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storylike dreams accompanying the episodes, and tend to have episodes later in the night. NREM sleep arousal disorders occur during NREM sleep, whereas nightmares usually oc- cur during REM sleep. Parents of children with NREM sleep arousal disorders may mis- interpret reports of fragme ntary imagery as nightmares. Breathing-related sleep disorders. Breathing disorders during sleep can also produce
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interpret reports of fragme ntary imagery as nightmares. Breathing-related sleep disorders. Breathing disorders during sleep can also produce confusional arousals with subsequent amnesi a. However, breathin g-related sleep disor- ders are also characterized by characteristic symptoms of snoring, breathing pauses, and
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Non–Rapid Eye Movement Sleep Arousal Disorders 403 daytime sleepiness. In some individuals, a breathing-related sleep disorder may precipi- tate episodes of sleepwalking. REM sleep behavior disorder. REM sleep behavior disorder may be difficult to distin- guish from NREM sleep arousal disorders. REM sleep behavior disorder is characterized by episodes of prominent, co mplex movements, often involving personal injury arising from sleep. In contrast to NR EM sleep arousal disorders, REM sleep behavior disorder oc- curs during REM sleep. Individuals with REM sleep behavior disorder awaken easily and report more detailed and vivid dream content than do individuals wi th NREM sleep arousal disorders. They often report that they “act out dreams.” Parasomnia overlap syndrome. Parasomnia overlap syndrome consists of clinical and polysomnographic features of both sleepwa lking and REM sleep behavior disorder. Sleep-related seizures. Some types of seizures can prod uce episodes of very unusual behaviors that occur predominantly or exclu sively during sleep. Nocturnal seizures may closely mimic NREM sleep arousal disorders but tend to be more stereotypic in nature, oc- cur multiple times nightly, and be more likel y to occur from daytime naps. The presence of sleep-related seizures does not preclude the presence of NREM sleep arousal disorders. Sleep-related seizures should be classified as a form of epilepsy. Alcohol-induced blackouts. Alcohol-induced blackouts may be associated with extremely complex behaviors in the absence of other suggestions of intoxication. They do not involve the loss of consciousness but rather reflect an isolated di sruption of memory for events during a drinking episode. By history, these behaviors may be indistinguishable from those seen in NREM sleep arousal disorders.
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seen in NREM sleep arousal disorders. Dissociative amnesia, with dissociative fugue. Dissociative fugue may be extremely difficult to distinguish from sleepwalking. Unlike all other parasomnias, nocturnal disso- ciative fugue arises from a period of wakefu lness during sleep, rather than precipitously from sleep without intervening wakefulness. A history of recurrent childhood physical or sexual abuse is usually present (b ut may be difficult to obtain). Malingering or other voluntary behavior occurring during wakefulness. As with disso- ciative fugue, malingering or other voluntary behavior o ccurring during wakefulness arises from wakefulness. Panic disorder. Panic attacks may also cause abrupt awakenings from deep NREM sleep accompanied by fearfulness, but these episode s produce rapid and complete awakening with- out the confusion, amnesia, or motor activity typical of NREM sleep arousal disorders. Medication-induced complex behaviors. Behaviors similar to those in NREM sleep arousal disorders can be induced by use of, or withdrawal from, substances or medica- tions (e.g., benzodiazepines, nonbenzodiazepine sedative-hypnotics, opiates, cocaine, nic- otine, antipsychotics, tricyclic antidepressant s, chloral hydrate). Such behaviors may arise from the sleep period and may be extremel y complex. The underlying pathophysiology appears to be a relatively isolated amnesia. In such cases, substance/medication-induced sleep disorder, parasomnia type, should be diagnosed (see “Substance/Medication- Induced Sleep Disorder” la ter in this chapter). Night eating syndrome. The sleep-related eating disorder form of sleepwalking is to be
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Night eating syndrome. The sleep-related eating disorder form of sleepwalking is to be differentiated from night eating syndrome, in which there is a delay in the circadian rhythm of food ingestion and an association with insomnia and/or depression. Comorbidity In adults, there is an association between sleepwalking and major depressive episodes and obsessive-compulsive disorder. Children or ad ults with sleep terro rs may have elevated scores for depression and anxiet y on personality inventories.
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404 Sleep-Wake Disorders Relationship to Internat ional Classification of Sleep Disorders The International Classificat ion of Sleep Disorders, 2nd Edition, includes “confusional arousal” as a NREM sleep arousal disorder. Nightmare Disorder Diagnostic Criteria 307.47 (F51.5) A. Repeated occurrences of extended, extremely dysphoric, and well-remembered dreams that usually involve efforts to avoid threats to survival, security, or physical in- tegrity and that generally occur during the second half of the major sleep episode. B. On awakening from the dysphoric dreams, the individual rapidly becomes oriented and alert. C. The sleep disturbance causes clinically significant distress or impairment in social, oc- cupational, or other important areas of functioning. D. The nightmare symptoms are not attributable to the physiological effects of a sub- stance (e.g., a drug of abuse, a medication). E. Coexisting mental and medical disorders do not adequately explain the predominant complaint of dysphoric dreams. Specify if: During sleep onset Specify if: With associated non–sleep disorder, including substance use disorders With associated othe r medical condition With associated ot her sleep disorder Coding note: The code 307.47 (F51.5) applies to all three specifiers. Code also the relevant associated mental disorder, medical condition, or other sleep disorder imme- diately after the code for nightmare disorder in order to indicate the association. Specify if: Acute: Duration of period of nightmares is 1 month or less. Subacute: Duration of period of nightmares is greater than 1 month but less than 6 months. Persistent: Duration of period of nightmares is 6 months or greater. Specify current severity:
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Specify current severity: Severity can be rated by the frequency with which the nightmares occur: Mild: Less than one episode per week on average. Moderate: One or more episodes per week but less than nightly. Severe: Episodes nightly. Diagnostic Features Nightmares are typically lengthy, elaborate, st orylike sequences of dream imagery that seem real and that incite anxiety, fear, or other dysphoric emotio ns. Nightmare content typically focuses on attempts to avoid or cope with immi nent danger but may involve themes that evoke other negative emotions. Nightmares occurring after traumatic experi- ences may replicate the threatening situation (“replicative nightmares”), but most do not. On awakening, nightmares are well remembered and can be described in detail. They arise
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Nightmare Disorder 405 almost exclusively during rapid eye moveme nt (REM) sleep and can thus occur through- out sleep but are more likely in the second half of the major sleep episode when dreaming is longer and more intense. Factors that incr ease early-night REM intensity, such as sleep fragmentation or deprivation, jet lag, and REM-sensitive medications, might facilitate nightmares earlier in the night, including at sleep onset. Nightmares usually terminate with awakening and rapid return of full alertness. How- ever, the dysphoric emotions may persist into wakefulness and contribute to difficulty re- turning to sleep and lasting daytime distress . Some nightmares, known as “bad dreams,” may not induce awakening and are recalled only later. If nightmare s occur during sleep- onset REM periods ( hypnagogic ), the dysphoric emotion is frequently accompanied by a sense of being both awake and unable to move voluntarily ( isolated sleep paralysis ). Associated Features Supporting Diagnosis Mild autonomic arousal, including sweating, tachycardia, and tachypnea, may character- ize nightmares. Body movements and vocalizations are not characteristic because of REM sleep–related loss of skeletal muscle tone, but such behavi ors may occur under situations of emotional stress or sleep fragmentation and in posttraumatic stress disorder (PTSD). When talking or emoting occurs, it is typica lly a brief event terminating the nightmare. Individuals with frequent nightmares are at substantially greater r isk for suicidal ide- ation and suicide attempts, even when gender and mental illness are taken into account. Prevalence Prevalence of nightmares in creases through childhood into adolescence. From 1.3% to 3.9% of parents report that their preschool children have nightmares “often” or “always”.
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Prevalence increases from ages 10 to 13 for both males and females but continues to in- crease to ages 20–29 for females (while decreasing for males), when it can be twice as high for females as for males. Prevalen ce decreases steadily with age for both sexes, but the gen- der difference remains. Among adults, prevalen ce of nightmares at least monthly is 6%, whereas prevalence for frequent nightmare s is 1%–2%. Estimates often combine idio- pathic and posttraumatic nightmares indiscriminately. Development and Course Nightmares often begin between ages 3 and 6 years but reach a peak prevalence and se- verity in late adolescence or early adulthood. Nigh tmares most likely appear in children exposed to acute or chronic psychosocial stressors and thus may not resolve spontane- ously. In a minority, frequent nightmares pers ist into adulthood, becoming virtually a life- long disturbance. Although specific nightm are content may reflect the individual’s age, the essential features of the diso rder are the same across age groups. Risk and Prognostic Factors Temperamental. Individuals who experience nightmares report more frequent past ad- verse events, but not necessarily trauma, an d often display personality disturbances or psychiatric diagnosis. Environmental. Sleep deprivation or fragmentation, and irregular sleep-wake schedules that alter the timing, intensity, or quantity of REM sleep, can put individuals at risk for nightmares. Genetic and physiological. Twin studies have identified genetic effects on the disposi- tion to nightmares and their co-occurrence with other parasomnias (e.g., sleeptalking). Course modifiers. Adaptive parental bedside behaviors, such as soothing the child fol- lowing nightmares, may protect agai nst developing chronic nightmares.
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406 Sleep-Wake Disorders Culture-Related Diagnostic Issues The significance attributed to nightmares may va ry by culture, and sensitivity to such be- liefs may facilitate disclosure. Gender-Related Diagnostic Issues Adult females report having ni ghtmares more frequently th an do adult males. Nightmare content differs by sex, with adult females tend ing to report themes of sexual harassment or of loved ones disappearing/dying, and adult males tending to report themes of physical aggression or war/terror. Diagnostic Markers Polysomnographic studies demonstrate abrupt awakenings from REM sleep, usually during the second half of the night, prior to report of a nightmare. Heart, respiratory, and eye move- ment rates may quicken or increase in variab ility before awakening. Nightmares following traumatic events may also arise during non-REM (NREM), particularly stage 2, sleep. The typ- ical sleep of individuals with nightmares is mild ly impaired (e.g., redu ced efficiency, less slow- wave sleep, more awakenings), with more frequent periodic leg movements in sleep and rel- ative sympathetic nervous system activation after REM sleep deprivation. Functional Consequences of Nightmare Disorder Nightmares cause more significant subjective distress than demonstrable social or occu- pational impairment. However, if awakenings are frequent or result in sleep avoidance, individuals may experience excessive daytime sleepiness, poor concentration, depression, anxiety, or irritability. Freque nt childhood nightmares (e.g., several per week), may cause significant distress to parents and child. Differential Diagnosis Sleep terror disorder. Both nightmare disorder and sleep terror disorder include awak- enings or partial awakenings with fearfuln ess and autonomic activation, but the two dis- orders are differentiable. Nightmares typically occur later in the night, during REM sleep,
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orders are differentiable. Nightmares typically occur later in the night, during REM sleep, and produce vivid, storylike, and clearly re called dreams; mild autonomic arousal; and complete awakenings. Sleep terrors typically arise in the first third of the night during stage 3 or 4 NREM sleep and produce either no dream recall or images without an elabo- rate storylike quality. The terr ors lead to partial awakenings that leave the individual con- fused, disoriented, and only partially resp onsive and with substantial autonomic arousal. There is usually amnesia for the event in the morning. REM sleep behavior disorder. The presence of complex motor activity during fright- ening dreams should prompt further evaluation for REM sleep behavior disorder, which occurs more typically among late middle-age males and, unlike nightmare disorder, is as- sociated with often violent dream enactment s and a history of nocturnal injuries. The dream disturbance of REM sleep behavior diso rder is described by patients as nightmares but is controlled by appropriate medication. Bereavement. Dysphoric dreams may occur during bereavement but typically involve loss and sadness and are followed by self-refle ction and insight, rather than distress, on awakening. Narcolepsy. Nightmares are a frequent complaint in narcolepsy, but the presence of ex- cessive sleepiness and cataplexy differentiates this condition from nightmare disorder. Nocturnal seizures. Seizures may rarely manifest as nightmares and should be evalu- ated with polysomnography and continuous video electroencephalography. Nocturnal seizures usually involve stereotypical motor activity. Associated nightmares, if recalled,
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Rapid Eye Movement Sleep Behavior Disorder 407 are often repetitive in nature or reflect epilept ogenic features such as the content of diurnal auras (e.g., unmotivated dread), phosphenes, or ictal imagery. Disord ers of arousal, espe- cially confusional arousals, may also be present. Breathing-related sleep disorders. Breathing-related sleep disorders can lead to awaken- ings with autonomic arousal, but these are not usually accompanied by recall of nightmares. Panic disorder. Attacks arising during sleep can produce abrupt awakenings with au- tonomic arousal and fearfulness, but nightmares are typically not reported and symptoms are similar to panic attacks arising during wakefulness. Sleep-related dissociative disorders. Individuals may recall actual physical or emo- tional trauma as a “dream” during elec troencephalography-documented awakenings. Medication or substance use. Numerous substances/medicat ions can precipitate night- mares, including dopaminergics; beta-adrener gic antagonists and other antihypertensives; amphetamine, cocaine, and other stimulants; antidepressants; smoking cessation aids; and melatonin. Withdrawal of REM sleep–su ppressant medications (e.g., antidepressants) and alcohol can produce REM sleep rebound accompanied by nightmares. If nightmares are sufficiently severe to warrant independent clinical attention, a diagnosis of substance/ medication-induced sleep disorder should be considered. Comorbidity Nightmares may be comorbid with several medical conditions, including coronary heart disease, cancer, parkinsonism, and pain, and ca n accompany medical treatments, such as he- modialysis, or withdrawal from medications or substances of abuse. Nightmares frequently
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modialysis, or withdrawal from medications or substances of abuse. Nightmares frequently are comorbid with other mental disorders, in cluding PTSD; insomnia disorder; schizophrenia; psychosis; mood, anxiety, adjustment, and personality disorders; and grief during be- reavement. A concurrent nightmare disorder diagnosis should only be considered when in- dependent clinical attention is warranted (i.e., Criteria A–C are met). Otherwise, no separate diagnosis is necessary. These conditions should be listed under the appropriate comorbid category specifier. However, nightmare disorder may be diagnosed as a separate disorder in individuals with PTSD if the nightmares are temporally unrelated to PTSD (i.e., preceding other PTSD symptoms or persisting after other PTSD symptoms have resolved). Nightmares are normally characteristic of REM sleep behavior disorder, PTSD, and acute stress disorder, but nightmare di sorder may be independently coded if nightmares preceded the condition and their frequency or severity necessitates independent clinical attention. The latter may be determined by asking whether ni ghtmares were a proble m before onset of the other disorder and whether they continued after other symptoms had remitted. Relationship to Internat ional Classification of Sleep Disorders The International Classification of Sleep Disorders, 2nd Edition (ICSD-2), presents similar di- agnostic criteria fo r nightmare disorder. Rapid Eye Movement Sleep Behavior Disorder Diagnostic Criteria 327.42 (G47.52) A. Repeated episodes of arousal during sleep associated with vocalization and/or com- plex motor behaviors. B. These behaviors arise during rapid eye movement (REM) sleep and therefore usually occur more than 90 minutes after sleep onset, are more frequent during the later por- tions of the sleep period, and uncommonly occur during daytime naps.
