statements)

References 21 publications

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“…Three studies have evaluated the longitudinal evolution of narcolepsy symptoms; however, they were limited by the retrospective nature of the research 10–12. While some reported no changes in daytime sleepiness,10,11 Büchele et al reported that sleepiness is not always stable, with reported spontaneous deterioration or improvement in 27% 12. To the best of our knowledge, no study has specifically and prospectively addressed the course of cataplexy over time.…”

Section: Introductionmentioning

confidence: 99%

<p>A 10-Year Longitudinal Observational Study Of Cataplexy In A Cohort Of Narcolepsy Type 1 Patients</p>

Almeneessier

Alballa²,

Alsalman³

et al. 2019

NSS

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PurposeNarcolepsy type 1 (NT1) is thought to have a chronic persistent course. This study aimed to assess the natural course of cataplexy in patients with NT1 at 2, 6, and 10 years after stabilizing symptoms. Other secondary objectives included assessing sleep quality, body mass index (BMI), and comorbidities at recruitment and 10 years later.Patients and methodsCataplexy symptoms, the Epworth sleepiness scale (ESS), sleep quality (assessed using the Pittsburgh sleep quality index [PSQI]), BMI, and comorbid conditions were prospectively monitored in 38 patients with NT1. The study sample comprised 38 patients with narcolepsy (males=27). The mean ages at disease onset and recruitment were 17.7 ± 5.6 years and 24.3 ± 8.6 years, respectively.ResultsIn 42% of the cohort, the anti-cataplectic medications were stopped at the end of the study without disturbing symptoms of cataplexy. Additionally, there was an apparent significant reduction in the frequency of cataplexy over time. The mean ESS score decreased by more than 4 points from 19.4 ± 2.9 to 15 ± 4.3 (p<0.001) while on the same pharmacotherapy. The number of patients with a PSQI score of <5 (indicating good sleep quality) increased from 6 (15.8%) to 15 (39.5%) (p=0.004). The BMI increased from 30 ± 5.1 to 33.3 ± 6 kg/m2 (p=0.001). No changes were documented in comorbidities.ConclusionThe findings suggest that the course of NT1 is not stable. Over a 10-year period, cataplexy symptoms improved or disappeared in a large proportion of patients, and there was an improvement in daytime sleepiness and nighttime sleep quality. More prospective studies that repeatedly monitor CSF-HCRT are needed to confirm the current findings.

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Section: Introductionmentioning

confidence: 99%

<p>A 10-Year Longitudinal Observational Study Of Cataplexy In A Cohort Of Narcolepsy Type 1 Patients</p>

Almeneessier

Alballa²,

Alsalman³

et al. 2019

NSS

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“…9 More recently, impaired cognition, inattention, and a high prevalence of mood disorders have been reported in cross-sectional studies of patients with narcolepsy, suggesting a broad range of comorbidities that affect quality of life and daytime functionality. 10,11 To gain better understanding of the narcolepsy disease experience for future drug development, the United States Food and Drug Administration (FDA) held a public meeting entitled "Narcolepsy Patient-Focused Drug Development" on September 24, 2014 with narcolepsy advocacy groups and patients with narcolepsy.…”

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

111

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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.

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“…As a lifelong disease, narcolepsy was reported to destroy the stability of the cortex and induced a decline in attention, reaction, executive ability and cognitive function (19)(20)(21)(22). As a significant number of patients have the disease onset in adolescents, which is considered as the golden period for learning in one's life (23,24), it was important to find the potential influence on school/work performance.…”

Section: Discussionmentioning

confidence: 99%

The Impact of Sleep Pattern in School/Work Performance During the COVID-19 Home Quarantine in Patients With Narcolepsy

et al. 2022

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ObjectivesNarcolepsy patients were observed improvements in their academic performance during the COVID-19 home quarantine. Therefore, we aim to investigate the influence of sleep behavioral changes on school/work performance in narcolepsy patients during the home quarantine.MethodsPatients admitted to Shandong Provincial Qianfoshan Hospital from Jan 1, 2017 to Jan 1, 2021 who were diagnosed with narcolepsy were studied by online questionnaires in two different periods (during and 1 year after the COVID-19 home quarantine), including five aspects: (1) changes in school/work performance (percentile ranking in class/Sheehan Disability Scale 1, SDS1); (2) daytime functions; (3) clinical symptoms; (4) psychological moods; (5) medication situations.ResultsA total of 46 narcolepsy patients 34 (73.9%) narcolepsy type 1, 12 (26.1%) narcolepsy type 2 with average age of 20.76 ± 8.99 years and an equal number of age and gender matched control subjects were enrolled. During the COVID-19 home quarantine, the narcolepsy patients were found that they altered sleep patterns, including later get up time (P < 0.001), longer total sleep time (TST, P = 0.001), better sleep quality (PSQI, P = 0.001), and lower anxiety level (P = 0.005). Their school/work performance improved parallelly [with better percentile ranking (P = 0.001) and lower SDS1 scores (P = 0.002)]. The results of multiple linear stepwise regression analysis showed a linear regression relationship between TST [efficient (95%) −7.356 (−13.570 to 1.143)], SDS1 score [efficient (95%) 6.580 (2.346–10.815), P = 0.004] and the percentile ranking after adjusting for potential effects. Both the improvements of sleep behavior and school/work performance disappeared after the end of COVID-19 home quarantine. No similar fluctuation was found in the control group.DiscussionChanges in sleep pattern during the COVID-19 home quarantine, such as longer sleep time and later wake-up time, can reduce the degree of daytime sleepiness and increase the degree of daytime wakefulness of narcolepsy patients, which can alleviate the impact of the disease on school/work performance.

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The Natural History of Health and Symptoms in Narcolepsy: A to-year Longitudinal Study

Cited by 10 publications

References 21 publications

<p>A 10-Year Longitudinal Observational Study Of Cataplexy In A Cohort Of Narcolepsy Type 1 Patients</p>

<p>A 10-Year Longitudinal Observational Study Of Cataplexy In A Cohort Of Narcolepsy Type 1 Patients</p>

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

The Impact of Sleep Pattern in School/Work Performance During the COVID-19 Home Quarantine in Patients With Narcolepsy

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