Diagnosing Narcolepsy Through the Simultaneous Clinical and Electrophysiologic Analysis of Cataplexy

Dyken, Mark Eric; Yamada, Thoru; Lin-Dyken, Deborah C.; Seaba, Peter J.; Yeh, Malcolm

doi:10.1001/archneur.1996.00550050086028

Cited by 30 publications

(8 citation statements)

References 31 publications

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“…Next, we addressed the functional relevance of the HSPT discharges observed in OX ko/ko mice to human narcolepsy. Earlier reports mention the presence of slow-alpha activity mixed with theta rhythms in the EEG of patients with narcolepsy during cataplexy (Guilleminault, 1976;Dyken et al, 1996;Rubboli et al, 2000;Vetrugno et al, 2010). Moreover, cases of prominent hypersynchronous theta activity during cataplexy in children close to disease onset have recently been noticed (G.P., personal observation).…”

Section: Hypersynchronous Paroxysmal Theta Events In Mice and Humansmentioning

confidence: 95%

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Electroencephalogram paroxysmal theta characterizes cataplexy in mice and children

Vassalli

Dellepiane

Emmenegger

et al. 2013

Brain

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Astute control of brain activity states is critical for adaptive behaviours and survival. In mammals and birds, electroencephalographic recordings reveal alternating states of wakefulness, slow wave sleep and paradoxical sleep (or rapid eye movement sleep). This control is profoundly impaired in narcolepsy with cataplexy, a disease resulting from the loss of orexin/hypocretin neurotransmitter signalling in the brain. Narcolepsy with cataplexy is characterized by irresistible bouts of sleep during the day, sleep fragmentation during the night and episodes of cataplexy, a sudden loss of muscle tone while awake and experiencing emotions. The neural mechanisms underlying cataplexy are unknown, but commonly thought to involve those of rapid eye movement-sleep atonia, and cataplexy typically is considered as a rapid eye movement sleep disorder. Here we reassess cataplexy in hypocretin (Hcrt, also known as orexin) gene knockout mice. Using a novel video/electroencephalogram double-blind scoring method, we show that cataplexy is not a state per se, as believed previously, but a dynamic, multi-phased process involving a reproducible progression of states. A knockout-specific state and a stereotypical paroxysmal event were introduced to account for signals and electroencephalogram spectral characteristics not seen in wild-type littermates. Cataplexy almost invariably started with a brief phase of wake-like electroencephalogram, followed by a phase featuring high-amplitude irregular theta oscillations, defining an activity profile distinct from paradoxical sleep, referred to as cataplexy-associated state and in the course of which 1.5-2 s high-amplitude, highly regular, hypersynchronous paroxysmal theta bursts (∼7 Hz) occurred. In contrast to cataplexy onset, exit from cataplexy did not show a predictable sequence of activities. Altogether, these data contradict the hypothesis that cataplexy is a state similar to paradoxical sleep, even if long cataplexies may evolve into paradoxical sleep. Although not exclusive to overt cataplexy, cataplexy-associated state and hypersynchronous paroxysmal theta activities are highly enriched during cataplexy in hypocretin/orexin knockout mice. Their occurrence in an independent narcolepsy mouse model, the orexin/ataxin 3 transgenic mouse, undergoing loss of orexin neurons, was confirmed. Importantly, we document for the first time similar paroxysmal theta hypersynchronies (∼4 Hz) during cataplexy in narcoleptic children. Lastly, we show by deep recordings in mice that the cataplexy-associated state and hypersynchronous paroxysmal theta activities are independent of hippocampal theta and involve the frontal cortex. Cataplexy hypersynchronous paroxysmal theta bursts may represent medial prefrontal activity, associated in humans and rodents with reward-driven motor impulse, planning and conflict monitoring.

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Section: Hypersynchronous Paroxysmal Theta Events In Mice and Humansmentioning

confidence: 95%

“…In humans, EEG correlates of cataplexy have remained largely elusive, although descriptions of paradoxical sleep-like, pre-paradoxical sleep-like, and wakefulness-like activities were reported (Dyken et al, 1996).…”

Section: Cataplexy As a Multi-phased Processmentioning

confidence: 99%

Electroencephalogram paroxysmal theta characterizes cataplexy in mice and children

Vassalli

Dellepiane

Emmenegger

et al. 2013

Brain

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“…Sleep Paralysis Sleep paralytic events are episodes that are reported to occur either at sleep onset (hypnagogic events) or upon arousal from sleep (hypnopompic events). 1,20,21 Sleep paralytic events have been reported to occur at least once in the entire lifetime of 40 to 50% of all normal individuals in the general population and have been reported to occur on a routine basis in 17 to 40% of all individuals diagnosed with narcolepsy. The onset of this disorder generally occurs in adolescence or during the early adult years.…”

Section: Parasomnias Usually Associated With Rem Sleepmentioning

confidence: 99%

Polysomnographic Assessment of Spells in Sleep: Nocturnal Seizures Versus Parasomnias

Dyken

Yamada²,

Lin-Dyken³

2001

Semin Neurol

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“…leptics (Duntley et al, 1995;Dyken and Yamada, 2005;Dyken et al, 1994Dyken et al, , 1996. In this study we used similar techniques to analyze a patient with classic narcolepsy.…”

Section: Discussionmentioning

confidence: 99%

REM Alpha Rhythm: Diagnostic for Narcolepsy?

Dyken¹,

Wenger²,

Yamada³

2006

Journal of Clinical Neurophysiology

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A young man, in whom narcolepsy was subsequently diagnosed, had the simultaneous onset of quadriparesis and a rapid eye movement (REM)-sleep polysomnographic pattern. During this REM-sleep pattern, a waking alpha EEG rhythm, appearing when he was asked to close his eyes, immediately attenuated when he was instructed to open his eyes, after which the REM-sleep pattern persisted. The juxtaposition of REM sleep patterns and reactive alpha rhythms are likely unique to sleep paralysis and may prove valuable in diagnosing narcolepsy.

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Diagnosing Narcolepsy Through the Simultaneous Clinical and Electrophysiologic Analysis of Cataplexy

Abstract: The diagnosis of narcolepsy can be greatly enhanced by documenting cataplexy with thorough clinical assessment and demonstration of typical rapid eye movement sleep patterns during provoked spells in the course of polysomnographic monitoring sessions.

Cited by 30 publications

References 31 publications

Electroencephalogram paroxysmal theta characterizes cataplexy in mice and children

Electroencephalogram paroxysmal theta characterizes cataplexy in mice and children

Polysomnographic Assessment of Spells in Sleep: Nocturnal Seizures Versus Parasomnias

REM Alpha Rhythm: Diagnostic for Narcolepsy?

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