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408 Sleep-Wake Disorders C. Upon awakening from these episodes, the individual is completely awake, alert, and not confused or disoriented. D. Either of the following: 1. REM sleep without atonia on polysomnographic recording. 2. A history suggestive of REM sleep behavior disorder and an established synuclein- opathy diagnosis (e.g., Parkinson’s disease, multiple system atrophy). E. The behaviors cause clinically significant distress or impairment in social, occupa- tional, or other important areas of functioning (which may include injury to self or the bed partner). F. The disturbance is not attributable to the physiological effects of a substance (e.g., a drug of abuse, a medication) or another medical condition. G. Coexisting mental and medical disorders do not explain the episodes. Diagnostic Features The essential feature of rapid eye movement (REM) sleep behavior disorder is repeated episodes of arousal, often associated with vo calizations and/or complex motor behaviors arising from REM sleep (Criterion A). These be haviors often reflect motor responses to the content of action-filled or violent dreams of being attacked or trying to escape from a threatening situation, which may be termed dream enacting behaviors. The vocalizations are often loud, emotion-fill ed, and profane. These behaviors may be very bothersome to the individual and the bed partner and may result in significant injury (e.g., falling, jumping, or flying out of bed; running, punching, thru sting, hitting, or kicking). Upon awakening, the individual is immediately awake, alert, and oriented (Criterion C) and is often able to recall dream mentation, which closely correlates with the observed behavior. The eyes typically remain closed during these events. The diagnosis of REM sleep behavior disor- der requires clinically significant distress or impairment (Criterion E); this determination
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der requires clinically significant distress or impairment (Criterion E); this determination will depend on a number of factors, including the frequency of events, the potential for vi- olence or injurious behaviors, embarrassment , and distress in othe r household members. Associated Features Supporting Diagnosis Severity determination is best made based on the nature or conseq uence of the behavior rather than simply on frequency. Although the behaviors are typically prominent and vi- olent, lesser behaviors may also occur. Prevalence The prevalence of REM sleep behavior disord er is approximately 0.38%–0.5% in the gen- eral population. Prevalence in patients with psychiatric disorders may be greater, possibly related to medications prescribed for the psychiatric disorder. Development and Course The onset of REM sleep behavior disorder may be gradual or rapid, and the course is usu- ally progressive. REM sleep behavior disorder associated with neurodegenerative disor- ders may improve as the unde rlying neurodegenerative disorder progresses. Because of the very high association with the later appe arance of an underlyi ng neurodegenerative disorder, most notably one of the synucleino pathies (Parkinson’s disease, multiple system atrophy, or major or mild neurocognitive di sorder with Lewy bodies), the neurological status of individuals with REM sleep behavior disorder should be closely monitored. REM sleep behavior disorder overwhelmingly affects males older than 50 years, but in- creasingly this disorder is being identified in females and in younger individuals. Symp-
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Rapid Eye Movement Sleep Behavior Disorder 409 toms in young individuals, particularly young females, should raise the possibility of narcolepsy or medication-induced REM sleep behavior disorder. Risk and Prognostic Factors Genetic and physiological. Many widely prescribed medications, including tricyclic antidepressants, selective serotonin reuptake inhibitors, serotonin-norepinephrine reup- take inhibitors, and beta-blockers, may resu lt in polysomnographic evidence of REM sleep without atonia and in frank REM sleep behavior disorder. It is not known whether the medications per se result in REM sleep behavi or disorder or they unmask an underlying predisposition. Diagnostic Markers Associated laboratory findin gs from polysomnography indicate increased tonic and/or phasic electromyographic activi ty during REM sleep that is normally associated with mus- cle atonia. The increased muscle activity variably affects different muscle groups, mandating more extensive electromyographi c monitoring than is employed in conventional sleep stud- ies. For this reason, it is su ggested that electrom yographic monitoring include the submen- talis, bilateral extensor digito rum, and bilateral anterior tibi alis muscle groups. Continuous video monitoring is mandator y. Other polysomnographic findings may include very fre- quent periodic and aperiodic extremity el ectromyography acti vity during non-REM (NREM) sleep. This polysomnography observation, termed REM sleep without atonia, is pres- ent in virtually all cases of REM sleep behavi or disorder but may also be an asymptomatic polysomnographic finding. Clinical dream-enacting behaviors coupled with the polysom- nographic finding of REM without atonia is necessary for the diagnosis of REM sleep behav-
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nographic finding of REM without atonia is necessary for the diagnosis of REM sleep behav- ior disorder. REM sleep without atonia with out a clinical history of dream-enacting behaviors is simply an asymptomatic polyso mnographic observation. It is not known whether isolated REM sleep without atonia is a precursor to REM sleep behavior disorder. Functional Consequences of Rapid Eye Movement Sl eep Behavior Disorder REM sleep behavior disorder may occur in isol ated occasions in otherwise unaffected in- dividuals. Embarrassment concer ning the episodes can impair social relationships. Indi- viduals may avoid situations in which others might become aware of the disturbance, visiting friends overnight, or sleeping with be d partners. Social isolation or occupational difficulties can result. Uncommonly, REM sleep behavior disorder may result in serious injury to the victim or to the bed partner. Differential Diagnosis Other parasomnias. Confusional arousals, sleepwalking, and sleep terrors can easily be confused with REM sleep behavior disorder. In general, these disorders occur in younger individuals. Unlike REM sleep behavior disorder, they arise from deep NREM sleep and therefore tend to occur in the early portion of the sleep period. Awakening from a confu- sional arousal is associated with confusion, disorientation, and incomplete recall of dream mentation accompanying the behavior. Polysomnographic monitoring in the disorders of arousal reveals normal REM atonia. Nocturnal seizures. Nocturnal seizures may perfectly mimic REM sleep behavior disor- der, but the behaviors are generally more st ereotyped. Polysomnographic monitoring em- ploying a full electroencephalographic seiz ure montage may differentiate the two. REM sleep without atonia is not present on polysomnographic monitoring.
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410 Sleep-Wake Disorders Obstructive sleep apnea. Obstructive sleep apnea may result in behaviors indistin- guishable from REM sleep behavior disorder . Polysomnographic monitoring is necessary to differentiate between the two. In this ca se, the symptoms resolv e following effective treatment of the obstructive sleep apnea, and REM sleep without atonia is not present on polysomnography monitoring. Other specified dissociative disorder (sleep-related psychogenic dissociative disorder). Unlike virtually all other parasomnias, which arise precipitously from NREM or REM sleep, psychogenic dissociative behaviors aris e from a period of well-defined wakefulness during the sleep period. Unlike REM sleep behavi or disorder, this condition is more prev- alent in young females. Malingering. Many cases of malingering in which the individual reports problematic sleep movements perfectly mimic the clinical features of REM sleep behavior disorder, and polysomnographic documentation is mandatory. Comorbidity REM sleep behavior diso rder is present concurrently in approximately 30% of patients with narcolepsy. When it occurs in narcolep sy, the demographics reflect the younger age range of narcolepsy, with equal frequency in males and females. Based on findings from individuals presenting to sleep clinics, most individuals (>50%) with initially “idiopathic” REM sleep behavior disorder will eventually develop a neurodegenerative disease—most notably, one of the synucleinopathies (Parkins on’s disease, multiple system atrophy, or major or mild neurocognitive disorder with Lewy bodies). REM sleep behavior disorder often predates any other sign of these disorder s by many years (often more than a decade). Relationship to Internat ional Classification of Sleep Disorders
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Relationship to Internat ional Classification of Sleep Disorders REM sleep behavior disorder is virtually identical to REM sleep beha vior disorder in the International Classification of Sleep Disorders, 2nd Edition (ICSD-2). Restless Legs Syndrome Diagnostic Criteria 333.94 (G25.81) A. An urge to move the legs, usually accompanied by or in response to uncomfortable and unpleasant sensations in the legs, characterized by all of the following: 1. The urge to move the legs begins or wors ens during periods of rest or inactivity. 2. The urge to move the legs is partial ly or totally relieved by movement. 3. The urge to move the legs is worse in the evening or at night than during the day, or occurs only in the evening or at night. B. The symptoms in Criterion A occur at least three times per week and have persisted for at least 3 months. C. The symptoms in Criterion A are accompanied by significant distress or impairment in social, occupational, educational, academic, behavioral, or other important areas of functioning. D. The symptoms in Criterion A are not attributable to another mental disorder or medical condition (e.g., arthritis, l eg edema, peripheral ischemia, leg cramps) and are not better explained by a behavioral condition (e.g., positional discomfort, h abitual foot tapping). E. The symptoms are not attributable to the physiological effects of a drug of abuse or medication (e.g., akathisia).
