On the reported association of psychopathology with sleep terrors in adults.
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Biomedical subjects
Publications and source records attributed to C H Schenck.
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Parasomnias are unpleasant or undesirable behavioral or experiential phenomena that occur predominately or exclusively during sleep. These phenomena were initially thought to represent a unitary event, often attributed to psychiatric disease. Recent clinical and polygraphic analysis has revealed that they are, in fact, the result of a large number of very different conditions, most of which are diagnosable and treatable. In fact, most are not the manifestation of psychiatric disorders, and they are far more prevalent than previously suspected. Although there are many parasomnias (1,2), from a practical standpoint only the few that comprise the overwhelming majority will be discussed in this review. These include disorders of arousal, rapid-eye-movement sleep behavior disorder, nocturnal seizures, and restless legs syndrome. Most parasomnias are readily diagnosable and, more importantly, are treatable.
Sleep can be a troubling experience for persons plagued by nocturnal disorders known as parasomnias. While they are "asleep," such persons may be walking, screaming in terror, rearranging furniture, eating odd food concoctions, or wielding weapons. Or they may be unable to fall asleep because of the unpleasant sensations of restless legs syndrome. Although these disorders are indeed bizarre, effective treatments are available. In this article, Drs Schenck and Mahowald discuss the evaluation and treatment of parasomnias and provide illustrative patient vignettes from their extensive experience at a sleep disorders center.
Sleepiness and sleep disorders are increasingly raising interesting and important medical-legal issues in three areas: violent or injurious behavior arising from the sleep period, accidents or errors in judgment caused by sleepiness behind the wheel or in the workplace, and disability determinations caused by sleepiness-induced work impairment. Sleep-related violence may be caused by many conditions, most of which are diagnosable and treatable. Legal issues raised by these behaviors are usually enigmatic. The nature of such behaviors may be extremely complex, and documenting that a given violent act was caused by such a behavior, after the fact, may be difficult. Guidelines for the medical-legal evaluation of such behaviors have been developed and are evolving. Culpability determination in sleepiness-related industrial or motor vehicle accidents is in the developmental stage, and varies by jurisdiction. Disability determination for workplace sleepiness caused by sleep disorders is in its infancy, and poses a challenge, given the erroneous but pervasive societal attitude that sleepiness is a manifestation of laziness, depression, sloth, work-avoidance behavior, or a defect of character.
The three states of mammalian being, W, REM sleep, and NREM sleep, are not mutually exclusive, and may occur simultaneously, oscillate rapidly, or appear in dissociated or incomplete form to produce primary sleep parasomnias. In addition, dysfunctions of a wide variety of organ systems may take adwide variety of organ systems may take advantage of the sleeping state to declare themselves, resulting in secondary sleep parasomnias. Contrary to popular opinion, the majority of the often bizarre and frightening experiences are not the manifestation of underlying psychological or psychiatric conditions. There is an interesting interaction between sleep-disordered breathing and parasominas. Formal study in an experienced sleep disorders center will usually reveal a diagnosable and treatable condition that explains the spells. Continued study of unusual sleep-related events undoubtedly will reveal more fascinating conditions, expanding our knowledge of sleep physiology, and strengthening the bonds between clinicians and basic-science sleep researchers.
A systematic study of electrophysiologic events [eight-channel EEG, electrocardiogram, electromyogram (EMGs)] surrounding 252 arousals from slow-wave sleep (SWS) in adults with sleepwalking (SW) and sleep terrors (ST) is reported. Hospital-based, overnight polysomnographic monitoring was conducted in 38 adults presenting to a sleep disorders center with injurious SW, ST (21 males, 17 females; mean age 29 years, range 17-69 years). Before nonbehavioral or behavioral arousals from SWS, neither EEG "delta wave buildup," nor heart rate (HR) acceleration, nor tonic/phasic EMG activation was identified. The postarousal EEG demonstrated three patterns: (a) diffuse, rhythmic, delta activity with a typical frequency of 2.2 Hz, a typical amplitude of 85 microV, and a typical duration of 20 s; (b) diffuse delta and theta activity intermixed with alpha and beta activity; and (c) prominent alpha and beta activity. Multichannel, high-voltage, delta activity was observed in <2% of all prearousal periods. HR acceleration emerged abruptly with SWS arousals, with significant changes in mean pre- versus postarousal HR (p < .001). Macrostructural sleep parameters ("sleep architecture") were intact. Therefore, our findings in adults with SW, ST strongly support the classification of SW/ST as disorders of (abrupt) arousal.
