Psychology
Narcolepsy
Narcolepsy is a neurological disorder characterized by excessive daytime sleepiness, sudden muscle weakness, and sometimes hallucinations and sleep paralysis. People with narcolepsy may experience sudden, uncontrollable episodes of falling asleep during the day, even in the midst of activities. It is often caused by a deficiency in the brain chemical hypocretin, which regulates wakefulness and REM sleep.
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12 Key excerpts on "Narcolepsy"
Available until 4 Dec |Learn more- S. R. Pandi-Perumal, Rocco R Ruoti, Milton Kramer, S. R. Pandi-Perumal, Rocco R Ruoti, Milton Kramer(Authors)
- 2007(Publication Date)
- CRC Press(Publisher)
191 14 Narcolepsy: Psychosocial, socioeconomic, and public health considerations M Goswami, SR Pandi-Perumal SYMPTOMS OF Narcolepsy The earliest reported description of Narcolepsy appears to be that recorded by Oliver in 1704. 1 Following reports of cases with symptoms of Narcolepsy by Graves in 1851, 2 Caffé in 1862, 3 and Westphal in 1877, 4 it was Gelineau in 1880 5 who ascribed the term narcolepsie to describe a condition characterized by brief episodes of irre-sistible sleep and by falls ( astasias ) associated with emotional stimuli. Narcolepsy is a chronic debilitating neurologic disorder, the hallmarks of which are hypersomnia and cataplexy (a sudden and transient decrement of muscle tone and loss of deep tendon reflexes, leading to muscle weakness, paralysis, or postural collapse, usually in response to an external stimulus). 6 Persons with Narcolepsy often have intrusion of rapid eye movement (REM) sleep into wakefulness. Characteristically, patients report relief of discom-fort from episodes of sleep attacks upon taking short naps. Cataplectic attacks, another form of REM intrusion, are usually evoked by a strong or deeply felt outburst of intense emotional expression such as laughter, joy, anger or fright, surprise, amuse-ment, or excitement. 7,8 Yoss and Daly 9 described a classic tetrad of symptoms for the diagnosis of Narcolepsy: excessive daytime somnolence (EDS – which is usually the first symptom), cataplexy (70–80%), hypnagogic or hypnopompic halluci-nations (which can occur just prior to sleep onset or upon arousal (70%)), and sleep paralysis (loss of muscle tone at sleep onset or on awakening (60%)). However, each of these symptoms can appear at various stages of the disorder and in different com-binations and degrees of severity. 8 Cataplexy is characterized by a sudden bilateral loss of postural muscle tone – weakness with loss of deep tendon reflex often occurs in response to emotional triggers.
eBook - ePub- S.R. Pandi-Perumal, Meera Narasimhan, Milton Kramer, S.R. Pandi-Perumal, Meera Narasimhan, Milton Kramer(Authors)
- 2017(Publication Date)
- CRC Press(Publisher)
7 Narcolepsy and psychosomatic illnesses MEETA GOSWAMI, KENNETH BUTTO, AND SEITHIKURIPPU R. PANDI-PERUMAL- The Symptoms of Narcolepsy
- Diagnosis
- Pathophysiology
- Prevalence
- Disease Onset
- Treatment (Non-Pharmacological and Pharmacological)
- Impact of Narcolepsy on health and Welfare: Quality of Life Issues
- Needs of Persons With Narcolepsy
- Is Narcolepsy a Disability Or a Developmental Disability?
