Chapter 52 - NREM parasomnias

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Abstract

Considerable progress has been made in the systematic study of nonrapid eye movement (NREM) sleep parasomnias. This chapter focuses on the clinical features, prevalence, pathophysiology, associated sleep parameters, and clinical variants of the prototypic NREM sleep parasomnias, namely confusional arousals, sleepwalking, and sleep terrors. Whereas the occurrence of NREM parasomnias in children is frequently viewed as relatively benign, these disorders often pose greater problems, including sleep-related injuries, in affected adults. Most episodes arise from sudden but incomplete arousal from slow-wave sleep and sometimes from stage 2 sleep. Factors that deepen or fragment sleep can facilitate or precipitate NREM parasomnias in predisposed individuals. NREM parasomnias can be associated with various primary sleep disorders or with medical conditions. Diagnosis of NREM parasomnias can often be made based on a detailed history, although some patients may require more extensive evaluations, including polysomnographic study with an expanded EEG montage. Sleep deprivation and the presentation of auditory stimuli during slow-wave sleep are two techniques that can increase the occurrence of behavioral manifestations under laboratory conditions. A variety of nonpharmacological treatments have been recommended for long-term management of NREM parasomnias, whereas pharmacological agents should be considered only if the behaviors are hazardous or extremely disruptive.

Introduction

Parasomnias are undesirable physical or behavioral phenomena that occur during entry into sleep, within sleep, or during partial arousals from sleep (American Academy of Sleep Medicine, 2005). The focus of this chapter is confusional arousals, sleepwalking (somnambulism), and sleep terrors. These sleep disorders constitute the prototypic nonrapid-eye-movement (NREM) sleep parasomnias and are collectively termed “disorders of arousal” (Broughton, 1968) because of the autonomic and motor arousal that propels the patient towards partial wakefulness. A summary and comparison of the main features of NREM and REM sleep parasomnias are presented in Table 52.1.

Disorders of arousal are more common in childhood than in adulthood and their prevalence rate decreases significantly with age. However, whereas the occurrence of NREM parasomnias in children is frequently viewed as a relatively benign and common event that will resolve spontaneously, these disorders often pose greater problems, including social inconvenience and sleep-related injury, in affected adults. In fact, injurious NREM sleep parasomnias in adults may be more prevalent than commonly believed (Schenck et al., 1989, Ohayon et al., 1999, Mahowald and Schenck, 2000c).

The symptoms and manifestations of these NREM parasomnias can be considered along a spectrum. For instance, the patient's affective expression can range from calm to extremely agitated, and the actual physical behaviors can range from simple and isolated actions (e.g., sitting up in bed, mumbling, fingering bed sheets) to complex behaviors (e.g., rearranging furniture, inappropriate sexual activity, playing a musical instrument, driving an automobile). Moreover, an episode can be comprised of two overlapping disorders, such as a sleep terror followed by sleepwalking.

Disorders of arousal share a number of characteristics. Most episodes arise from sudden but incomplete arousal from slow-wave (stages 3 and 4) sleep (Jacobson et al., 1965, Kavey et al., 1990, Espa et al., 2000) and sometimes from stage 2 sleep (Kavey et al., 1990, Zucconi et al., 1995, Joncas et al., 2002). Consequently, these parasomnias tend to occur in the first third of the sleep period when slow-wave sleep (SWS) is predominant. Episodes are generally characterized by misperception and relative unresponsiveness to external stimuli, mental confusion, automatic behaviors, and variable retrograde amnesia. This state is indicative of a high arousal threshold. A common genetic component is also suspected, as a positive family history is often reported by people with an arousal disorder (Hublin et al., 1997, Hublin et al., 2001, Hublin and Kaprio, 2003).

Factors that deepen sleep, such as intense physical activity (Vecchierini, 2001), hyperthyroidism (Ajlouni et al., 2005), fever (Dorus, 1979, Kales et al., 1979, Larsen et al., 2004), sleep deprivation (Rauch and Stern, 1986, Mayer et al., 1998, Joncas et al., 2002), and neuroleptics (Charney, 1979, Landry and Montplaisir, 1998) or medications with depressive CNS effects (Lee-Chiong, 2002, Mahowald, 2002), can facilitate or precipitate NREM parasomnias in predisposed individuals. Factors that fragment sleep, including sleep-disordered breathing (Guilleminault et al., 1998, Espa et al., 2002, Guilleminault et al., 2005a), periodic leg movement syndrome (Guilleminault et al., 2003), stress (Kales et al., 1980b, Klackenberg, 1982, Crisp et al., 1990, Ohayon et al., 1999), and environmental or endogenous stimuli (Gastaut and Broughton, 1965, Kales et al., 1966, Broughton and Gastaut, 1974), can have similar effects. Hormonal factors may also influence the frequency with which women experience parasomnias, as sleep terrors and injurious sleepwalking can emerge premenstrually (Schenck and Mahowald, 1995b), and sleepwalking may decrease during pregnancy, particularly in primiparas (Hedman et al., 2002).