The final judgment from a recent criminal trial in the British Columbia (Canada) Supreme Court is summarized and discussed. The trial involved sexual misconduct with a child, excessive alcohol use, and a successful 'sleepwalking (SW) defence' (non-insane automatism). Our comments on this trial provide an opportunity to present our arguments buttressing two newly proposed forensic categories: (i) parasomnia with continuing danger as a non-insane automatism, which originally was proposed for cases involving recurrent, sleep-related violence, but which can also be applied to SW cases involving sleep-related sexual misconduct and alcohol abuse (and other high-risk self-abusive behaviours); (ii) (intermittent) state-dependent continuing danger, an intermediate category within the 'continuing danger' concept, with the core feature being that a person acquitted of a criminal charge on the basis of the 'SW defence' (or some other parasomnia defence), in which the SW episode was provoked by a high-risk behaviour (e.g. alcohol intoxication) should bear full legal culpability for any future episode that was provoked by a recurrence of the high-risk behaviour--provided that the individual had wilfully engaged in that behaviour.
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A series of 33 patients with combined (injurious) sleepwalking, sleep terrors, and rapid eye movement (REM) sleep behavior disorder (viz. "parasomnia overlap disorder") was gathered over an 8-year period. Patients underwent clinical and polysomnographic evaluations. Mean age was 34 +/- 14 (SD) years; mean age of parasomnia onset was 15 +/- 16 years (range 1-66); 70% (n = 23) were males. An idiopathic subgroup (n = 22) had a significantly earlier mean age of parasomnia onset (9 +/- 7 years) than a symptomatic subgroup (n = 11) (27 +/- 23 years, p = 0.002), whose parasomnia began with either of the following: neurologic disorders, n = 6 [congenital Mobius syndrome, narcolepsy, multiple sclerosis, brain tumor (and treatment), brain trauma, indeterminate disorder (exaggerated startle response/atypical cataplexy)]; nocturnal paroxysmal atrial fibrillation, n = 1; posttraumatic stress disorder/major depression, n = 1; chronic ethanol/amphetamine abuse and withdrawal, n = 1; or mixed disorders (schizophrenia, brain trauma, substance abuse), n = 2. The rate of DSM-III-R (Diagnostic and Statistical Manual, 3rd edition, revised) Axis 1 psychiatric disorders was not elevated; group scores on various psychometric tests were not elevated. Forty-five percent (n = 15) had previously received psychologic or psychiatric therapy for their parasomnia, without benefit. Treatment outcome was available for n = 20 patients; 90% (n = 18) had substantial parasomnia control with bedtime clonazepam (n = 13), alprazolam and/or carbamazepine (n = 4), or self-hypnosis (n = 1). Thus, "parasomnia overlap disorder" is a treatable condition that emerges in various clinical settings and can be understood within the context of current knowledge on parasomnias and motor control/dyscontrol during sleep.
The newly identified association of human nonnarcoleptic rapid eye movement (REM) sleep behavior disorder (RBD) with human leukocyte antigen (HLA) DQw1 class II genes raises the possibility that RBD may arise from autoimmune mechanisms. Two recent case reports involving postmortem brain stem histochemical analyses in elderly males with RBD identified severe monoaminergic cell loss in the locus ceruleus (LC). Thus, we designed a study to detect anti-LC antibodies in RBD. Ten Caucasian males (mean age, 66 years) with polygraphically confirmed RBD (n = 5, idiopathic RBD: n = 5, RBD with Parkinson's disease), but without narcolepsy, idiopathic hypersomnia, or autoimmune disease, were recruited for this study, along with 10 Caucasian male controls (mean age, 63 years) without a history of sleep disorder or autoimmune disease. In a blinded design, sera from the RBD patients and their controls were tested against human LC and other brainstem neurons. Brainstem tissue was obtained from autopsies of neurologically normal individuals. The presence of anti-LC antibodies was examined using immunohistochemistry on brainstem sections. Sections incubated with sera from normal individuals and sera from patients with paraneoplastic antineuronal antibodies (anti-Hu and anti-Ri) were used as controls. No reactivity with LC or any other brainstem area was identified with sera from either RBD patients or their controls, or from the other group of normal individuals. In contrast, sera from patients with paraneoplastic anti-Hu and anti-Ri antibodies reacted strongly with nuclei of LC and other brainstem neurons, sparing the nucleoli, and reacted to a lesser extent with the cytoplasm of these neurons. Therefore, it is unlikely that human RBD is associated with anti-LC antibodies. However, an autoimmune process in RBD has not been excluded by this study.
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Data were gathered regarding the associates of chronic fatigue syndrome (CFS) with: (1) speed of cognitive processing, (2) motor speed, (3) ability to sustain attention, and (4) mood. Patients were given a brief neuropsychological test battery before and after double-blind treatment with terfenadine or placebo and completed a daily mood rating scale (Positive and Negative Affect Schedule) during the study. CFS patients exhibited slower cognitive processing and motor speed and lower positive affect, as compared to data reported from previous studies of healthy subjects and other patient groups; however, CFS patients did not exhibit deficits in sustained attention in comparison to other groups. The CFS patients' ability to attend to verbal versus figural stimuli and mood ratings were different from those reported in studies of patients with depression. Because of methodological limitations, these findings are preliminary, but they encourage further assessment of cognitive dysfunction and mood in CFS.
