Grey zone between narcolepsy type 1 and type 2

Fernandes, Gustavo Bruniera Peres; Cremaschi, Renata Carvalho; Tufik, Sérgio; Coelho, Fernando Morgadinho Santos

doi:10.1590/0004-282x20170106

Cited by 3 publications

(5 citation statements)

References 4 publications

(2 reference statements)

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“…Patients with type II narcolepsy usually have levels of hypocretin-1, one greater than 200 pg/mL. 1,35 The literature should provide a more comprehensive discussion of the gray zone between 110 pg/mL and 200 pg/mL. Studies have reported cases of patients with hypocretin-1 levels within this range.…”

Section: Diagnosis and Classificationmentioning

confidence: 99%

“…While biomarkers are beneficial for identifying type I narcolepsy, identifying patients with narcolepsy type II remains challenging in many cases. 35 Unfortunately, in real life, there is a delay of over ten years for the diagnosis of narcolepsy around the world. 36 Many causes can explain this reality, such as the lack of knowledge on the disorder, incorrect diagnosis of depression, prejudice of familiar members and society about EDS, and few tools for clinicians.…”

Section: Diagnosis and Classificationmentioning

confidence: 99%

“…Patients with type II narcolepsy usually have levels of hypocretin-1, one greater than 200 pg/mL. 1 35…”

Section: Pathophysiological Hypotheses Of Narcolepsymentioning

confidence: 99%

“…While biomarkers are beneficial for identifying type I narcolepsy, identifying patients with narcolepsy type II remains challenging in many cases. 35…”

Section: Pathophysiological Hypotheses Of Narcolepsymentioning

confidence: 99%

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Narcolepsy: an interface among neurology, immunology, sleep, and genetics

Coelho

2024

Arq Neuropsiquiatr

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Narcolepsy is a primary disorder of the central nervous system resulting from genetic, environmental, and immunological interactions defined as excessive daytime sleepiness plus cataplexy, hallucinations, sleep paralysis, and sleep fragmentation. The pathophysiology is not entirely known, but the interaction among genetic predisposition, environmental exposition, and immune component with consequent hypocretin-1 deficiency is the model to explain narcolepsy type I. The mechanism of narcolepsy type II is less understood. There is a delay of over ten years for the diagnosis of narcolepsy around the world. Patients with narcolepsy have many comorbidities with a negative impact on quality of life. The treatment of narcolepsy must contain an educational approach for the family, coworkers, and patients. Scheduled naps and sleep hygiene are essential to minimize the dose of medications. Much progress has been seen in the pharmacological treatment of narcolepsy with new stimulants, different presentations of oxybate, and recent studies with orexin agonists. Narcolepsy is a rare disease that needs to be more understood and highlighted to avoid delayed diagnosis and severe disabilities in patients.

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Section: Diagnosis and Classificationmentioning

confidence: 99%

Section: Diagnosis and Classificationmentioning

confidence: 99%

“…Patients with type II narcolepsy usually have levels of hypocretin-1, one greater than 200 pg/mL. 1 35…”

Section: Pathophysiological Hypotheses Of Narcolepsymentioning

confidence: 99%

“…While biomarkers are beneficial for identifying type I narcolepsy, identifying patients with narcolepsy type II remains challenging in many cases. 35…”

Section: Pathophysiological Hypotheses Of Narcolepsymentioning

confidence: 99%

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Narcolepsy: an interface among neurology, immunology, sleep, and genetics

Coelho

2024

Arq Neuropsiquiatr

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“…Subsequent studies have implemented these cutoffs [ 9 , 10 ], as has the ICSD3 [ 3 , 11 ]. Consequently, an intermediate CSF hypocretin-1 range of 111–200 pg/mL was created, resulting in a patient subgroup that is more difficult to diagnose unless there are clear features of typical cataplexy and positive MSLT and PSG findings [ 12 ]. Moreover, at the time these hypocretin-1 cutoff values were determined, the criteria for the differential diagnosis of NT1 and narcolepsy type 2 (NT2) were not yet established, as narcolepsy was then classified as narcolepsy with or without cataplexy [ 8 ] We need to characterize individuals with intermediate CSF hypocretin-1 levels, by re-examining the current hypocretin-1 cutoff value.…”

Section: Introductionmentioning

confidence: 99%

Intermediate hypocretin-1 cerebrospinal fluid levels and typical cataplexy: their significance in the diagnosis of narcolepsy type 1

Hoeven

Fronczek

Schinkelshoek

et al. 2022

Sleep

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Study Objectives The diagnosis of narcolepsy type 1 (NT1) is based upon the presence of cataplexy and/or a cerebrospinal fluid (CSF) hypocretin-1/orexin-A level ≤110 pg/mL. We determined the clinical and diagnostic characteristics of patients with intermediate hypocretin-1 levels (111-200 pg/mL) and the diagnostic value of cataplexy characteristics in individuals with central disorders of hypersomnolence. Methods Retrospective cross-sectional study of 355 people with known CSF hypocretin-1 levels who visited specialized Sleep-Wake Centers in the Netherlands. For n=271, we had full data on cataplexy type (‘typical’ or ‘atypical’ cataplexy). Results Compared to those with normal hypocretin-1 levels (>200 pg/mL), a higher percentage of individuals with intermediate hypocretin-1 levels had typical cataplexy (75% or 12/16 vs 9% or 8/88, p<.05), and/or met the diagnostic polysomnographic (PSG) and Multiple Sleep Latency Test (MSLT) criteria for narcolepsy (50 vs 6%, p<.001). Of those with typical cataplexy, 88% had low, 7% intermediate, and 5% normal hypocretin-1 levels (p<.001). Atypical cataplexy was also associated with hypocretin deficiency but to a lesser extent. A hypocretin-1 cutoff of 150 pg/mL best predicted the presence of typical cataplexy and/or positive PSG and MSLT findings. Conclusions Individuals with intermediate hypocretin-1 levels or typical cataplexy more often have outcomes fitting the PSG and MSLT criteria for narcolepsy than those with normal levels or atypical cataplexy. In addition, typical cataplexy has a much stronger association with hypocretin-1 deficiency than atypical cataplexy. We suggest increasing the NT1 diagnostic hypocretin-1 cutoff and adding the presence of clearly-defined typical cataplexy to the diagnostic criteria of NT1.

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