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Restless Legs Syndrome 411 Diagnostic Features Restless legs syndrome (RLS) is a sensorimotor, neurological sleep disorder characterized by a desire to move the legs or arms, usua lly associated with uncomfortable sensations typically described as creeping, crawling, tingling, burning, or itch ing (Criterion A). The diagnosis of RLS is based primarily on patient self-report and history. Symptoms are worse when the individual is at rest, and freq uent movements of the legs occur in an effort to relieve the uncomfortable sensations. Sympto ms are worse in the evening or night, and in some individuals they occur only in the ev ening or night. Evening worsening occurs in- dependently of any differences in activity. It is important to differentiate RLS from other conditions such as positional discom fort and leg cramps (Criterion D). The symptoms of RLS can delay sleep onset and awaken the individual from sleep and are associated with significant sleep fragmentation. The relief obtained from moving the legs may no longer be apparent in severe ca ses. RLS is associated with daytime sleepiness and is frequently accompanied by significant clinical distress or functional impairment. Associated Features Supporting Diagnosis Periodic leg movements in sleep (PLMS) can serve as corroborating ev idence for RLS, with up to 90% of individuals di agnosed with RLS demonstratin g PLMS when recordings are taken over multiple nights. Periodic leg mo vements during wakefulness are supportive of an RLS diagnosis. Reports of difficulty initiating and maintaining sleep and of excessive daytime sleepiness may also support the diagnosis of RLS. Additional supportive features include a family history of RLS among first- degree relatives and a reduction in symptoms, at least initially, with dopaminergic treatment. Prevalence
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at least initially, with dopaminergic treatment. Prevalence Prevalence rates of RLS vary widely when br oad criteria are utilized but range from 2% to 7.2% when more defined criteria are employed . When frequency of symptoms is at least three times per week with moderate or severe distress, the prevalence rate is 1.6%; when frequency of symptoms is a minimum of one ti me per week, the prevalence rate is 4.5%. Females are 1.5–2 times more likely than males to have RLS. RLS also increases with age. The prevalence of RLS may be lower in Asian populations. Development and Course The onset of RLS typically occurs in the seco nd or third decade. Approximately 40% of in- dividuals diagnosed with RLS during adul thood report having experienced symptoms before age 20 years, and 20% report having experienced symptoms before age 10 years. Prevalence rates of RLS increase steadily with age until about age 60 years, with symptoms remaining stable or decreasing slightly in older age groups. Compared with nonfamilial cases, familial RLS usually has a younger age at onset and a slower pr ogressive course. The clinical course of RLS differs by age at onset. When onset occurs before age 45, there is of- ten a slow progression of symptoms. In late-onset RLS, rapid progression is typical, and aggravating factors are common. Symptoms of RLS appear similar acro ss the lifespan, re- maining stable or decreasing slightly in older age groups. Diagnosis of RLS in children can be diff icult because of the self-report component. While Criterion A for adults assumes that the description of “urge to move” is by the pa-
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tient, pediatric diagnosis requir es a description in the child’s own words rather than by a parent or caretaker. Typically children age 6 years or older are able to provide detailed, ad- equate descriptors of RLS. However, children rarely use or understand the word “urge,” reporting instead that their legs “have to” or “got to” move. Also, potentially related to prolonged periods of sitting during class, tw o-thirds of children and adolescents report daytime leg sensations. Thus, fo r diagnostic Criterion A3, it is important to compare equal
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412 Sleep-Wake Disorders duration of sitting or lying down in the day to sitting or lying down in the evening or night. Nocturnal worsening tends to persist even in the context of pediatric RLS. As with RLS in adults, there is a significant negative impact on sleep, mood, cognition, and function. Im- pairment in children and adolescents is mani fested more often in behavioral and educa- tional domains. Risk and Prognostic Factors Genetic and physiological. Predisposing factors includ e female gender, advancing age, genetic risk variants, an d family history of RLS. Precipitating factors are often time- limited, such as iron defici ency, with most individuals resuming normal sleep patterns after the initial triggering even t has disappeared. Genetic risk variants also play a role in RLS secondary to such disorder s as uremia, suggesting that individuals with a genetic sus- ceptibility develop RLS in the presence of further risk factors. RLS has a strong familial component. There are defined pathophysi ological pathways subserving RLS. Genome-wide asso- ciation studies have found that RLS is significantly associated with common genetic vari- ants in intronic or intergenic regions in MEIS1 , BTBD9 , and MAP2K5 on chromosomes 2p, 6p, and 15q, respectively. The ass ociation of these three varian ts with RLS has been inde- pendently replicated. BTBD9 confers a very large (80%) exce ssive risk when even a single allele is present. Because of the high frequenc y of this variant in individuals of European descent, the population attributable risk (P AR) approximates 50%. At-risk alleles associ-
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ated with MEIS1 and BTBD9 are less common in individuals of African or Asian descent, perhaps suggesting lower risk fo r RLS in these populations. Pathophysiological mechanisms in RLS also in clude disturbances in the central dopa- minergic system and disturbances in iron metabolism. The endo genous opiate system may also be involved. Treatment effect s of dopaminergic drugs (primarily D2 and D3 non- ergot agonists) provide further support that RLS is grounded in dysfunctional central dopaminergic pathways. While the effective treatment of RLS has also been shown to sig- nificantly reduce depressive symptoms, seroto nergic antidepressants can induce or aggra- vate RLS in some individuals. Gender-Related Diagnostic Issues Although RLS is more prevalent in females than in males, there are no diagnostic differ- ences according to gender. However, the prev alence of RLS during pregnancy is two to three times greater than in the general population. RLS associated with pregnancy peaks during the third trimester and improves or reso lves in most cases soon after delivery. The gender difference in prevalence of RLS is explai ned at least in part by parity, with nullipa- rous females being at the same r isk of RLS as age-matched males. Diagnostic Markers Polysomnography demonstrates significant abnormalities in RLS, commonly increased latency to sleep, and higher arousal index. Polysomnography with a preceding immobili- zation test may provide an indicator of the motor sign of RLS, periodic limb movements, under standard conditions of sleep and during quiet resting, both of which can provoke RLS symptoms. Functional Consequences of Restless Legs Syndrome
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symptoms. Functional Consequences of Restless Legs Syndrome Forms of RLS severe enough to si gnificantly impair functioning or associated with mental dis- orders, including depression an d anxiety, occur in approximately 2%–3% of the population. Although the impact of milder symptoms is less well ch aracterized, in dividuals with RLS complain of disruption in at least one activity of daily li ving, with up to 50% reporting
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Substance/Medication-Induced Sleep Disorder 413 a negative impact on mood, and 47.6% reporting a lack of energy. The most common conse- quences of RLS are sleep disturbance, includ ing reduced sleep time , sleep fragmentation, and overall disturbance; depression, generalize d anxiety disorder, panic disorder, and post- traumatic stress disorder; and quality-of-life impairments. RLS can result in daytime sleep- iness or fatigue and is frequently accompanied by significant distress or impairment in affective, social, occupational, educational, academic, behavioral, or cognitive functioning. Differential Diagnosis The most important conditions in the differential diagnosis of RLS are leg cramps, posi- tional discomfort, arthralgias/arthritis, myalgias, positional ischemia (numbness), leg edema, peripheral neuropathy, radiculopathy, and habitual foot tapping. “Knotting” of the muscle (cramps), relief with a single postural shift, limitation to joints, soreness to pal- pation (myalgias), and other abnormalities on physical exam ination are not characteristic of RLS. Unlike RLS, nocturnal leg cramps do not typically present with the desire to move the limbs nor are there frequent limb movements. Less common conditions to be differen- tiated from RLS include neuroleptic-induced akathisia, myelopathy, symptomatic venous insufficiency, peripheral artery disease, ec zema, other orthopedic problems, and anxiety- induced restlessness. Worsening at night an d periodic limb movements are more common in RLS than in medication-induced akathisia or peripheral neuropathy. While is it important that RLS symptoms not be solely accounted for by another medical
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While is it important that RLS symptoms not be solely accounted for by another medical or behavioral condition, it should also be appreciated that any of thes e similar conditions can occur in an individual with RLS. This necessi tates a separate focus on each possible condi- tion in the diagnostic process and when assessing impact. For cases in which the diagnosis of RLS is not certain, evaluation for the supportive features of RLS, particularly PLMS or a fam- ily history of RLS, may be helpful. Clinical features, such as response to a dopaminergic agent and positive family history for RLS, can help with the differential diagnosis. Comorbidity Depressive disorders, anxiety disorders, an d attentional disorder s are commonly comor- bid with RLS and are discussed in the sectio n “Functional Consequences of Restless Legs Syndrome.” The main medical disorder comorbid with RLS is cardiovascular disease. There may be an association with numerous other medical disorders, including hyperten- sion, narcolepsy, migraine, Parkinson’s disease, multiple sclerosis, peripheral neuropathy, obstructive sleep apnea, diabetes mellitus, fibromyalgia, osteoporosis, obesity, thyroid disease, and cancer. Iron defi ciency, pregnancy, and chronic renal failure are also comor- bid with RLS. Relationship to Internat ional Classification of Sleep Disorders The International Classifica tion of Sleep Disorders, 2nd Edition (ICSD-2), presents similar diag- nostic criteria for RLS but does not contain a criterion specifying fr equency or duration of symptoms. Substance/Medication-Induced Sleep Disorder Diagnostic Criteria A. A prominent and severe disturbance in sleep. B. There is evidence from the history, physical examination, or laboratory findings of both
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B. There is evidence from the history, physical examination, or laboratory findings of both (1) and (2):
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414 Sleep-Wake Disorders 1. The symptoms in Criterion A developed during or soon after substance intoxication or after withdrawal from or exposure to a medication. 2. The involved substance/medication is capable of producing the symptoms in Crite- rion A. C. The disturbance is not better explained by a sleep disorder that is not substance/ medication-induced. Such evidence of an independent sleep disorder could include the following: The symptoms precede the onset of the substance/medication use; the symptoms persist for a substantial period of time (e.g., about 1 month) after the cessation of acute withdrawal or severe intoxication; or there is other evidence suggesting the existence of an independent non-substance/medication-induced sleep disorder (e.g., a history of recurrent non-substance/medication-related episodes). D. The disturbance does not occur exclusively during the course of a delirium. E. The disturbance causes clinically significant distress or impairment in social, occupa- tional, or other important areas of functioning. Note: This diagnosis should be made instead of a diagnosis of substance intoxication or substance withdrawal only when the symptoms in Criterion A predominate in the clinical picture and when they are sufficiently severe to warrant clinical attention. Coding note: The ICD-9-CM and ICD-10-CM codes for the [specific substance/medica- tion]-induced sleep disorders are indicated in the table below. Note that the ICD-10-CM code depends on whether or not there is a comorbid substance use disorder present for the same class of substance. If a mild substance use disorder is comorbid with the sub- stance-induced sleep disorder, the 4th position character is “1,” and the clinician should
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record “mild [substance] use disorder” before the substance-induced sleep disorder (e.g., “mild cocaine use disorder with cocaine-induc ed sleep disorder”). If a moderate or severe substance use disorder is comorbid with the substance-induced sleep disorder, the 4th po- sition character is “2,” and the clinician shoul d record “moderate [substance] use disorder” or “severe [substance] use disorder,” depending on the severity of the comorbid substance use disorder. If there is no comorbid substance use disorder (e.g., after a one-time heavy use of the substance), then the 4th position character is “9,” and the clinician should record only the substance-induced sleep disorder. A m oderate or severe tobacco use disorder is required in order to code a tobacco-induced sleep disorder; it is not permissible to code a comorbid mild tobacco use disorder or no tobacco use disorder with a tobacco-induced sleep disorder. Specify whether: Insomnia type: Characterized by difficulty falling asleep or maintaining sleep, frequent nocturnal awakenings, or nonrestorative sleep. Daytime sleepiness type: Characterized by predominant complaint of excessive sleepiness/fatigue during waking hours or, less commonly, a long sleep period. Parasomnia type: Characterized by abnormal behavioral events during sleep. Mixed type: Characterized by a substance/m edication-induced sleep problem charac- terized by multiple types of sleep symptoms, but no symptom clearly predominates. Specify if (see Table 1 in the chapter “Substance-Related and Addictive Disorders” for di- agnoses associated with substance class): With onset during intoxication: This specifier should be used if criteria are met for
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With onset during intoxication: This specifier should be used if criteria are met for intoxication with the substance/medication and symptoms developed during the intox- ication period. With onset during disco ntinuation/withdrawal: This specifier should be used if cri- teria are met for discontinuation/withdrawal from the substance/medication and symp- toms developed during, or shortly after, discontinuation of the substance/medication.
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Substance/Medication-Induced Sleep Disorder 415 Recording Procedures ICD-9-CM. The name of the substance/medication-induced sleep disorder begins with the specific substance (e.g., cocaine, bupropion) that is presumed to be causing the sleep disturbance. The diagnostic code is selected from the table included in the criteria set, which is based on the drug class. For substances that do not fit into any of the classes (e.g., bupropion), the code for “other substance” should be used; and in cases in which a sub- stance is judged to be an etiological factor but the specific class of substance is unknown, the category “unknown substance” should be used. The name of the disorder is followed by the sp ecification of onset (i.e., onset during in- toxication, onset during discontinuation/withdrawal), followed by the subtype designa- tion (i.e., insomnia type, daytime sleepine ss type, parasomnia type, mixed type). Unlike the recording procedures for ICD-10-CM, wh ich combine the substance-induced disorder and substance use disorder into a single code, for ICD-9-CM a separate diagnostic code is given for the substance use disorder. For ex ample, in the case of insomnia occurring during withdrawal in a man with a severe lorazepam use disorder, the diagnosis is 292.85 lorazepam-induced sleep disorder, with onset during withdrawal, insomnia type. An ad- ditional diagnosis of 304.10 severe lorazepam us e disorder is also given. When more than one substance is judged to play a significant role in the developmen t of the sleep distur- bance, each should be listed separately (e.g., 292.85 alcohol-induced sleep disorder, with
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onset during intoxication, insomnia type; 292. 85 cocaine-induced sleep disorder, with on- set during intoxication, insomnia type). ICD-10-CM. The name of the substance/medication-induced sleep disorder begins with the specific substance (e.g., cocaine, bupropion) that is presumed to be causing the sleep distur- bance. The diagnostic code is selected from the ta ble included in the criteria set, which is based on the drug class and presence or absence of a comorbid substance use disorder. For sub- stances that do not fit into any of the classes (e.g., bupropion), the code for “other substance” should be used; and in cases in which a substance is judged to be an etiological factor but the specific class of substance is unknown, the category “unknown substance” should be used. When recording the name of the disorder, th e comorbid substance use disorder (if any) is listed first, followed by the word “with,” followed by the name of the substance-induced sleep disorder, followed by the specification of onset (i.e., onset during intoxication, onsetICD-10-CM ICD-9-CMWith use disorder, mildWith use disorder, moderate or severeWithout use disorder Alcohol 291.82 F10.182 F10.282 F10.982 Caffeine 292.85 F15.182 F15.282 F15.982 Cannabis 292.85 F12.188 F12.288 F12.988 Opioid 292.85 F11.182 F11.282 F11.982
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Opioid 292.85 F11.182 F11.282 F11.982 Sedative, hypnotic, or anxiolytic 292.85 F13.182 F13.282 F13.982 Amphetamine (or other stimulant)292.85 F15.182 F15.282 F15.982 Cocaine 292.85 F14.182 F14.282 F14.982 Tobacco 292.85 NA F17.208 NA Other (or unknown) substance 292.85 F19.182 F19.282 F19.982
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416 Sleep-Wake Disorders during discontinuation/withdrawal), followed by the subtype designation (i.e., insomnia type, daytime sleepiness type, parasomnia type , mixed type). For example, in the case of insomnia occurring during withdrawal in a ma n with a severe lorazepam use disorder, the diagnosis is F13.282 severe lorazepam use disorder with lorazepam-induced sleep disor- der, with onset during withdrawal, insomnia type. A separate diagnosis of the comorbid severe lorazepam use disorder is not given. If the substance-induced sleep disorder occurs without a comorbid substance use disorder (e.g., with medication use), no accompanying substance use disorder is noted (e.g., F19.98 2 bupropion-induced sleep disorder, with on- set during medication use, insomnia type). When more than one substance is judged to play a significant role in the development of the sleep disturbance, each should be listed separately (e.g., F10.282 severe alcohol use disorder with alcohol-induced sleep disorder, with onset during intoxication, insomnia ty pe; F14.282 severe cocaine use disorder with cocaine-induced sleep disord er, with onset during into xication, insomnia type). Diagnostic Features The essential feature of substance/medication -induced sleep disorder is a prominent sleep disturbance that is suffi ciently severe to warrant independen t clinical attent ion (Criterion A) and that is judged to be primarily associated with the pharmacological effects of a substance (i.e., a drug of abuse, a medication, toxin ex posure) (Criterion B). Depending on the sub- stance involved, one of four types of sleep di sturbances is reported. Insomnia type and day-
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time sleepiness type are most common, while parasomnia type is seen less often. The mixed type is noted when more than one type of sl eep disturbance–related symptom is present and none predominates. The disturbance must not be better explained by another sleep disorder (Criterion C). A substance/medication-induced sleep disorder is distinguished from insom- nia disorder or a disorder associated with excessive daytime sleepines s by considering onset and course. For drugs of abuse, there must be evidence of intoxication or withdrawal from the history, physical examination, or laboratory findings. Substance/medication-induced sleep disorder arises only in association wi th intoxication or di scontinuation/withdrawal states, whereas other sleep disorders may preced e the onset of substance use or occur during times of sustained abstinence. As discontinuation/withdrawal states for some substances can be protracted, onset of th e sleep disturbance can occur 4 weeks after cessation of sub- stance use, and the disturbance may have feat ures atypical of other sleep disorders (e.g., atypical age at onset or course). The diagnosi s is not made if the sleep disturbance occurs only during a delirium (Criterion D). The symptoms must cause clinically significant dis- tress or impairment in social, occupational, or other important areas of functioning (Crite- rion E). This diagnosis should be made instea d of a diagnosis of substance intoxication or substance withdrawal only when the symptoms in Criterion A predominate in the clinical picture and when the symptoms warrant independent clinical attention. Associated Features Supporting Diagnosis During periods of substance/medication use, intoxication, or withdrawal, individuals fre- quently complain of dysphoric mood, includin g depression and anxiety, irritability, cog- nitive impairment, inability to concentrate, and fatigue.