Twenty-five white men with rapid eye movements (REM) sleep behavior disorder, but without narcolepsy, underwent HLA class II antigen typing: 84% (N=21) were DQwl (DQB1*05,06) positive (28% [N=7] were DR2 positive); DQB1*0501 (N=9) and DQB1*0602 (N=7) were the most common phenotypes. The 84% DQwl rate in men with REM sleep behavior disorder was significantly greater (p=0.015) than the 56% DQwl rate found in a local white comparison group (N=66), and was greater than the 39 to 66% DQwl rates published for 12 white groups (N=40-418/group). Thus, another disorder of REM sleep dysregulation (besides narcolepsy) appears to be strongly associated with specific HLA class II genes.
PURPOSE: To assess possible triggers and cofactors for chronic fatigue syndrome (CFS) and to compare levels of selected cytokines between cases and an appropriately matched control group. PATIENTS AND METHODS: We conducted a case-control study of 47 cases of CFS obtained through a regional CFS research program maintained at a tertiary care medical center. One age-, gender-, and neighborhood-matched control was identified for each case through systematic community telephone sampling. Standardized questionnaires were administered to cases and controls. Sera were assayed for transforming growth factor-beta (TGF-beta), interleukin-1 beta, interleukin-6, tumor necrosis factor-alpha, and antibody to Borrelia burgdorferi and Babesia microti. RESULTS: Cases were more likely to have exercised regularly before illness onset than controls (67% versus 40%; matched odds ratio (MOR) = 3.4; 95% CI = 1.2 to 11.8; P = 0.02). Female cases were more likely to be nulliparous prior to onset of CFS than controls (51% versus 31%; MOR = 8.0; 95% CI = 1.03 to 170; P = 0.05). History of other major factors, including silicone-gel breast implants (one female case and one female control), pre-morbid history of depression (15% of cases, 11% of controls) and history of allergies (66% of cases, 51% of controls) were similar for cases and controls. However, cases were more likely to have a diagnosis of depression subsequent to their diagnosis of CFS compared to a similar time frame for controls (MOR = undefined; 95% CI lower bound = 2.5; P < 0.001). Positive antibody titers to B burgdorferi (one case and one control) and B microti (zero cases and two controls) were also similar. CONCLUSIONS: Further investigation into the role of prior routine exercise as a cofactor for CFS is warranted. This study supports the concurrence of CFS and depression, although pre-morbid history of depression was similar for both groups.
PURPOSE: To assess the efficacy, dose stability, safety, and abuse potential of long-term, nightly benzodiazepine treatment of chronic disorders of disrupted nocturnal sleep. PATIENTS AND METHODS: During a 12-year period, one author evaluated and treated 170 adult referrals for > or = 6 months with nightly benzodiazepine therapy for longstanding, sleep-disruptive disorders: injurious sleepwalking and sleep terrors (69); rapid eye movement sleep behavior disorder (52); chronic, severe insomnia (25); and restless legs syndrome/periodic limb movement disorder (24). RESULTS: Complete/substantial control of the sleep disorders was achieved by 146 patients (86%); 8% had adverse effects requiring medication changes; 2% had relapses of alcohol or chemical abuse requiring hospitalization; another 2% at times misused their medications. A total of 136 patients received clonazepam nightly for a mean 3.5 (+/- 2.4) years, with no significant difference in inital versus final mean dose: 0.77 mg (+/- 0.46) versus 1.10 mg (+/- 0.96). Similar results were obtained with chronic alprazolam treatment and with other benzodiazepine treatments. CONCLUSION: Long-term, nightly benzodiazepine treatment of injurious parasomnias and other disorders of disrupted nocturnal sleep resulted in sustained efficacy in most cases, with low risk of dosage tolerance, adverse effects, or abuse. Data from this study on the treatment of chronic, severe insomnia (a small subset of all insomnia) are not generalizable to the typical insomnia patient.
The three states of mammalian being--wakefulness, REM sleep, and NREM sleep--are not mutually exclusive and may occur simultaneously, oscillate rapidly, or appear in dissociated or incomplete form to produce primary sleep parasomnias. Dysfunctions of a wide variety of organ systems may take advantage of the sleeping state to declare themselves, resulting in the secondary sleep parasomnias. Contrary to popular opinion, most of these often bizarre and frightening experiences are not the manifestation of underlying psychological or psychiatric conditions. Formal study in an experienced sleep disorders center usually reveals a diagnosable and treatable condition. Various parasomnias may result in injurious or violent behavior. The forensic science implications are beyond the scope of this article but have been reviewed extensively elsewhere. Continued study of unusual sleep-related events undoubtedly will reveal more fascinating conditions, expanding our knowledge of sleep physiology and strengthening the bonds between clinicians and basic science sleep researchers.
This article focuses primarily on REM sleep behavior disorder, a recently identified syndrome with a corresponding animal model. Diagnostic methods and criteria, treatment, and the world literature are reviewed, and the experience of the authors' institution--The Minnesota Regional Sleep Disorders Center (Minneapolis, MN)--is summarized. Other REM parasomnias, including nightmares, REM sleep-related sinus arrest, and sleep-related painful erections, are discussed briefly.