The MSLT is Repeatable in Narcolepsy Type 1 But Not Narcolepsy Type 2: A Retrospective Patient Study

Ruoff, Chad; Pizza, Fabio; Trotti, Lynn Marie; Šonka, Karel; Vandi, Stefano; Cheung, Joseph; Pinto, Swaroop; Einen, Mali; Simakajornboon, Narong; Han, Fang; Peppard, Paul E.; Nevšímalová, Soňa; Plazzi, Giuseppe; Rye, David B.; Mignot, Emmanuel

doi:10.5664/jcsm.6882

Cited by 81 publications

(54 citation statements)

References 44 publications

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“…Ruoff et al. () performed a study closer to ours, looking specifically at type‐1 and type 2 narcoleptics. These authors showed again a much greater variability of results at one repeated MSLT in type‐2 narcoleptics, who changed to idiopathic hypersomnia or to a negative MSLT 26% and 57% of the time, respectively.…”

Section: Discussionmentioning

confidence: 85%

Multiple sleep latency test in narcolepsy type 1 and narcolepsy type 2: A 5‐year follow‐up study

Huang

Guilleminault

Lin

et al. 2018

Journal of Sleep Research

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Excessively sleepy teenagers and young adults without sleep-disordered breathing are diagnosed with either narcolepsy type 1 or narcolepsy type 2, or hypersomnia, based on the presence/absence of cataplexy and the results of a multiple sleep latency test. However, there is controversy surrounding this nomenclature. We will try to find the differences between different diagnoses of hypersomnia from the results of the long-term follow-up evaluation of a sleep study. We diagnosed teenagers who had developed excessive daytime sleepiness based on the criteria of the International Classification of Sleep Disorders, 3rd edition. Each individual received the same clinical neurophysiologic testing every year for 5 years after the initial diagnosis of narcolepsy type 1 (n = 111) or type 2 (n = 46). The follow-up evaluation demonstrated that narcolepsy type 1 (narcolepsy-cataplexy) is a well-defined clinical entity, with very reproducible clinical neurophysiologic findings over time, whereas patients with narcolepsy type 2 presented clear clinical and test variability. By the fifth year of the follow-up evaluation, 17.6% of subjects did not meet the diagnostic criteria of narcolepsy type 2, and 23.9% didn't show any two sleep-onset rapid eye movement periods in multiple sleep latency during the 5-year follow-up. Therefore narcolepsy type 1 (narcolepsy-cataplexy) is a well-defined syndrome, with the presentation clearly related to the known consequences of destruction of hypocretin/orexin neurons. Narcolepsy type 2 covers patients with clinical and test variability over time, thus bringing into question the usage of the term "narcolepsy" to label these patients.

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Section: Discussionmentioning

confidence: 85%

Multiple sleep latency test in narcolepsy type 1 and narcolepsy type 2: A 5‐year follow‐up study

Huang

Guilleminault

Lin

et al. 2018

Journal of Sleep Research

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“…Rather, it may be another indication that the MSLT does not optimally classify hypersomnolent patients. The MSLT is normal in a substantial group of patients with symptoms of IH (Vernet & Arnulf, ) or problematic sleepiness (Pizza et al., ), and has poor test–retest reliability in several hypersomnolence disorders (Ruoff et al., ; Trotti, Staab, & Rye, ).…”

Section: Discussionmentioning

confidence: 99%

Systemic exertion intolerance disease/chronic fatigue syndrome is common in sleep centre patients with hypersomnolence: A retrospective pilot study

Maness

Saini

Bliwise

et al. 2018

Journal of Sleep Research

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Symptoms of the central disorders of hypersomnolence extend beyond excessive daytime sleepiness to include non-restorative sleep, fatigue and cognitive dysfunction. They share much in common with myalgic encephalomyelitis/chronic fatigue syndrome, recently renamed systemic exertion intolerance disease, whose additional features include post-exertional malaise and orthostatic intolerance. We sought to determine the frequency and correlates of systemic exertion intolerance disease in a hypersomnolent population. One-hundred and eighty-seven hypersomnolent patients completed questionnaires regarding sleepiness and fatigue; questionnaires and clinical records were used to assess for systemic exertion intolerance disease. Sleep studies, hypocretin and cataplexy were additionally used to assign diagnoses of hypersomnolence disorders or sleep apnea. Included diagnoses were idiopathic hypersomnia (n = 63), narcolepsy type 2 (n = 25), persistent sleepiness after obstructive sleep apnea treatment (n = 25), short habitual sleep duration (n = 41), and sleepiness with normal sleep study (n = 33). Twenty-one percent met systemic exertion intolerance disease criteria, and the frequency of systemic exertion intolerance disease was not different across sleep diagnoses (p = .37). Patients with systemic exertion intolerance disease were no different from those without this diagnosis by gender, age, Epworth Sleepiness Scale, depressive symptoms, or sleep study parameters. The whole cohort reported substantial fatigue on questionnaires, but the systemic exertion intolerance disease group exhibited more profound fatigue and was less likely to respond to traditional wake-promoting agents (88.6% versus 67.7%, p = .01). Systemic exertion intolerance disease appears to be a common co-morbidity in patients with hypersomnolence, which is not specific to hypersomnolence subtype but may portend a poorer prognosis for treatment response.

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“…As a surrogate for hypocretin deficiency, the current PSG/MSLT protocol plus cataplexy is sufficient to diagnose NT1 in most cases. The retest reliability of the PSG/MSLT for NT1 is high at 78%–81.3% [ 8 , 9 ].…”

Section: Challenges With Current Classificationmentioning

confidence: 99%

“…Thus, the second reason for lumping NT2 and IH without long sleep time springs from the poor retest reliability of MSLT for IH or NT2 diagnosis. Patients initially diagnosed with these disorders who undergo repeat testing will frequently change the diagnosis, either because of a change in SOREMs or a change in sleep latency [ 8 , 9 , 39 ]. This may be because of inconsistent MSLT testing protocols, unstable symptom characteristics, and/or that number of SOREMs on MSLT is not a stable feature of these disorders.…”

Section: International Classification Of Sleep Disorders—ideas For Thmentioning

confidence: 99%

To split or to lump? Classifying the central disorders of hypersomnolence

Fronczek

Arnulf

Baumann

et al. 2020

Sleep

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The classification of the central disorders of hypersomnolence has undergone multiple iterations in an attempt to capture biologically meaningful disease entities in the absence of known pathophysiology. Accumulating data suggests that further refinements may be necessary. At the 7th International Symposium on Narcolepsy, a group of clinician-scientists evaluated data in support of keeping or changing classifications, and as a result suggest several changes. First, idiopathic hypersomnia with long sleep durations appears to be an identifiable and meaningful disease subtype. Second, idiopathic hypersomnia without long sleep time and narcolepsy without cataplexy share substantial phenotypic overlap and cannot reliably be distinguished with current testing, and so combining them into a single disease entity seems warranted at present. Moving forward, it is critical to phenotype patients across a wide variety of clinical and biological features, to aid in future refinements of disease classification.

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The MSLT is Repeatable in Narcolepsy Type 1 But Not Narcolepsy Type 2: A Retrospective Patient Study

Cited by 81 publications

References 44 publications

Multiple sleep latency test in narcolepsy type 1 and narcolepsy type 2: A 5‐year follow‐up study

Multiple sleep latency test in narcolepsy type 1 and narcolepsy type 2: A 5‐year follow‐up study

Systemic exertion intolerance disease/chronic fatigue syndrome is common in sleep centre patients with hypersomnolence: A retrospective pilot study

To split or to lump? Classifying the central disorders of hypersomnolence

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