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nitive impairment, inability to concentrate, and fatigue. Prominent and severe sleep disturbances can occur in association with intoxication with the following classes of substances: alco hol; caffeine; cannabis; opioids; sedatives, hypnotics, or anxiolytics; st imulants (including cocaine); and other (or unknown) sub- stances. Prominent and severe sleep disturbances can occur in association with withdrawal
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hypnotics, or anxiolytics; st imulants (including cocaine); and other (or unknown) sub- stances. Prominent and severe sleep disturbances can occur in association with withdrawal from the following classes of substances: alcohol; caffeine; cannabis; opioids; sedatives, hypnotics, or anxiolytics; stimulant (includi ng cocaine); tobacco; and other (or unknown) substances. Some medications that invoke sl eep disturbances include adrenergic agonists and antagonists, dopamine agonists and antagonists, cholinergic agonists and antagonists, serotonergic agonists and antagonists, antihistamines, and corticosteroids.
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Substance/Medication-Induced Sleep Disorder 417 Alcohol. Alcohol-induced sleep disorder typica lly occurs as insomnia type. During acute intoxication, alcohol produces an immedi ate sedative effect depending on dose, ac- companied by increased stages 3 and 4 non– rapid eye movement (NREM) sleep and re- duced rapid eye movement (REM) sleep. Following these initial e ffects, there may be increased wakefulness, restless sleep, and vivid and anxiety-laden dreams for the remain- ing sleep period. In parallel, stages 3 and 4 sleep are reduced, and wakefulness and REM sleep are increased. Alcohol can aggravate br eathing-related sleep disorder. With habitual use, alcohol continues to show a short-lived sedati ve effect in the first half of the night, fol- lowed by sleep continuity disruption in the second half. During alcohol withdrawal, there is extremely disrupted sleep continuity, and an increased amount and intensity of REM sleep, associated frequently with vivid drea ming, which in extreme form, constitutes part of alcohol withdrawal delirium. After acut e withdrawal, chronic alcohol users may con- tinue to complain of light, fragmented sleep for weeks to years associated with a persistent deficit in slow-wave sleep. Caffeine. Caffeine-induced sleep disorder produces insomnia in a dose-dependent man- ner, with some individuals presenting with daytime sleepiness related to withdrawal. Cannabis. Acute administration of cannabis may shorten sleep latency, though arous- ing effects with increments in sleep latenc y also occur. Cannabis enhances slow-wave sleep and suppresses REM sleep after acute administration. In chronic users, tolerance to the sleep-inducing and slow-wave sleep–enha ncing effects develops. Upon withdrawal, sleep difficulties and unpleasant dreams have been reported lasting for several weeks.
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sleep difficulties and unpleasant dreams have been reported lasting for several weeks. Polysomnography studies demonstrate reduced slow-wave sleep and increased REM sleep during this phase. Opioids. Opioids may produce an increase in sleepiness and in subjective depth of sleep, and reduced REM sleep, during acute short-te rm use. With continued administration, tol- erance to the sedative effects of opioids de velops and there are co mplaints of insomnia. Consistent with their respiratory depressant effects, opioids exacerbate sleep apnea. Sedative, hypnotic, or anxiolytic substances. Sedatives, hypnotics, and anxiolytics (e.g., barbiturates, benzodiazepines receptor agon ists, meprobamate, glutethimide, methypry- lon) have similar effects as opioids on sleep. During acute intoxication, sedative-hypnotic drugs produce the expected incr ease in sleepiness and decrea se in wakefulness. Chronic use (particularly of barbiturates and the ol der nonbarbiturate, no nbenzodiazepine drugs) may cause tolerance with subsequent return of insomnia. Daytime sleepiness may occur. Sedative-hypnotic drugs can increase the frequency and severity of obstructive sleep ap- nea events. Parasomnias are associated with us e of benzodiazepine receptor agonists, es- pecially when these medications are taken at higher doses and when they are combined with other sedative drugs. Ab rupt discontinuation of chronic sedative, hypnotic, or anx- iolytic use can lead to withdrawal but mo re commonly rebound insomnia, a condition of an exacerbation of insomnia upon drug disc ontinuation for 1–2 days reported to occur
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even with short-term use. Sedative, hypnotic, or anxiolytic drugs with short durations of action are most likely to produce complain ts of rebound insomnia, whereas those with longer durations of action are more often asso ciated with daytime sl eepiness. Any sedative, hypnotic, or anxiolytic drug can potentiall y cause daytime sedation, withdrawal, or re- bound insomnia. Amphetamines and related substances and other stimulants. Sleep disorders induced by amphetamine and related substances and other stimulants are characterized by insomnia
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bound insomnia. Amphetamines and related substances and other stimulants. Sleep disorders induced by amphetamine and related substances and other stimulants are characterized by insomnia during intoxication and excessi ve sleepiness during withdrawal. During acute intoxication, stimulants reduce the total amount of sleep, incr ease sleep latency and sleep continuity distur- bances, and decrease REM sleep. Slow-wave slee p tends to be reduced. During withdrawal from chronic stimulant use, there is both pr olonged nocturnal sleep duration and excessive daytime sleepiness. Multiple sleep latency te sts may show increased daytime sleepiness dur-
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418 Sleep-Wake Disorders ing the withdrawal phase. Drugs like 3,4- methylenedioxymethamphetamine (MDMA; “ec- stasy”) and related substances lead to restless and disturbed sleep with in 48 hours of intake; frequent use of these compounds is associated with persisting symptoms of anxiety, depres- sion, and sleep disturbances, even during longer-term abstinence. Tobacco. Chronic tobacco consumption is associat ed primarily with symptoms of insom- nia, decreased slow-wave sleep with a reductio n of sleep efficiency, and increased daytime sleepiness. Withdrawal from tobacco can lead to impaired sleep. Individuals who smoke heavily may experience regular nocturnal awakenings caused by tobacco craving. Other or unknown substances/medications. Other substances/medications may pro- duce sleep disturbances, particularly medications that affect the central or autonomic nervous systems (e.g., adrenergic agonists an d antagonists, dopamine agonists and antag- onists, cholinergic agonists an d antagonists, serotonergic agonists and antagonists, anti- histamines, corticosteroids). Development and Course Insomnia in children can be identified by eith er a parent or the child. Often the child has a clear sleep disturbance associated with initiation of a medication but may not report symptoms, although parents observe the slee p disturbances. The use of some illicit sub- stances (e.g., cannabis, ecstasy) is prevalen t in adolescence and early adulthood. Insomnia or any other sleep disturbance encountered in this age group should prompt careful con- sideration of whether the sleep disturbance is due to consumption of these substances. Help-seeking behavior for the sleep disturbance in these age groups is limited, and thus
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Help-seeking behavior for the sleep disturbance in these age groups is limited, and thus corroborative report may be elic ited from a parent, caregiver, or teacher. Older individuals take more medications and are at increased risk for developing a substance/medication- induced sleep disorder. They may interpret sleep disturbance as part of normal aging and fail to report symptoms. Individuals with ma jor neurocognitive disorder (e.g., dementia) are at risk for substance/me dication-induced sleep disord ers but may not report symp- toms, making corroborative report from caregiver(s) particularly important. Risk and Prognostic Factors Risk and prognostic factors involved in subs tance abuse/dependence or medication use are normative for certain age groups. They are relevant for, and likely applicable to, the type of sleep disturbance encountered (see th e chapter “Substance-Related and Addictive Disorders” for descriptions of resp ective substance use disorders). Temperamental. Substance use generally precipitates or accompanies insomnia in vul- nerable individuals. Thus, presence of insomnia in response to stress or change in sleep en- vironment or timing can represent a risk for developing substance/medication-induced sleep disorder. A similar risk may be present for individuals with other sleep disorders (e.g., individuals with hypersomnia who use stimulants). Culture-Related Diagnostic Issues The consumption of substances, including pres cribed medications, may depend in part on cultural background and specif ic local drug regulations. Gender-Related Diagnostic Issues Gender-specific prevalences (i.e., females affected more than males at a ratio of about 2:1) exist for patterns of consumption of some substances (e.g., alcohol). The same amount and duration of consumption of a given substance may lead to highly different sleep-related outcomes in
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of consumption of a given substance may lead to highly different sleep-related outcomes in males and females based on, for example, gender -specific differences in hepatic functioning.
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Substance/Medication-Induced Sleep Disorder 419 Diagnostic Markers Each of the substance/medica tion-induced sleep disorders produces electroencephalo- graphic sleep patterns that are as sociated with, but cannot be considered diagnostic of, other disorders. The electroencephalo graphic sleep profile for each substance is related to the stage of use, whether intake/intoxication, chronic use, or wi thdrawal following discontinu- ation of the substance. All-night polysomnography can help define the severity of insomnia complaints, while the multiple sleep latency test provides information about the severity of daytime sleepiness. Monitoring of nocturnal respiration and periodic limb movements with polysomnography may verify a substance’s im pact on nocturnal breathing and motor be- havior. Sleep diaries for 2 weeks and actigraphy are considered helpful in confirming the presence of substance/medication-induced sleep disorder. Dr ug screening ca n be of use when the individual is not aware or unwilling to relate information about substance intake. Functional Consequences of Substance/Medication-I nduced Sleep Disorder While there are many functional consequences associated with sleep disorders, the only unique consequence for substa nce/medication-induced sleep disorder is increased risk for relapse. The degree of sleep disturbance during alcohol withdrawal (e.g., REM sleep rebound predicts risk of relapse of drinking ). Monitoring of sleep quality and daytime sleepiness during and after withdrawal may provide clinically meaningful information on whether an individual is at increased risk for relapse. Differential Diagnosis Substance intoxication or substance withdrawal. Sleep disturbances are commonly en- countered in the context of substance intoxi cation or substance discontinuation/with- drawal. A diagnosis of substance/medication-induced sleep disorder should be made
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drawal. A diagnosis of substance/medication-induced sleep disorder should be made instead of a diagnosis of substance intoxicati on or substance withdrawal only when the sleep disturbance is predominant in the clinic al picture and is suffi ciently severe to war- rant independent clinical attention. Delirium. If the substance/medication-induced sl eep disturbance occurs exclusively dur- ing the course of a delirium, it is not diagnosed separately. Other sleep disorders. A substance/medication-induced sl eep disorder is distinguished from another sleep disorder if a substance/medication is judged to be etiologically related to the symptoms. A su bstance/medication-induc ed sleep disorder attributed to a prescribed medication for a mental disorder or medical co ndition must have its onset while the individual is receiving the medication or during disconti nuation, if there is a discontinuation/with- drawal syndrome associated with the medication. Once treatment is discontinued, the sleep disturbance will usually remit within days to several weeks. If symptoms persist beyond 4 weeks, other causes for the sleep disturbanc e–related symptoms should be considered. Not infrequently, individuals with another sleep di sorder use medications or drugs of abuse to self-medicate their symptoms (e.g., alcohol fo r management of insomn ia). If the substance/ medication is judged to play a significant role in the exacerbation of the sleep disturbance, an additional diagnosis of a substance/medicati on-induced sleep disorder may be warranted. Sleep disorder due to an other medical condition. Substance/medication-induced sleep disorder and sleep disorder associated with another medical condition may produce sim- ilar symptoms of insomnia, daytime sleepiness, or a parasomnia. Many individuals with other medical conditions that cause sleep disturbance are treated with medications that
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other medical conditions that cause sleep disturbance are treated with medications that may also cause sleep disturbances. The chrono logy of symptoms is the most important fac- tor in distinguishing between th ese two sources of sleep symptoms. Difficulties with sleep that clearly preceded the use of any medication for treatment of a medical condition would
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420 Sleep-Wake Disorders suggest a diagnosis of sleep disorder associated with another medical condition. Con- versely, sleep symptoms that appear only afte r the initiation of a particular medication/ substance suggest a substance/medication-ind uced sleep disorder. If the disturbance is comorbid with another medical condition and is also exacerbated by substance use, both diagnoses (i.e., sleep disorder associated with another medical condition and substance/ medication-induced sleep disorder) are given. When there is insuffici ent evidence to de- termine whether the sleep distur bance is attributable to a substance/medication or to an- other medical condition or is primary (i.e., not due to either a substance/medication or another medical condition), a diagnosis of ot her specified sleep-wake disorder or unspec- ified sleep-wake disorder is indicated. Comorbidity See the “Comorbidity” sections for other sleep disorders in this chapter, including insom- nia, hypersomnolence, centra l sleep apnea, sleep-related hypoventilation, and circadian rhythm sleep-wake disorders, shift work type. Relationship to Internat ional Classification of Sleep Disorders The International Classifica tion of Sleep Disorders, 2nd Edition (ICSD-2), lists sleep disorders “due to drug or substance” under their respective phenotypes (e.g., insomnia, hypersomnia). Other Specified Insomnia Disorder 780.52 (G47.09) This category applies to presentations in which symptoms characteristic of insomnia disorder that cause clinically significant distress or impairment in social, occupational, or other important areas of functioning predominate but do not meet th e full criteria for insomnia disorder or any of the disorders in the sleep-wake disorders diagnostic class. The other specified insomnia dis-
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of the disorders in the sleep-wake disorders diagnostic class. The other specified insomnia dis- order category is used in situations in which the clinician chooses to communicate the specific reason that the presentation does not meet the criteria for insomnia disorder or any specific sleep-wake disorder. This is done by recording “other specified insomnia disorder” followed by the specific reason (e.g., “brief insomnia disorder”). Examples of presentations that can be specified using the “other specified” designation include the following: 1.Brief insomnia disorder: Duration is less than 3 months. 2.Restricted to no nrestorative sleep: Predominant complaint is nonrestorative sleep unaccompanied by other sleep symptoms such as difficulty falling asleep or remaining asleep. Unspecified Insomnia Disorder 780.52 (G47.00) This category applies to presentations in which symptoms characteristic of insomnia disor- der that cause clinically significant distress or impairment in social, occupational, or other important areas of functioning predominate but do not meet the full criteria for insomnia dis- order or any of the disorders in the sleep-wake disorders diagnostic class. The unspecified
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Other Specified Hypersomnolence Disorder 421 insomnia disorder category is used in situations in which the clinician chooses not to specify the reason that the criteria are not met for insomnia disorder or a specific sleep-wake dis- order, and includes presentations in which there is insufficient information to make a more specific diagnosis. Other Specified Hypersomnolence Disorder 780.54 (G47.19) This category applies to presentations in which symptoms characteristic of hypersomno- lence disorder that cause clinically signific ant distress or impairment in social, occupation- al, or other important areas of functioning predominate but do not meet the full criteria for hypersomnolence disorder or any of the disorders in the sleep-wake disorders diagnostic class. The other specified hypersomnolence disorder category is used in situations in which the clinician chooses to communicate the specific reason that the presentation does not meet the criteria for hypersomnolence diso rder or any specific sleep-wake disorder. This is done by recording “other specified hypersomnolence disorder” followed by the spe- cific reason (e.g., “brief-duration hyperso mnolence,” as in Kleine-Levin syndrome). Unspecified Hypersomnolence Disorder 780.54 (G47.10) This category applies to presentations in which symptoms characteristic of hypersomno- lence disorder that cause clinically signific ant distress or impairment in social, occupation- al, or other important areas of functioning predominate but do not meet the full criteria for hypersomnolence disorder or any of the disorders in the sleep-wake disorders diagnostic class. The unspecified hypersomnolence disorder category is used in situations in which the clinician chooses not to specify the reason that the criteria are not met for hypersom-
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the clinician chooses not to specify the reason that the criteria are not met for hypersom- nolence disorder or a specific sleep-wake disorder, and includes presentations in which there is insufficient information to make a more specific diagnosis. Other Specified Sleep-Wake Disorder 780.59 (G47.8) This category applies to presentations in which symptoms characteristic of a sleep-wake disorder that cause clinically significant distress or impairment in social, occupational, or other important areas of functioning predominate but do not meet the full criteria for any of the disorders in the sleep-wake disorders diagnostic class and do not qualify for a diagno- sis of other specified insomnia disorder or other specified hypersomnolence disorder. The other specified sleep-wake disorder category is used in situations in which the clinician chooses to communicate the specific reason that the presentation does not meet the criteria for any specific sleep-wake disorder . This is done by recording “other specified sleep-wake disorder” followed by the specific reason (e.g., “repeated arousals during rapid eye movement sleep without polysomnography or history of Parkinson’s disease or other synucleinopathy”).