- Implications for Patients, Family, and Community
- References
The Symptoms of Narcolepsy
The earliest reported description of Narcolepsy appears to be recorded by Oliver in 1704.1 Following reports of cases with symptoms of Narcolepsy by Graves in 1851,2 Caffé in 1862,3 and Westphal in 1877,4 it was Gelineau in 18815 who ascribed the term narcolepsie to describe a condition characterized by brief episodes of irresistible sleep and by falls (astasias ) associated with emotional stimuli.Narcolepsy is a chronic, debilitating neurological disorder, the hallmarks of which are hypersomnia and cataplexy (a sudden and transient decrement of muscle tone and loss of deep tendon reflexes, leading to muscle weakness, paralysis, or postural collapse, usually in response to an external stimulus).6 Persons with Narcolepsy often have rapid eye movement (REM) sleep intrusion into wakefulness. Characteristically, patients report relief of discomfort from episodes of sleep attacks upon taking short naps. Cataplectic attacks— another form of REM intrusion—are usually evoked by a strong or deeply felt outburst of intense emotional expression such as laughter, joy, anger or fright, surprise, amusement or excitement.7 ,8 Yoss and Daly9 described a classic tetrad of symptoms for the diagnosis of Narcolepsy: excessive daytime somnolence (EDS), which is usually the first symptom; cataplexy (70%–80%); hypnagogic or hypnopompic hallucinations (which can occur just prior to sleep onset or upon arousal) (70%); and sleep paralysis (loss of muscle tone at sleep onset or on awakening) (60%). However, each of these symptoms can appear at various stages of the disorder and in different combinations and degrees of severity.8
eBook - PDFAM:STARs Sleep and Sleep Disorders in Adolescents
Adolescent Medicine: State of the Art Reviews, Volume 21, Number 3
- Amy E. Sass, David W. Kaplan, Amy E. Sass, David W. Kaplan(Authors)
- 2010(Publication Date)
- American Academy of Pediatrics(Publisher)
Narcolepsy in Adolescents Shannon S. Sullivan, MD* Stanford University School of Medicine; Stanford Sleep Disorders Clinic, 450 Broadway, M/C 5704, Redwood City, CA 94063 The evaluation and treatment of the sleepy adolescent can be daunting. Sleepi-ness might be due to poor sleep habits, substance use, or underlying medical or psychiatric conditions. This article reviews recent advances in our understanding of Narcolepsy pathophysiology, diagnosis, and treatment. Narcolepsy with cataplexy is a chronic neurologic disorder in which the bound-aries between the awake, sleeping, and dreaming are blurred. It is characterized by excessive daytime sleepiness, disturbed nocturnal sleep, and cataplexy, as well as pathologic manifestations related to rapid eye movement (REM) sleep, such as hypnagogic/hypnopompic hallucinations and sleep paralysis. The human form of this disease is caused by the loss of 70 000 or so hypothalamic neurons that produce the neuropeptide hypocretin or orexin. 1,2 Both sporadic and familial forms exist in humans, with the sporadic form much more common (95%). 3 The disease is associated with human leukocyte antigen (HLA) allele DQB1*0602. 4–6 Like other HLA-associated diseases, the cause of Narcolepsy is hypothesized to be autoimmune, which is supported by recent work showing that hypocretin deficiency is highly associated with certain T-cell receptor -polymorphisms and that streptococcus infections are a possible trigger for Narcolepsy. 7,8 CLINICAL CHARACTERISTICS The cardinal features of Narcolepsy-cataplexy are daytime somnolence, cata-plexy, sleep paralysis, and hypnagogic hallucinations. 9 While the first convincing clinical descriptions of Narcolepsy occurred well over 100 years ago, for most practitioners, Narcolepsy is a relatively rare occurrence in the clinic and it is worthwhile to discuss the details of these symptoms.