Some researchers (Mahowald and Schenck, 1992, Mahowald and Schenck, 1999, Mahowald and Schenck, 2005) have cogently argued that a proper understanding of many parasomnias rests on the appreciation that sleep and wakefulness are not always mutually exclusive states and that various variables implicated in generation of wakefulness, REM sleep, and NREM sleep (the three primary states of being) may occur simultaneously, interact dynamically, or oscillate rapidly. It should also be noted that NREM parasomnia-related behaviors are not unlike the natural occurrence of clinically wakeful behavior during physiological sleep documented in the animal kingdom (Almanar and Ball, 1994, Rattenborg et al., 1999).

Section snippets

Clinical features

That people sometimes experience confused awakenings from deep sleep in which they appear to be partially awake and partially asleep was noted over 150 years ago – a condition termed “ivresse du sommeil” (French) or “sleep drunkenness”. Other terms used to describe this disorder include Schaftrunkenheit (German) and Elpenor syndrome (derived from the story of Elpenor, who broke his neck during such an episode in Homer's The Odyssey).

Confusional arousals, often seen in children, consist of

Clinical features

Like some other parasomnias, sleepwalking was once thought to be a behavioral manifestation of dreaming-related processes. Sleepwalking is now considered a disorder of arousal involving a physiological dysfunction in the neural regulation of generalized cortical activation. Somnambulistic actions may be complex, such as dressing, playing a musical instrument, or driving a car, and may be performed with substantial dexterity; more often, however, they are mundane, stereotyped, and accompanied by

Clinical features

Sleep terrors, also known as night terrors, are sometimes called “pavor nocturnus” in children. As outlined by Broughton (2000), the term sleep terror is preferable to night terror, because episodes can occur during daytime sleep or naps. Historically, sleep terrors have been confused with nightmares, a distinct REM sleep parasomnia (see Chapter 53 for details of REM sleep parasomnias). Gastaut and Broughton (1965) first observed polysomnographically that sleep terrors were not associated with

Clinical Variants

The behaviors manifested during an arousal disorder can be relatively distinct and specialized. Two variants of NREM parasomnias involve sleep-related eating and sleep-related sexual behaviors.

NREM Parasomnias Associated with Primary Sleep Disorders

Several lines of evidence indicate that in both children and adult populations sleep terrors and sleepwalking can be secondary to sleep respiratory events, such as obstructive sleep apnea (OSA) and upper airway resistance syndrome, or to other sleep disorders. Parent-reported parasomnias in children with OSA suggest that sleep terrors and sleepwalking are more frequent in sleep-disordered children than in normative controls (Owens et al., 1997). Similar results were reported by Ipsiroglu et al.

NREM Parasomnias Associated with Medical Conditions

Rarely, NREM parasomnias may develop as a result of medical or neurological conditions. De novo sleep terrors have been reported in association with a right thalamic lesion (Di Gennaro et al., 2004) and a brainstem lesion (Mendez, 1992). De novo somnambulism has been described in patients presenting with thyrotoxicosis caused by diffuse toxic goiter or Graves' disease (Ajlouni et al., 2001, Ajlouni et al., 2005). Disorders of arousal can also be triggered by medication. These include

Diagnostic Considerations

The DSM-IV and ICSD-II clinical criteria for sleepwalking and sleep terrors are presented in Table 52.2. Diagnosis of NREM parasomnias can often be made based on a detailed history, including complete description of the time course and content of sleep-related behaviors. Given that variable retrograde amnesia characterizes disorders of arousal, descriptive information from family members or a bed partner can be particularly valuable. Similarly, home video recording may also be helpful in

Treatment

Several factors must be taken into account while considering treatment options for disorders of arousal. These include the frequency and chronicity of the episodes, the potential danger to the patient or to others, and the disruptive nature of the disorder for the patient, bed partner, or family. When the episodes are benign and not associated with harm potential, treatment is often unnecessary (Mahowald and Schenck, 1996). Reassuring the patient and significant others about the generally

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