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422 Sleep-Wake Disorders Unspecified Sleep-Wake Disorder 780.59 (G47.9) This category applies to presentations in which symptoms characteristic of a sleep-wake disorder that cause clinically significant distress or impairment in social, occupational, or other important areas of functioning predominate but do not meet the full criteria for any of the disorders in the sleep-wake disorders di agnostic class and do not qualify for a diagno- sis of unspecified insomnia disorder or unspecified hypersomnolence disorder. The un- specified sleep-wake disorder category is used in situations in which the clinician chooses not to specify the reason that the criteria are not met for a specific sleep-wake disorder, and includes presentations in which there is insufficient information to make a more spe- cific diagnosis.
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423Sexual Dysfunctions Sexual dysfunctions include delayed ejaculation, er ectile disorder, female orgasmic disorder, female sexual intere st/arousal disorder, genito-pelvic pain/penetration disorder, male hypoactive sexual desire disorder, premature (early) ejac ulation, substance/medication- induced sexual dysfunction, other specified se xual dysfunction, and unspecified sexual dys- function. Sexual dysfunctions ar e a heterogeneous group of disorders that are typically char- acterized by a clinically significant disturbance in a person’s ability to respond sexually or to experience sexual pleasure. An individual may have several sexual dysfunctions at the same time. In such cases, all of the dysfunctions should be diagnosed. Clinical judgment should be used to determin e if the sexual difficulties are the result of inadequate sexual stimulation; in these cases, there may still be a need for care, but a di- agnosis of a sexual dysfunctio n would not be made. These cases may include, but are not limited to, conditions in which lack of know ledge about effective stimulation prevents the experience of arousal or orgasm. Subtypes are used to designate the onset of the difficulty. In many individuals with sexual dysfunctions, the time of onset may in dicate different etiolo gies and interventions. Lifelong refers to a sexual problem that has been present from first sexu al experiences, and acquired applies to sexual disorders that develop after a period of relatively normal sexual function. Generalized refers to sexual difficulties that are not limited to certain types of stimulation, situatio ns, or partners, and situational refers to sexual difficulties that only oc- cur with certain types of stimul ation, situations, or partners. In addition to the lifelong/ acquired and generalized/situational subtypes, a number
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In addition to the lifelong/ acquired and generalized/situational subtypes, a number of factors must be considered during the asse ssment of sexual dysfunction, given that they may be relevant to etiology and/or treatment, and that may contribute, to varying degrees, across individuals: 1) partner factors (e.g., pa rtner’s sexual problems; partner’s health sta- tus); 2) relationship factors (e.g., poor communication; disc repancies in desire for sexual activity); 3) individual vulnerability factors (e .g., poor body image; history of sexual or emo- tional abuse), psychiatric comorbidity (e.g., depr ession, anxiety), or stre ssors (e.g., job loss, bereavement); 4) cultural or religious factors (e.g., inhibitions re lated to prohibitions against sexual activity or pleasure; attitudes toward sexuality); and 5) medical factors relevant to prognosis, course, or treatment. Clinical judgment about the di agnosis of sexual dysfunction should take into consideration cultural factors that may influe nce expectations or engender pr ohibitions about the experience of sexual pleasure. Aging may be associated wi th a normative decrease in sexual response. Sexual response has a requisite biological underpinning, yet is usually experienced in an intrapersonal, interpersonal, and cultural context. Thus, sexual fu nction involves a com- plex interaction among biological, sociocultural, and psychological factors. In many clinical contexts, a precise understanding of the etiolo gy of a sexual problem is unknown. Nonethe- less, a sexual dysfunction diagnosis requires ruling out problems that are better explained by a nonsexual mental disorder, by the effects of a substance (e.g., drug or medication), by
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a medical condition (e.g., due to pelvic nerve damage), or by severe relationship distress, partner violence, or other stressors. If the sexual dysfunction is mostly explainabl e by another nonsexual mental disorder (e.g., depressive or bipolar disorder, anxiety disord er, posttraumatic stress disorder, psychotic dis-
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424 Sexual Dysfunctions order), then only the other mental disorder diagnosis should be made. If the problem is thought to be better explained by the use/misuse or discontinuation of a drug or substance, it should be diagnosed accordingly as a substance/medication-induced sexual dysfunction. If the sexual dysfunction is attributable to anot her medical condition (e.g., peripheral neuropa- thy), the individual would not receive a psychiatric diagnosis. If severe relationship distress, partner violence, or significant stressors better ex plain the sexual difficulties, then a sexual dys- function diagnosis is not made, but an appropriat e V or Z code for the relationship problem or stressor may be listed. In many cases, a precise etiological relationship between another con- dition (e.g., a medical condition) and a sexual dysfunction cannot be established. Delayed Ejaculation Diagnostic Criteria 302.74 (F52.32) A. Either of the following symptoms must be experienced on almost all or all occasions (approximately 75%–100%) of partnered sexual activity (in identified situational con- texts or, if generalized, in all contexts), and without the individual desiring delay: 1. Marked delay in ejaculation. 2. Marked infrequency or absence of ejaculation. B. The symptoms in Criterion A have persist ed for a minimum duration of approximately 6 months. C. The symptoms in Criterion A cause clinically significant distress in the individual. D. The sexual dysfunction is not better explained by a nonsexual mental disorder or as a consequence of severe relationship distress or other significant stressors and is not at- tributable to the effects of a substance/medication or another medical condition. Specify whether: Lifelong: The disturbance has been present since the individual became sexually active.
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Lifelong: The disturbance has been present since the individual became sexually active. Acquired: The disturbance began after a period of relatively normal sexual function. Specify whether: Generalized: Not limited to certain types of stimulation, situations, or partners. Situational: Only occurs with certain types of stimulation, situations, or partners. Specify current severity: Mild: Evidence of mild distress over the symptoms in Criterion A. Moderate: Evidence of moderate distress ov er the symptoms in Criterion A. Severe: Evidence of severe or extreme distress over the symptoms in Criterion A. Diagnostic Features The distinguishing feature of delayed ejaculation is a marked delay in or inability to achieve ejaculation (Cri terion A). The man reports difficulty or inability to ejaculate de- spite the presence of adequate sexual stimul ation and the desire to ejaculate. The present- ing complaint usually involves partnered sexual activity. In most cases, the diagnosis will be made by self-report of the individual. The defini tion of “delay” does not have precise boundaries, as there is noconsensus as to what constitutes a reasonable time to reach or- gasm or what is unacceptably long fo r most men and their sexual partners. Associated Features Supporting Diagnosis The man and his partner may report prolonged thrusting to achieve or gasm to the point of exhaustion or genital discomfo rt and then ceasing efforts. Some men may report avoiding
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Delayed Ejaculation 425 sexual activity because of a re petitive pattern of difficulty ejaculating. Some sexual partners may report feeling less sexually attractive because their partner ca nnot ejaculate easily. In addition to the subtypes “lifelong/acqu ired” and “generalized/situational,” the fol- lowing five factors must be considered during assessment and diagnosis of delayed ejacu- lation, given that they may be relevant to etiology and/or treatment: 1) partner factors (e.g., partner’s sexual pr oblems, partner’s health status); 2) relationship factors (e.g., poor com- munication, discrepancies in desire for sexual activity); 3) individual vulnerability factors (e.g., poor body image; history of sexual or emotional abuse), psychiatric comorbidity (e.g., depression, anxiety), or stressor s (e.g., job loss, bereavement); 4) cultural/religious factors (e.g., inhibitions related to prohibitions agains t sexual activity; attitudes toward sexuality); and 5) medical factors relevant to prognosis, course, or treatment. Each of these factors may contribute differently to the presenting symptoms of different men with this disorder. Prevalence Prevalence is unclear because of the lack of a precise definition of this syndrome. It is the least common male sexual complaint. Only 75% of men report always ejaculating during sexual activity, and less than 1% of men will complain of problems with reaching ejacula- tion that last more than 6 months. Development and Course Lifelong delayed ejaculation begins with ea rly sexual experiences and continues through- out life. By definition, acquired delayed ejaculation begins after a period of normal sexual function. There is minimal evidence concerni ng the course of acquired delayed ejacula-
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function. There is minimal evidence concerni ng the course of acquired delayed ejacula- tion. The prevalence of delaye d ejaculation appears to remain relatively constant until around age 50 years, when the incidence begins to increase significantly. Men in their 80s report twice as much difficulty ejac ulating as men younger than 59 years. Risk and Prognostic Factors Genetic and physiological. Age-related loss of the fast-conducting peripheral sensory nerves and age-related decreased sex steroid secretion may be associated with the increase in delayed ejaculation in men older than 50 years. Culture-Related Diagnostic Issues Complaints of ejaculatory delay vary across countries and cultures. Such complaints are more common among men in Asian population s than in men living in Europe, Australia, or the United States. This variation may be attributable to cultural or genetic differences between cultures. Functional Consequences of Delayed Ejaculation Difficulty with ejaculation may contribute to difficulties in conception. Delayed ejacula- tion is often associated with considerable psychological distre ss in one or both partners. Differential Diagnosis Another medical condition. The major differential diagnosis is between delayed ejacu- lation fully explained by another medical illness or injury and delayed ejaculation with a psychogenic, idiopathic, or combined psycho logical and medical etiology. A situational aspect to the complaint is suggestive of a psychological basis for the problem (e.g., men who can ejaculate during sexual activity with one sex but not the other; men who can ejac- ulate with one partner but not another of the same sex; men with paraphilic arousal pat-
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426 Sexual Dysfunctions terns; men who require highly ritualized ac tivity to ejaculate during partnered sexual activity). Another medical illness or injury ma y produce delays in ejaculation independent of psychological issues. For example, inability to ejaculate can be caused by interruption of the nerve supply to the genitals , such as can occur after traumatic surgical injury to the lumbar sympathetic ganglia, abdominoperitoneal surgery, or lumbar sympathectomy. Ejaculation is thought to be under autonomic nervous system control involving the hypo- gastric (sympathetic) and pudendal (parasym pathetic) nerves. A number of neurodegen- erative diseases, such as multiple sclerosis and diabetic and alcoholic neuropathy, can cause inability to ejaculate. Delayed ejaculat ion should also be diffe rentiated from retro- grade ejaculation (i.e., ejaculation into the bladder), which may follow transurethral pros- tatic resection. Substance/medication use. A number of pharmacological agents, such as antidepres- sants, antipsychotics, alpha sympathetic drugs, and opioid drugs, can cause ejaculatory problems. Dysfunction with orgasm. It is important in the history to ascertain whether the com- plaint concerns delayed ejaculation or the sensation of orgasm, or both. Ejaculation occurs in the genitals, whereas the experience of orga sm is believed to be primarily subjective. Ejaculation and orgasm usually occur together but not always. For example, a man with a normal ejaculatory pattern may complain of decreased pleasure (i.e., anhedonic ejacula- tion). Such a complaint would not be coded as delayed ejaculation but could be coded as other specified sexual dysfunction or unspecified sexual dysfunction. Comorbidity
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other specified sexual dysfunction or unspecified sexual dysfunction. Comorbidity There is some evidence to sugge st that delayed ejaculation may be more common in severe forms of major de pressive disorder. Erectile Disorder Diagnostic Criteria 302.72 (F52.21) A. At least one of the three following symptoms must be experienced on almost all or all (approximately 75%–100%) occasions of sexual activity (in identified situational con- texts or, if generalized, in all contexts): 1. Marked difficulty in obtaining an erection during sexual activity. 2. Marked difficulty in maintaining an erection until the completion of sexual activity. 3. Marked decrease in erectile rigidity. B. The symptoms in Criterion A have persist ed for a minimum duration of approximately 6 months. C. The symptoms in Criterion A cause clinically significant distress in the individual. D. The sexual dysfunction is not better explained by a nonsexual mental disorder or as a consequence of severe relationship distress or other significant stressors and is not at- tributable to the effects of a substance/medication or another medical condition. Specify whether: Lifelong: The disturbance has been present since the individual became sexually ac- tive. Acquired: The disturbance began after a period of relatively normal sexual function. Specify whether: Generalized: Not limited to certain types of stimulation, situations, or partners. Situational: Only occurs with certain types of stimulation, situations, or partners.