eBook - PDF- Nathaniel F. Watson, Bradley V. Vaughn, Nathaniel F. Watson, Bradley V. Vaughn(Authors)
- 2006(Publication Date)
- CRC Press(Publisher)
7 Narcolepsy and Disorders of Excessive Sleepiness Nathaniel F. Watson Department of Neurology, University of Washington School of Medicine, Seattle, Washington, U.S.A. Symptoms Diagnoses Sleepiness Narcolepsy Cataplexy Idiopathic hypersomnia Sleep paralysis Hypersomnia due to medical condition (posttraumatic hypersomnia) Hallucinations Fatigue Automatic behavior Recurrent hypersomnia Kleine–Levin syndrome Menstrual-related hypersomnia Behaviorally induced insufficient sleep syndrome 209 INTRODUCTION Excessive sleepiness is pervasive in our society affecting up to 13% of the population (1). Sleepiness is experienced as a subjective difficulty maintaining alertness accompanied by a rapid entrance into sleep when the person is seden-tary. Often this leads to decrements in quality-of-life, cognitive impairment, and increased accidents (2,3). Each year in the United States greater than 50,000 motor vehicle accidents are attributed to driving while drowsy (4). Slee-piness has many causes including insufficient sleep, sleep disorders, medical and neurological illness, and medication side effects. This chapter focuses on sleep disorders where excessive sleepiness is the primary symptom; this includes Narcolepsy (sporadic and secondary), idiopathic hypersomnia, hyper-somnia due to medical conditions (posttraumatic hypersomnia), recurrent hypersomnia, and behaviorally induced insufficient sleep syndrome. Narcolepsy is a nonprogressive sleep disorder of unknown etiology with well-characterized clinical features. Sleepiness is the hallmark of the dis-ease, along with rapid eye movement (REM) sleep phenomena that intrude into wakefulness such as cataplexy, hypnagogic (occurring at sleep onset) hal-lucinations, and sleep paralysis. These symptoms comprise the ‘‘narcoleptic tetrad’’ and are experienced by patients to different degrees (5). Disturbed nocturnal sleep and automatic behavior are other common symptoms of nar-colepsy.
eBook - PDFSleep Disorders
Diagnosis and Therapeutics
- S. R. Pandi-Perumal, Joris Verster, Jaime Monti, Salomon Langer, S. R. Pandi-Perumal, Joris Verster, Jaime Monti, Salomon Langer(Authors)
- 2008(Publication Date)
- CRC Press(Publisher)
INTRODUCTION Sleep disorders are generally associated with increased tiredness and sleepiness during the daytime. 1 In recent years, research efforts have been aimed at elucidating potential cognitive changes accompanying various sleep disorders. One major issue of interest relates to the question of whether cognitive dysfunction is sim-ply caused by increased sleepiness or whether there are other underlying determinants linked to the sleep disorder. The aim of this chapter is to provide an overview on the available evidence for cognitive dys-function in three different sleep disorders, which have been most comprehensively studied in recent years: Narcolepsy, insomnia, and obstructive sleep apnea (OSA). The literature on cognitive deficits associated with each disorder will be summarized and discussed, followed by a comparison of the neuropsychological profiles of the three disorders, with special emphasis on the potential underlying neurobiological and psy-chological mechanisms. Narcolepsy Excessive daytime sleepiness is the most prominent symptom of Narcolepsy. Typically, patients have two or more sleep attacks during the day. These attacks can occur anytime, independent of what the sufferer is currently engaged in (e.g. working, eating, etc.). Sleepiness, cataplexy, and sleep paralysis accompanied by hallucinations together constitute the ‘narcoleptic tetrad’. 2 Cataplexy refers to a sudden reduction or complete loss of skeletal muscle tone that can affect single body parts or even the whole body. During these attacks, which are frequently triggered by emo-tional experiences and may last several minutes, the patients are fully conscious. Sleep paralysis is a similar symptom. As in cataplexy, the Narcolepsy sufferer is awake and unable to move, but sleep paralysis occurs only during periods of falling asleep or waking up. This condition can last up to 10 minutes and is often accompanied by, mostly visual, hallucinations, which may also occur independently.
eBook - PDF- Lois E. Krahn, Michael H. Silber, Timothy I. Morgenthaler, Lois E. Krahn, Michael H. Silber, Timothy I. Morgenthaler(Authors)
- 2010(Publication Date)
- CRC Press(Publisher)
7 Narcolepsy Michael H. Silber, MBChB, FCP (SA) ABBREVIATIONS CSF, cerebrospinal fluid hcrt, hypocretin HLA, human leukocyte antigen REM, rapid eye movement Narcolepsy has been recognized as a distinct condition for 130 years. First described by the German neuropsychiatrist Karl Friedrich Otto Westphal and the French physician Jean Baptiste Ge ´lineau, Narcolepsy is the classic example of a disor-der of excessive sleep (1). It also illustrates how advances in the knowledge of sleep physiology have led to dramatic insights into the pathogenesis of a sleep disorder. Parallel with the understanding of the mechanisms of the disease has come the development of new medications for its treatment. The prevalence of Narcolepsy in various American and European studies ranges from 26 to 67 per 100,000 population (2), indicating that one might expect to find about 400 persons with Narcolepsy in an area with a population of 1 million at any one time. The incidence rate of Narcolepsy has been calculated as 1.4 per 100,000 population per year, which translates to the diagnosis of 14 new patients each year in a population of 1 million (3). The disorder most frequently commences between the ages of 10 and 20 years, and rarely starts after age 40 (Figure 1). It is slightly more common in men than in women (1.5:1) (3). CLINICAL FEATURES The essential clinical characteristic of Narcolepsy is excessive sleepiness. Although this symptom is not specific for the dis-order, the sleepiness inherent to Narcolepsy is usually more severe than that in many other conditions causing hypersom-nia. Persons with Narcolepsy can fall asleep in any sedentary situation, such as while reading, studying, watching television, or sitting in an audience. Driving can be especially problematic because persons with Narcolepsy may fall asleep while stopped at red traffic lights and frequently experience near-accidents.