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Erectile Disorder 427 Specify current severity: Mild: Evidence of mild distress over the symptoms in Criterion A. Moderate: Evidence of moderate distress ov er the symptoms in Criterion A. Severe: Evidence of severe or extreme distress over the symptoms in Criterion A. Diagnostic Features The essential feature of erectile disorder is the repeated failure to obtain or maintain erec- tions during partnered sexual activities (Crite rion A). A careful sexual history is necessary to ascertain that the problem has been present for a significant duration of time (i.e., at least approximately 6 months) and occurs on the majo rity of sexual occasions (i.e., at least 75% of the time). Symptoms may occur only in sp ecific situations involving certain types of stimulation or partners, or they may occur in a generalized manner in all types of situa- tions, stimulatio n, or partners. Associated Features Supporting Diagnosis Many men with erectile disorder may have lo w self-esteem, low self-c onfidence, and a de- creased sense of masculinity, and may experience depressed affect. Fear and/or avoid- ance of future sexual encounters may occu r. Decreased sexual satisfaction and reduced sexual desire in the individual’s partner are common. In addition to the subtypes “lifelong/acqu ired” and “generalized/situational,” the fol- lowing five factors must be considered during assessment and diagnosis of erectile disorder given that they may be relevant to etiology and/or treatment: 1) partner factors (e.g., part- ner’s sexual problems, partner’s health status); 2) relationship factors (e.g., poor communi- cation, discrepancies in desire for sexual activity); 3) in dividual vulnerability factors (e.g.,
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poor body image, history of sexual or emotional abuse), psychiatric comorbidity (e.g., de- pression, anxiety), or stressors (e.g., job loss, bereavement); 4) cultural/religious factors (e.g., inhibitions related to prohibitions against sexual activity; attitudes toward sexuality); and 5) medical factors relevant to prognosis, cour se, or treatment. Each of these factors may con- tribute differently to the pr esenting symptoms of differe nt men with this disorder. Prevalence The prevalence of lifelong vers us acquired erectile disorder is unknown. There is a strong age-related increase in both prevalence and incidence of problems with erection, particu- larly after age 50 years. Approximately 13%–21% of men ages 40–80 years complain of oc- casional problems with erections. Approxim ately 2% of men younger than age 40–50 years complain of frequent problems with erecti ons, whereas 40%–50% of men older than 60–70 years may have significant problems with erec tions. About 20% of me n fear erectile prob- lems on their first sexual experience, wherea s approximately 8% experienced erectile prob- lems that hindered penetration duri ng their first sexual experience. Development and Course Erectile failure on first sexual attempt has been found to be related to having sex with a pre- viously unknown partner, concomit ant use of drugs or alcohol, not wanting to have sex, and peer pressure. There is minimal evidence rega rding the persistence of such problems after the first attempt. It is assumed that most of these problems spontane ously remit without pro- fessional intervention, but some men may contin ue to have episodic problems. In contrast,
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fessional intervention, but some men may contin ue to have episodic problems. In contrast, acquired erectile disorder is of ten associated with biological factors such as diabetes and car- diovascular disease. Acquired er ectile disorder is likely to be persistent in most men. The natural history of lifelong erectile disorder is unknown. Clinical observation sup- ports the association of lifelong erectile diso rder with psychological factors that are self-
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428 Sexual Dysfunctions limiting or responsive to ps ychological interventions, wher eas, as noted above, acquired erectile disorder is more likely to be related to biological factors and to be persistent. The incidence of erectile disorder increases with age. A minority of men diagnosed as having moderate erectile failure ma y experience spontaneous remission of symptoms without medical intervention. Distress as sociated with erectile disord er is lower in older men as compared with younger men. Risk and Prognostic Factors Temperamental. Neurotic personality traits may be associated with erectile problems in col- lege students, and submissive personality traits may be associated with erectile problems in men age 40 years and older. Alexithymia (i.e., deficits in cognitive processing of emotions) is common in men diagnosed with “psychogenic” er ectile dysfunction. Erectile problems are common in men diagnosed with depression and posttraumatic stress disorder. Course modifiers. Risk factors for acquired erectile disorder include age, smoking to- bacco, lack of physical exercise, diabetes, and decreased desire. Culture-Related Diagnostic Issues Complaints of erectile disorder have been foun d to vary across countries. It is unclear to what extent these differences represent differ ences in cultural expectations as opposed to genuine differences in the fr equency of erectile failure. Diagnostic Markers Nocturnal penile tumescence testing and meas ured erectile turgidity during sleep can be employed to help differentiate organic from psychogenic erectile problems on the as- sumption that adequate erections during ra pid eye movement sleep indicate a psycholog- ical etiology to the problem. A number of other diagnostic procedures may be employed depending on the clinician’s assessment of their relevance given the individual’s age, co-
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depending on the clinician’s assessment of their relevance given the individual’s age, co- morbid medical problems, and clinical pres entation. Doppler ultrasonography and intra- vascular injection of vasoactive drugs, as we ll as invasive diagnostic procedures such as dynamic infusion cavernosography, can be used to assess vascular integrity. Pudendal nerve conduction studies, including somato sensory evoked potentials, can be employed when a peripheral neuropathy is suspected. In men also complaining of decreased sexual desire, serum bioavailable or free testosterone is frequently assessed to determine if the difficulty is secondary to endocrinological factors. Thyroid function may also be assessed. Determination of fasting serum glucose is useful to screen for the pres ence of diabetes mel- litus. The assessment of serum lipids is important, as erectile disorder in men 40 years and older is predictive of the future risk of coronary artery disease. Functional Consequences of Erectile Disorder Erectile disorder can interfere with fertility and produce both indivi dual and interpersonal distress. Fear and/or avoidance of sexual en counters may interfere with the ability to de- velop intimate relationships. Differential Diagnosis Nonsexual mental disorders. Major depressive disorder and erectile disorder are closely associated, and erectile diso rder accompanying severe de pressive disorder may occur. Normal erectile function. The differential should include consideration of normal erec- tile function in men with excessive expectations.
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Female Orgasmic Disorder 429 Substance/medication use. Another major differential diagnosis is whether the erectile problem is secondary to substa nce/medication use. An onset that coincides with the be- ginning of substance/medication use and that dissipates with discontinuation of the sub- stance/medication or dose reduction is su ggestive of a substance/medication-induced sexual dysfunction. Another medical condition. The most difficult aspect of the differential diagnosis of erec- tile disorder is ruling out erec tile problems that are fully explained by medical factors. Such cases would not receive a diagnosis of a ment al disorder. The distinction between erectile disorder as a mental disorder and erectile dy sfunction as the result of another medical con- dition is usually unclear, and many cases will have complex, interactive biological and psy- chiatric etiologies. If the individual is older than 40–50 years and/or has concomitant medical problems, the differential diagnosis should include medical etiologies, especially vascular disease. The presence of an organic disease known to cause erectile problems does not confirm a causal relationship. For exampl e, a man with diabetes mellitus can develop erectile disorder in response to psychological stress. In general, erectile dysfunction due to organic factors is generalized and gradual in onset. An exception would be erectile problems after traumatic injury to the nervous innervation of the genital organs (e.g., spinal cord injury). Erectile problems that are situational and inco nsistent and that have an acute onset after a stressful life event are most often due to psychological events. An age of less than 40 years is also suggestive of a psychologi cal etiology to the difficulty. Other sexual dysfunctions. Erectile disorder may coexist with premature (early) ejacu- lation and male hypoactive sexual desire disorder.
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lation and male hypoactive sexual desire disorder. Comorbidity Erectile disorder can be comorbid with other sexual diagnoses, such as premature (early) ejaculation and male hypoactive sexual desire disorder, as well as with anxiety and de- pressive disorders. Erectile disorder is co mmon in men with lowe r urinary tract symptoms related to prostatic hypertrophy. Erectile diso rder may be comorbid with dyslipidemia, car- diovascular disease, hypogonadism, multiple sclerosis, diabetes mellitus, and other diseases that interfere with the vascular, neurological , or endocrine function necessary for normal erectile function. Relationship to Internationa l Classification of Diseases Erectile response is coded as failure of genital response in ICD-10 (F2.2). Female Orgasmic Disorder Diagnostic Criteria 302.73 (F52.31) A. Presence of either of the following symptoms and experienced on almost all or all (ap- proximately 75%–100%) occasions of sexual ac tivity (in identified situational contexts or, if generalized, in all contexts): 1. Marked delay in, marked infrequency of, or absence of orgasm. 2. Markedly reduced intensity of orgasmic sensations. B. The symptoms in Criterion A have persist ed for a minimum duration of approximately 6 months. C. The symptoms in Criterion A cause clinically significant distress in the individual. D. The sexual dysfunction is not better explained by a nonsexual mental disorder or as a consequence of severe relationship distress (e.g., partner violence) or other significant
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430 Sexual Dysfunctions stressors and is not attributable to the effects of a substance/medication or another medical condition. Specify whether: Lifelong: The disturbance has been present since the individual became sexually active. Acquired: The disturbance began after a period of relatively normal sexual function. Specify whether: Generalized: Not limited to certain types of stimulation, situations, or partners. Situational: Only occurs with certain types of stimulation, situations, or partners. Specify if: Never experienced an orgasm under any situation. Specify current severity: Mild: Evidence of mild distress over the symptoms in Criterion A. Moderate: Evidence of moderate distress ov er the symptoms in Criterion A. Severe: Evidence of severe or extreme distress over the symptoms in Criterion A. Diagnostic Features Female orgasmic disorder is characterize d by difficulty experiencing orgasm and/or markedly reduced intensity of orgasmic sensat ions (Criterion A). Women show wide vari- ability in the type or intensity of stimulation that elicits orgasm. Similarly, subjective descrip- tions of orgasm are extremely varied, suggesti ng that it is experienced in very different ways, both across women and on different occasions by the same woman. For a diagnosis of female orgasmic disorder, symptoms must be experienced on almost all or all (approx- imately 75%–100%) occasions of sexual activity (in identified situational contexts or, if generalized, in all contexts) and have a mi nimum duration of approximately 6 months. The use of the minimum severity and duration criteria is intended to distinguish transient orgasm difficulties from more persistent orgasmic dysfunction. The inclusion of “approx- imately” in Criterion B allows for clinician judgment in cases in which symptom duration does not meet the recommended 6-month threshold.
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does not meet the recommended 6-month threshold. For a woman to have a diagnosis of female or gasmic disorder, clinically significant dis- tress must accompany the symptoms (Criterion C). In many cases of orgasm problems, the causes are multifactorial or cannot be determined. If female org asmic disorder is deemed to be better explained by another mental diso rder, the effects of a substance/medication, or a medical condition, then a diagnosis of fe male orgasmic disorder would not be made. Finally, if interpersonal or significant contextual factors, such as severe relationship dis- tress, intimate partner violence, or other signi ficant stressors, are present, then a diagnosis of female orgasmic diso rder would not be made. Many women require clitoral stimulation to reach orgasm, and a relatively small pro- portion of women report that they always ex perience orgasm during penile-vaginal inter- course. Thus, a woman’s experiencing orgasm through clitoral stim ulation but not during intercourse does not meet criteria for a clinic al diagnosis of female orgasmic disorder. It is also important to consider whether orgasmic difficulties are the result of inadequate sex- ual stimulation; in these cases, there may still be a need for care, but a diagnosis of female orgasmic disorder would not be made. Associated Features Supporting Diagnosis Associations between specific patterns of personality traits or psychopathology and orgas- mic dysfunction have generally not been su pported. Compared with women without the disorder, some women with female orgasmic disorder may have greater difficulty com- municating about sexual issues. Overall sexual satisfaction, however, is not strongly cor- related with orgasmic experien ce. Many women report high le vels of sexual satisfaction
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Female Orgasmic Disorder 431 despite rarely or never experiencing orgasm . Orgasmic difficulties in women often co- occur with problems related to sexual interest and arousal. In addition to the subtypes “lifelong/acquired” and “generalized/situational,” the fol- lowing five factors must be considered during assessment and diagnosis of female orgas- mic disorder given that they may be relevant to etiology and/or treatment: 1) partner factors (e.g., partner’s sexual problems, partner’s health status); 2) relationship factors (e.g., poor communication, discre pancies in desire for sexual activity); 3) individual vul- nerability factors (e.g., poor body image, history of sexual or emotional abuse), psychiatric comorbidity (e.g., depression, anxiety), or st ressors (e.g., job loss, bereavement); (4) cul- tural/religious factors (e.g., inhibitions relate d to prohibitions against sexual activity; attitudes toward sexuality); and 5) medical fact ors relevant to prognosis, course, or treat- ment. Each of these factors may contribute differently to the presenting symptoms of dif- ferent women with this disorder. Prevalence Reported prevalence rates for female orgasmic problems in women vary widely, from 10% to 42%, depending on multiple factors (e.g., ag e, culture, duration, and severity of symp- toms); however, these estimates do not take into account the presence of distress. Only a proportion of women experiencing orgasm di fficulties also report associated distress. Variation in how symptoms are assessed (e.g., the duration of symptoms and the recall pe- riod) also influence prevalence rates. Approx imately 10% of women do not experience or-
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gasm throughout their lifetime. Development and Course By definition, lifelong female orgasmic disorder indicates that the orgasmic difficulties have always been present, whereas the acquired subtype would be assigned if the woman’s or- gasmic difficulties developed after a pe riod of normal orgasmic functioning. A woman’s first experience of orgasm can occur any time from the prepubertal period to well into adulthood. Women show a more variable pattern in age at first orgasm than do men, and women’s reports of having experien ced orgasm increase with age. Many women learn to experience orgasm as they experien ce a wide variety of stimulation and acquire more knowledge about their bodies. Women’s rates of orgasm consistency (defined as “usually or always” experiencing orgasm) are higher during masturbation than during sexual activity with a partner. Risk and Prognostic Factors Temperamental. A wide range of psychological factors, such as anxiety and concerns about pregnancy, can potentially interfere wi th a woman’s ability to experience orgasm. Environmental. There is a strong association betw een relationship problems, physical health, and mental health and orgasm difficulties in women. Sociocultural factors (e.g., gender role expectations and religious norms) are also important influences on the expe- rience of orgasmic difficulties. Genetic and physiological. Many physiological factors may influence a woman’s expe- rience of orgasm, including medical conditio ns and medications. Conditions such as mul- tiple sclerosis, pelvic nerve da mage from radical hysterectomy, and spinal cord injury can all influence orgasmic functioning in women. Selective serotonin reuptake inhibitors are known to delay or inhibit orgasm in women. Women with vulvovaginal atrophy (charac- terized by symptoms such as vaginal dryness, itching, and pain) are significantly more
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terized by symptoms such as vaginal dryness, itching, and pain) are significantly more likely to report orgasm difficulties than are women without this condition. Menopausal status is not consistently associated with the likelihood of orgasm difficulties. There may be a significant genetic contribution to vari ation in female orgasmic function. However,
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432 Sexual Dysfunctions psychological, sociocultural, and physiological factors likely interact in complex ways to influence women’s experience of orgasm and of orgasm difficulties. Culture-Related Diagnostic Issues The degree to which lack of orgasm in women is regarded as a problem that requires treat- ment may vary depending on cultural context. In addition, women differ in how important orgasm is to their sexual satisfaction. Ther e may be marked sociocultural and generational differences in women’s orgasmic ability. For example, the prevalence of inability to reach or- gasm has ranged from 17.7% (in Northern Europe) to 42.2% (i n Southeast Asia). Diagnostic Markers Although measurable physiological change s occur during female orgasm, including changes in hormones, pelvic floor musculature, and brain activation, there is significant variability in these indicators of orgasm across women. In clinical situations, the diagnosis of female orgasmic disorder is based on a woman’s self-report. Functional Consequences of Female Orgasmic Disorder The functional consequences of female orgasmic disorder are unclear. Although there is a strong association between relationship proble ms and orgasmic difficulties in women, it is unclear whether relationship factors are risk fa ctors for orgasmic difficulties or are conse- quences of those difficulties. Differential Diagnosis Nonsexual mental disorders. Nonsexual mental disorders, such as major depressive disorder, which is characterized by markedly dimi nished interest or pleasure in all, or al- most all, activities, may explain female orgasm ic disorder. If the orgasmic difficulties are better explained by another me ntal disorder, then a diagnosis of female orgasmic disorder would not be made. Substance/medication-induced sexual dysfunction. Substance/medication use may explain the orgasmic difficulties.
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explain the orgasmic difficulties. Another medical condition. If the disorder is due to an other medical condition (e.g., multiple sclerosis, spinal cord injury), then a diagnosis of female orgasmic disorder would not be made. Interpersonal factors. If interpersonal or significant co ntextual factors, such as severe relationship distress, intimate partner violen ce, or other significant stressors, are associ- ated with the orgasmic difficulties, then a diagnosis of female orgasmic disorder would not be made. Other sexual dysfunctions. Female orgasmic disorder may occur in association with other sexual dysfunctions (e.g., female sexual intere st/arousal disorder). Th e presence of another sexual dysfunction does not rule out a diagnosi s of female orgasmic disorder. Occasional or- gasmic difficulties that are short-term or infreq uent and are not accompanied by clinically sig- nificant distress or impairment are not diagno sed as female orgasmic disorder. A diagnosis is also not appropriate if the problems are the result of inadequate sexual stimulation. Comorbidity Women with female orgasmic disorder may have co-occurring sexu al interest/arousal difficulties. Women with diagnoses of other no nsexual mental disorder s, such as major de- pressive disorder, may experience lower sexu al interest/arousal, and this may indirectly increase the likelihood of orgasmic difficulties.
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Female Sexual Interest/Arousal Disorder 433 Female Sexual Interest/Arousal Disorder Diagnostic Criteria 302.72 (F52.22) A. Lack of, or significantly reduced, sexual interest/arousal, as manifested by at least three of the following: 1. Absent/reduced interest in sexual activity. 2. Absent/reduced sexual/erotic thoughts or fantasies. 3. No/reduced initiation of sexual activity, and typically unreceptive to a partner’s at- tempts to initiate. 4. Absent/reduced sexual excitement/pleasure during sexual activity in almost all or all (approximately 75%–100%) sexual encounters (in identified situational contexts or, if generalized, in all contexts). 5. Absent/reduced sexual interest/arousal in response to any internal or external sex- ual/erotic cues (e.g., written, verbal, visual). 6. Absent/reduced genital or nongenital sensations during sexual activity in almost all or all (approximately 75%–100%) sexual encounters (in identified situational con- texts or, if generalized, in all contexts). B. The symptoms in Criterion A have persist ed for a minimum duration of approximately 6 months. C. The symptoms in Criterion A cause clinically significant distress in the individual. D. The sexual dysfunction is not better explained by a nonsexual mental disorder or as a consequence of severe relationship distress (e.g., partner violence) or other significant stressors and is not attributable to the effects of a substance/medication or another medical condition. Specify whether: Lifelong: The disturbance has been present since the individual became sexually active. Acquired: The disturbance began after a period of relatively normal sexual function. Specify whether:
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Specify whether: Generalized: Not limited to certain types of stimulation, situations, or partners. Situational: Only occurs with certain types of stimulation, situations, or partners. Specify current severity: Mild: Evidence of mild distress over the symptoms in Criterion A. Moderate: Evidence of moderate distress ov er the symptoms in Criterion A. Severe: Evidence of severe or extreme distress over the symptoms in Criterion A. Diagnostic Features In assessing female sexual interest/arousal di sorder, interpersonal co ntext must be taken into account. A “desire discrepancy,” in whic h a woman has lower desire for sexual activ- ity than her partner, is not suff icient to diagnose female sexu al interest/arousal disorder. In order for the criteria for the disorder to be met, there must be absence or reduced fre- quency or intensity of at least three of six indicators (Criterion A) for a minimum duration of approximately 6 months (Criterion B). Th ere may be different symptom profiles across women, as well as variability in how sexual in terest and arousal are expressed. For exam- ple, in one woman, sexual interest/arousal di sorder may be expressed as a lack of interest in sexual activity, an absence of erotic or se xual thoughts, and reluct ance to initiate sexual activity and respond to a partner’s sexual invi tations. In another woman, an inability to be- come sexually excited, to resp ond to sexual stimuli with se xual desire, and a correspond-
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434 Sexual Dysfunctions ing lack of signs of physical sexual arousa l may be the primary features. Because sexual desire and arousal frequently coex ist and are elicited in respon se to adequate sexual cues, the criteria for female sexual in terest/arousal disorder take into account that difficulties in desire and arousal often simult aneously characterize the co mplaints of women with this disorder. Short-term changes in sexual interest or arousal are common and may be adaptive responses to events in a woma n’s life and do not represent a sexual dysfunction. Diagnosis of female sexual interest/arousal disorder requires a minimum duration of symptoms of approximately 6 months as a reflection that the symptoms must be a persistent problem. The estimation of persistence may be determin ed by clinical judgment when a duration of 6 months cannot be ascertained precisely. There may be absent or reduced frequency or inte nsity of interest in se xual activity (Crite- rion A1), which was previously termed hypoactive sexual desire disorder. The frequency or inten- sity of sexual and erotic thoughts or fantasie s may be absent or reduced (Criterion A2). The expression of fantasies varies widely across women and may include memories of past sexual experiences. The normative decline in sexual thoughts with age should be taken into account when this criterion is being assessed. Absence or reduced frequency of initiating sexual activ- ity and of receptivity to a partner’s sexual invitations (Criterion A3) is a behaviorally focused criterion. A couple’s beliefs and preferences for sexual initiation patterns are highly relevant to the assessment of this criterion. There may be ab sent or reduced sexual excitement or pleasure during sexual activity in almos t all or all (approximately 75%– 100%) sexual encounters (Cri-
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terion A4). Lack of pleasure is a common presenting clinical complaint in women with low de- sire. Among women who report low sexual desire , there are fewer sexual or erotic cues that elicit sexual interest or arousal (i.e., there is a lack of “responsive desire”). Assessment of the adequacy of sexual stimuli will assist in determining if there is a difficulty with responsive sex- ual desire (Criterion A5). Freque ncy or intensity of genital or nongenital sensations during sex- ual activity may be reduced or absent (Crite rion A6). This may include reduced vaginal lubrication/vasocongestion, but because physiological measures of genital sexual response do not differentiate women who report sexual arousal concerns from those who do not, the self- report of reduced or absent genital or nongenital sensations is sufficient. For a diagnosis of female sexual interest/arousal disorder to be made, clinically signif- icant distress must accompany the symptoms in Criterion A. Distress may be experienced as a result of the lack of sexual interest/arousal or as a result of significant interference in a woman’s life and well-being. If a lifelong lack of sexual desire is better explained by one’s self-identification as “asexual,” then a diagnosis of female sexual interest/arousal disor- der would not be made. Associated Features Supporting Diagnosis Female sexual interest/arousal disorder is fr equently associated with problems in experi- encing orgasm, pain experienced during sexual activity, infrequent sexual activity, and couple-level discrepancies in desire. Relationship difficulties and mood disorders are also frequently associated features of female sexual interest/arousal disorder. Unrealistic ex- pectations and norms regarding the “appropriate” level of sexual intere st or arousal, along
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pectations and norms regarding the “appropriate” level of sexual intere st or arousal, along with poor sexual techniques and lack of info rmation about sexuality, may also be evident in women diagnosed with female sexual interest/arousal disorder. The latter, as well as normative beliefs about gender roles, are importan t factors to consider.