eBook - PDFSleep in Children
Developmental Changes in Sleep Patterns, Second Edition
- Carole Marcus, John L. Carroll, David Donnelly, Gerald M. Loughlin, Carole Marcus, John L. Carroll, David Donnelly, Gerald M. Loughlin(Authors)
- 2008(Publication Date)
- CRC Press(Publisher)
10 Narcolepsy in Childhood SURESH KOTAGAL Mayo Clinic, Rochester, Minnesota, U.S.A. I. Introduction Narcolepsy is a life-long neurological disorder of rapid eye movement (REM) sleep in which there are attacks of irresistible daytime sleepiness, cataplexy (sudden loss of muscle control in the legs or neck in response to emotional triggers like laughter, fright, or rage, leading to head dropping or falls), hypn-agogic hallucinations (vivid and often terrifying dreams at sleep onset), and sleep paralysis (a momentary inability to move as one is drifting off to sleep). Thomas Willis can possibly be credited with the first description of nar-colepsy in the 17th century (1). He described persons with “a sleepy disposition— they eat and drink well, go abroad, take care well enough of their domestic affairs, yet whilst talking or walking, or eating, yea their mouths being full of meat, they shall nod, and unless roused by others, fall fast asleep” (1,2). The term narcolepsie was coined by Jean Baptiste Edouard Gelineau in 1880 (3). He recognized the Adapted with permission in part from: Kotagal S. Narcolepsy in Childhood. In Pediatric Sleep Medicine. Principles and Practice. Sheldon SH, Ferber R, Kryger MH (eds), Elsevier Saunders, 2005, pp 171–182. 243 sudden, brief attacks of sleepiness in patients with this disorder and the recurrent falls or astasias accompanying it, which were subsequently termed cataplexy. He characterized Narcolepsy as a “rare neurosis . . . characterized by an urgent necessity to sleep” Adie (4) was also impressed by the likely psychiatric basis for the disorder and wrote: “True Narcolepsy is a functional disorder of the nervous system, probably an undue fatigability of nerve cells, in individuals with a peculiar kind of nervous activity that allows excessive responses to emotional stimuli and favours the spread of inhibitions” (4,5).
eBook - PDFSleep Medicine
A Guide to Sleep and its Disorders
- John M. Shneerson(Author)
- 2009(Publication Date)
- Wiley-Blackwell(Publisher)
The specific effects of Narcolepsy are as follows (Table 6.8). 144 CHAPTER 6 Disorders of sleep Insomnia It is usual for those with Narcolepsy to fall asleep within 5 min of going to bed, but the sleep pattern is then unstable. Awakenings from sleep or shifts from deeper to lighter stages of NREM sleep are frequent. The total duration of sleep during the night is only slightly reduced, and episodes of REM sleep are shorter than in normal subjects. Most narcoleptics feel unrefreshed when they wake in the morning, but this does not correlate with the number or frequency of awakenings during the night. Excessive daytime sleepiness The total sleep time during each 24 h is normal or only slightly increased because the loss of sleep time at night is only just outweighed by the time spent asleep during the day. There are two components to daytime sleepiness. Daytime naps . These are usually of 5–30 min duration and rarely last for more than 1 h. They are temporarily refreshing and there is usually a refractory period of over an hour before the next nap is taken, often at a time corresponding to the 90 min ultradian REM sleep cycle. Sleep-onset REM sleep is common during the naps. The naps may occur in situations where sleep might be anticipated, such as while sitting as a car passenger, but sleep ‘attacks’ may be sudden and irresistible and occur in such situations as while talk-ing, walking or during sexual intercourse. Subalertness . This is probably due to a disorder of wakefulness rather than of sleep, although sleep frag-mentation and poor quality of NREM sleep may con-tribute. Cognitive function is normal, but attention and concentration span are reduced and reaction times prolonged. Poor memory is often reported, but this is probably due to low self-esteem, subalertness and the anterograde amnesia of microsleeps rather than a true memory defect.
eBook - ePub- Daniel J. Buysse, John M. Oldham, Michelle B. Riba(Authors)
- 2007(Publication Date)
- American Psychiatric Association Publishing(Publisher)
The EDS of Narcolepsy presents as an increased propensity to fall asleep, nod, or doze easily in relaxed or sedentary situations, or a need to exert extra effort to avoid sleeping in these situations. Additionally, irresistible or overwhelming urges to sleep commonly occur from time to time during wakeful periods in untreated patients. These “sleep attacks” are not instantaneous lapses into sleep, as is often thought by the general public, but represent episodes of profound sleepiness similar to that caused by severe sleep deprivation or other severe sleep disorders. Many but not all patients with Narcolepsy find that brief naps and longer sleep periods can be temporarily restorative or refreshing. This contrasts with the response to naps or sleep observed in idiopathic hypersomnia, wherein sleep of any duration is rarely restorative. In addition to frank sleepiness, the excessive daytime sleepiness of Narcolepsy can cause or contribute to related symptoms, including poor memory, reduced concentration or attention, and irritability. • Cataplexy. Cataplexy is the partial or complete loss of bilateral voluntary muscle tone in response to strong emotion. The reduced muscle tone may be minimal, occur in a few muscle groups, and cause minimal symptoms such as bilateral ptosis, head drooping, slurred speech, or dropping things from the hand. On the other hand, it may be so severe that total body paralysis occurs, resulting in complete collapse. Cataplectic events usually last from a few seconds to 2 or 3 minutes, but they occasionally continue longer (Honda 1988). The patient is usually alert and oriented during the event despite the inability to respond; thus, cataplectic episodes are distinct from sleep episodes. Positive emotions such as laughter trigger cataplexy more commonly than negative emotions. However, any strong emotion is a potential trigger (Gelb et al. 1994)
eBook - PDFCommon Pitfalls in Sleep Medicine
Case-Based Learning
- Ronald D. Chervin(Author)
- 2014(Publication Date)
- Cambridge University Press(Publisher)
SECTION THREE Diagnosis of Narcolepsy Narcolepsy affects about 0.03% of the population, but often patients remain undiagnosed for many years before their condition is recognized and treated. Much progress has been made in understanding the neurophysiologic basis for Narcolepsy with cataplexy. This condition appears to be caused at least in part by near absence of a neurotransmitter, hypocretin-1 (also called orexin A), that is normally produced in the hypothalamus and distributed widely to many regions of the brain, where it plays a critical function in maintaining alertness. In practice, the diagnosis of Narcolepsy with cataplexy is still accomplished in large part by a good clinical history and examination. Sleep laboratory testing for confirmation is also sought whenever possible, especially when the history of cataplexy is ambiguous. Narcolepsy without cataplexy, in contrast, does not appear likely to have the same neurophysiologic basis, and its diagnosis requires sleep laboratory confirmation. The clinical cases in this section show that significant pitfalls exist for the clinician in the assessment of patients for suspected Narcolepsy, of either type. The history alone can often reveal the diagnosis, but the history, like the Multiple Sleep Latency Test and other clues to the presence of Narcolepsy, can also at times be deceiving. With the advantage of the hindsight provided in the cases that follow, the reader will appreciate that persistence and a combination of serial investigations may be necessary, for some patients, to firmly establish a diagnosis that leads to the most effective treatment plans. 11 Narcolepsy with cataplexy can occur in the absence of a positive Multiple Sleep Latency Test Daniel I. Rifkin Case J. W. presented as a 20-year-old college student with a 3-year history of progressive daytime sleepiness. In high school, she started to fall asleep in both her morning and afternoon classes and took a nap when she returned home from school.
eBook - PDF- Antonio Culebras(Author)
- 2007(Publication Date)
- CRC Press(Publisher)
The combination of an insufficient dopaminergic activation of motor pathways together with a persistent histaminergic activation of arousal is indeed characteristic of cataplexy and differentiate it neurochemically from REM sleep atonia (114,127). Similarities and differences between the two phenomena have been recently discussed also at the clinical and neurophysiological level (142,143). Neurophysiology and Neuropathology The neurophysiological hallmark of Narcolepsy is the occurrence of REM sleep within 15 to 20 minutes from sleep onset (SOREMs, Fig. 2). Narcolepsy is, however, associated also with abnormalities of wakefulness and nonrapid eye movement (NREM) sleep in the presence of normal sleep amounts over 24 hours (144). Furthermore, dissociated states in which elements of wakefulness, NREM, and REM sleep intermingle are frequent in Narcolepsy and lead to such symptoms as cataplexy, sleep paralysis, and REM sleep behavior disorder. Narcolepsy may, therefore, result from a “state boundary dyscontrol”, in which homeostatic, circa-and ultradian sleep – wake regulatory mechanisms appear to be essentially preserved (145 – 147). Two recent studies of our group have indeed shown that homeostatic NREM-and REM-sleep regulation is functional in Narcolepsy (148). FIGURE 2 Sleep onset rapid eye movement (REM) sleep in a 22-year old woman with Narcolepsy with cataplexy and cerebrospinal fluid hypocretin deficiency. Narcolepsy 91 EDS is characterized neurophysiologically by an often-persistent (although waxing and waning in intensity) drowsiness (stage 1a sleep) during the day. Compared to controls, the wake EEG of narcoleptic patients is characterized by lower alpha and higher (and over the course of the day further increasing) delta, theta, and beta powers (149). Drowsiness can evolve into episodes of “microsleep” (stage 1b sleep) lasting 3 to 15 seconds (150). Longer episodes of sleep often also occur voluntarily or involuntarily (“sleep attacks”).
eBook - PDFSleep Disorders in Neurology
A Practical Approach
- Sebastiaan Overeem, Paul Reading, Sebastiaan Overeem, Paul Reading(Authors)
- 2010(Publication Date)
- Wiley-Blackwell(Publisher)
These disorders are discussed in the chapters devoted to the related dis- ease mechanism. What remains is a rather heterogeneous group of pri- mary hypersomnias, or “hypersomnias of central origin” according to the second edition of the International Classification of Sleep Disorders [2]. Narcolepsy is the prototypical disease in this category but a wide range of disorders, including idiopathic hypersomnia and periodic hypersomnias such as the Kleine-Levin syndrome, are included. Sleep Disorders in Neurology. Edited by S. Overeem and P. Reading. © 2010 Blackwell Publishing. 232 Sleep Disorders in Neurology Definition and classification The point at which sleepiness is pathological is not clearly defined in the literature. Originally, the term “hypersomnia” was reserved for condi- tions in which there was an increase in the total amount of sleep over a 24-hour period [3]. However, that is not always the case in disorders with daytime sleepiness as the core symptom. For example, because of disrupted overnight sleep, patients with Narcolepsy often have only mar- ginally increased total sleep times, despite multiple daytime sleep epi- sodes [4]. For the symptom of unwanted daytime sleep periods without a clear increase in total sleep time, the term “excessive daytime sleepiness” (EDS) has been coined. However, EDS and hypersomnia are often used interchangeably, and not in the strict sense. In fact, ICSD-2 groups the various disorders – including Narcolepsy – under hypersomnias of central origin (see table 19.1). To make matters even more confusing, two forms of “idiopathic hypersomnia” are discerned: one with and one without a “long sleep time.” From this perspective, it may be justified to use the term “hypersomnia” to denote a pathological condition or disease entity, and EDS to describe a symptom.
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