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in women diagnosed with female sexual interest/arousal disorder. The latter, as well as normative beliefs about gender roles, are importan t factors to consider. In addition to the subtypes “lifelong/acquired” and “generalized/situational,” the follow- ing five factors must be considered during asse ssment and diagnosis of female sexual interest/ arousal disorder given that they may be relevant to etiology and/or treatment: 1) partner fac- tors (e.g., partner’s sexual prob lems, partner’s health status); 2) relationship factors (e.g., poor communication, discrepancies in de sire for sexual activity); 3) individual vulnerability factors (e.g., poor body image, history of sexual or emotional abuse), ps ychiatric comorbidity (e.g., de- pression, anxiety), or stressors (e.g., job loss, bereavement); 4) cultural/religious factors (e.g., inhibitions related to prohibitions against sexu al activity; attitudes toward sexuality); and
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Female Sexual Interest/Arousal Disorder 435 5) medical factors relevant to pr ognosis, course, or treatment. Note that each of these factors may contribute differently to the presenting sy mptoms of different women with this disorder. Prevalence The prevalence of female sexual interest/arousal disorder, as defined in this manual, is unknown. The prevalence of low sexual desire and of problems with sexual arousal (with and without associated distress), as defined by DSM-IV or ICD-10, may vary markedly in relation to age, cultural setting, duration of symptoms, and presence of distress. Regard- ing duration of symptoms, there are striking differences in prevalence estimates between short-term and persistent problems related to lack of sexual intere st. When distress about sexual functioning is requir ed, prevalence estimates are markedly lower. Some older women report less distress ab out low sexual desire than y ounger women, although sexual desire may decrease with age. Development and Course By definition, lifelong female sexual interest /arousal disorder suggests that the lack of sexual interest or arousal has been present fo r the woman’s entire sexual life. For Criteria A3, A4, and A6, which assess functioning duri ng sexual activity, a subtype of lifelong would mean presence of symptoms since the in dividual’s first sexual experiences. The ac- quired subtype would be assigned if the difficu lties with sexual interest or arousal de- veloped after a period of nonproblematic se xual functioning. Adaptive and normative changes in sexual functioning may result from partner-related, interpersonal, or personal events and may be transient in nature. Howe ver, persistence of symptoms for approxi- mately 6 months or more would constitute a sexual dysfunction. There are normative changes in sexual interest and arousal across the life span. Fur-
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There are normative changes in sexual interest and arousal across the life span. Fur- thermore, women in relationships of longer du ration are more likely to report engaging in sex despite no obvious feelings of sexual desi re at the outset of a sexual encounter com- pared with women in shorter-duration relationships. Vaginal dryness in older women is related to age and menopausal status. Risk and Prognostic Factors Temperamental. Temperamental factors include negati ve cognitions and attitudes about sexuality and past history of mental disorders. Differences in propensity for sexual excitation and sexual inhibition may also predict the likelihood of developi ng sexual problems. Environmental. Environmental factors include relationship difficulties, partner sexual functioning, and developmental history, such as early relationships with caregivers and childhood stressors. Genetic and physiological. Some medical conditions (e.g., diabetes mellitus, thyroid dysfunction) can be risk factors for female se xual interest/arousal disorder. There appears to be a strong influence of genetic factors on vulnerability to sexual problems in women. Psychophysiological research using vaginal photoplethysmography has not found differ- ences between women with and without perceived lack of genital arousal. Culture-Related Diagnostic Issues There is marked variability in prevalence rates of low desire across cultures. Lower rates of sexual desire may be more common among East Asian women compared with Euro- Canadian women. Although the lower levels of sexual desire and arousal found in men and women from East Asian countries compared with Euro-American groups may reflect less interest in sex in those cultures, the possibility remains that such group differences are an artifact of the measures used to quanti fy desire. A judgment about whether low sexual
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436 Sexual Dysfunctions desire reported by a woman from a certain ethnocultural group meets criteria for female sexual interest/arousal disorder must take into account the fact that different cultures may pathologize some behaviors and not others. Gender-Related Diagnostic Issues By definition, the diagnosis of female sexual interest/arousal disorder is only given to women. Distressing difficulties with sexual desire in men would be considered under male hypoactive sexual desire disorder. Functional Consequences of Female Sexual Intere st/Arousal Disorder Difficulties in sexual interest/a rousal are often associated with decreased relationship sat- isfaction. Differential Diagnosis Nonsexual mental disorders. Nonsexual mental disorders, such as major depressive disorder, in which there is “markedly diminished interest or pleasure in all, or almost all, activities most of the day, nearly every day,” may explain the lack of sexual interest/ arousal. If the lack of interest or arousal is completely attributable to another mental dis- order, then a diagnosis of female sexual in terest/arousal disorder would not be made. Substance/medication use. Substance or medication use may explain the lack of inter- est/arousal. Another medical condition. If the sexual symptoms are co nsidered to be almost exclu- sively associated with the effe cts of another medical conditio n (e.g., diabetes mellitus, en- dothelial disease, thyroid dysfunction, centra l nervous system diseas e), then a diagnosis of female sexual inte rest/arousal disorder would not be made. Interpersonal factors. If interpersonal or significant co ntextual factors, such as severe relationship distress, intimate partner violence , or other significant stressors, explain the
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relationship distress, intimate partner violence , or other significant stressors, explain the sexual interest/arousal symptoms, then a diag nosis of female sexual interest/arousal dis- order would not be made. Other sexual dysfunctions. The presence of another sexu al dysfunction does not rule out a diagnosis of female sexual interest/arousal disorder. It is common for women to ex- perience more than one sexual dysfunction. For example, the presence of chronic genital pain may lead to a lack of desire for the (p ainful) sexual activity. Lack of interest and arousal during sexual activity may impair or gasmic ability. For some women, all aspects of the sexual response may be unsatisfying and distressing. Inadequate or absent sexual stimuli. When differential diagnoses are being considered, it is important to assess the adequacy of se xual stimuli within the woman’s sexual experi- ence. In cases where inadequate or absent sexual stimuli are co ntributing to the clinical pic- ture, there may be evidence for clinical care , but a sexual dysfunction diagnosis would not be made. Similarly, transient and adaptive alte rations in sexual functioning that are second- ary to a significant life or personal event must be considered in the differential diagnosis. Comorbidity Comorbidity between sexual interest/arousal problems and other sexual difficulties is extremely common. Sexual distress and dissatis faction with sex life are also highly cor- related in women with low sexual desire. Dist ressing low desire is associated with depres- sion, thyroid problems, anxiety, urinary incontinence, and other medical factors. Arthritis and inflammatory or i rritable bowel disease are also associated with sexual arousal prob-
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Genito-Pelvic Pain/Penetration Disorder 437 lems. Low desire appears to be comorbid wi th depression, sexual and physical abuse in adulthood, global mental functioning, and use of alcohol. Genito-Pelvic Pain/Penetration Disorder Diagnostic Criteria 302.76 (F52.6) A. Persistent or recurrent difficulties with one (or more) of the following: 1. Vaginal penetration during intercourse. 2. Marked vulvovaginal or pelvic pain during vaginal intercourse or penetration attempts. 3. Marked fear or anxiety about vulvovaginal or pelvic pain in anticipation of, during, or as a result of vaginal penetration. 4. Marked tensing or tightening of the pelvic floor muscles during attempted vaginal penetration. B. The symptoms in Criterion A have persist ed for a minimum duration of approximately 6 months. C. The symptoms in Criterion A cause clinically significant distress in the individual. D. The sexual dysfunction is not better explained by a nonsexual mental disorder or as a consequence of a severe relationship distress (e.g., partner violence) or other signifi- cant stressors and is not attributable to the effects of a substance/medication or an- other medical condition. Specify whether: Lifelong: The disturbance has been present since the individual became sexually active. Acquired: The disturbance began after a period of relatively normal sexual function. Specify current severity: Mild: Evidence of mild distress over the symptoms in Criterion A. Moderate: Evidence of moderate distress ov er the symptoms in Criterion A. Severe: Evidence of severe or extreme distress over the symptoms in Criterion A. Diagnostic Features Genito-pelvic pain/penetration disorder refe rs to four commonly comorbid symptom di-
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mensions: 1) difficulty having in tercourse, 2) genito-pelvic pain , 3) fear of pain or vaginal penetration, and 4) tension of the pelvic fl oor muscles (Criterion A). Because major diffi- culty in any one of these symptom dimensions is often sufficient to cause clinically sig- nificant distress, a diagnosis can be made on the basis of marked difficulty in only one symptom dimension. However, a ll four symptom dimensions should be assessed even if a diagnosis can be made on the basis of only one symptom dimension. Marked difficulty having vaginal intercourse/penetration (Criterion A1) can vary from a total in- ability to experience vaginal penetration in an y situation (e.g., interc ourse, gynecological ex- aminations, tampon insertion) to the ability to easily experience penetration in one situation and but not in another. Although the most comm on clinical situation is when a woman is un- able to experience intercourse or penetration with a partner, difficult ies in undergoing re- quired gynecological examinat ions may also be present. Marked vulvovaginal or pelvic pain during vaginal intercourse or penetration attempts (Criterion A2) refers to pain occurring in differ- ent locations in the genito-pelvic area. Location of pain as well as intensity should be assessed. Typically, pain can be characterized as superf icial (vulvovaginal or occurring during penetra- tion) or deep (pelvic; i.e., not felt until deeper penetrat ion). The intensity of the pain is often not linearly related to distress or interference with sexual intercourse or other sexual activities. Some genito-pelvic pain only occurs when provoked (i.e., by intercourse or mechanical stim-
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438 Sexual Dysfunctions ulation); other genito-pelvic pa in may be spontaneous as well as provoked. Genito-pelvic pain can also be usefully characterized qualitatively (e.g., “burning,” “cutting,” “shooting,” “throb- bing”). The pain may persist for a period afte r intercourse is completed and may also occur during urination. Typically, the pain experience d during sexual intercourse can be reproduced during a gynecological examination. Marked fear or anxiety about vulvov aginal or pelvic pain either in anticipation of, or during, or as a result of vaginal penetration (Criterion A3) is commonly reported by women who have regularly experienced pain during sexual inte rcourse. This “normal” reaction may lead to avoidance of sexual/intimate situations. In ot her cases, this marked fear does not appear to be closely related to the experience of pa in but nonetheless leads to avoidance of inter- course and vaginal penetration situations. Some have describe d this as similar to a phobic reaction except that the phobic object may be vaginal penetration or the fear of pain. Marked tensing or tightening of the pelvic floor muscles during attempted vaginal penetration (Criterion A4) can vary from reflexive-like spasm of the pelvic floor in response to at- tempted vaginal entry to “normal/voluntary” muscle guarding in response to the antici- pated or the repeated experience of pain or to fear or anxiety. In the case of “normal/ guarding” reactions, penetratio n may be possible under circumstances of relaxation. The characterization and assessment of pelvic floo r dysfunction is often best undertaken by a specialist gynecologist or by a pelvic floor physical therapist. Associated Features Supporting Diagnosis
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Associated Features Supporting Diagnosis Genito-pelvic pain/penetration di sorder is frequently associated with other sexual dysfunc- tions, particularly reduced sexual desire and interest (female sexual interest/arousal disor- der). Sometimes desire and interest are preserved in sexual situations that are not painful or do not require penetration. Even when indivi duals with geni to-pelvic pain/p enetration dis- order report sexual interest/mot ivation, there is of ten behavioral avoidance of sexual situ- ations and opportunities. Avoidance of gynecological examinations despite medical recommendations is also frequen t. The pattern of avoidance is si milar to that seen in phobic disorders. It is common for women who have no t succeeded in having sexual intercourse to come for treatment only when they wish to conceive. Many women wi th genito-pelvic pain/ penetration disorder will experience associated relationship/marital problems; they also of- ten report that the symptoms significantly diminish their fee lings of femininity. In addition to the subtype “lifelong/acquired,” five factors should be considered dur- ing assessment and diagnosis of genito-pelvi c pain/penetration di sorder because they may be relevant to etiology and/or treatmen t: 1) partner factors (e.g., partner’s sexual problems, partner’s health status); 2) relationship factors (e.g., poor communication, dis- crepancies in desire for sexual activity); 3) individual vulnerability factors (e.g., poor body image, history of sexual or emotional abuse), psychiatric comorbidity (e.g., depression, anxiety), or stressors (e.g., job loss, bereavemen t); 4) cultural/religious factors (e.g., inhi-
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bitions related to prohibitions against sexual activity; attitudes toward sexuality); and 5) medical factors relevant to prognosis, cour se, or treatment. Each of these factors may contribute differently to the presenting symp toms of different women with this disorder. There are no valid physiological measures of any of the component symptom dimen- sions of genito-pelvic pain/penetration diso rder. Validated psychome tric inventories may be used to formally assess the pain and anxiety components related to genito-pelvic pain/ penetration disorder. Prevalence
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be used to formally assess the pain and anxiety components related to genito-pelvic pain/ penetration disorder. Prevalence The prevalence of genito-pelvic pain/penetration disorder is unknown. Howe ver, approx- imately 15% of women in North America report recurrent pain during intercourse. Diffi- culties having intercourse appear to be a freque nt referral to sexual dysfunction clinics and to specialist clinicians.
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Genito-Pelvic Pain/Penetration Disorder 439 Development and Course The development and course of genito-pelvic pa in/penetration disorder is unclear. Because women generally do not seek treatment until they experience problems in sexual functioning, it can, in general, be difficult to characterize genito-pelvic pain/penetration disorder as life- long (primary) or acquired (secondary). Alth ough women typically come to clinical atten- tion after the initiation of sexual activity, there are often earlier clinical signs. For example, difficulty with or the avoidanc e of use of tampons is an important predictor of later problems. Difficulties with vaginal penetration (inability or fear or pain) may not be obvious until sex- ual intercourse is attempted. Ev en once intercourse is attemp ted, the frequency of attempts may not be significant or regular. In cases where it is difficult to establish whether symptom- atology is lifelong or acquired, it is useful to determine the presence of any consistent period of successful pain-, fear-, and tension-free intercourse. If the ex perience of such a period can be established, then genito-pelvic pain/penet ration disorder can be characterized as ac- quired. Once symptomatology is well establishe d for a period of approximately 6 months, the probability of spontaneous and significan t symptomatic remission appears to diminish. Complaints related to genito-pelvic pain pe ak during early adulthood and in the peri- and postmenopausal period. Women with compla ints about difficulty having intercourse appear to be primarily premenopausal. There may also be an increa se in genito-pelvic pain–related symptoms in the postpartum period. Risk and Prognostic Factors
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Risk and Prognostic Factors Environmental. Sexual and/or physical abuse have often been cited as predictors of the DSM-IV-defined sexual pain di sorders dyspareunia and vaginismus. This is a matter of con- troversy in the current literature. Genetic and physiological. Women experiencing superficial pain during sexual inter- course often report the onset of the pain after a history of vaginal infections. Even after the in- fections have resolved and there are no known residual physical findings, the pain persists. Pain during tampon insertion or the inability to insert tampons before any sexual contact has been attempted is an importan t risk factor for genito-pelvic pain/penetration disorder. Culture-Related Diagnostic Issues In the past, inadequate sexual education and religious orthodoxy have often been consid- ered to be culturally related predisposing fa ctors to the DSM-IV diagnosis of vaginismus. This perception appears to be confirmed by recent reports from Turkey, a primarily Mus- lim country, indicating a strikingly high prev alence for the disorder. However, most avail- able research, although limited in scope, does not support this notion (Lahaie et al. 2010). Gender-Related Diagnostic Issues By definition, the diagnosis of genito-pelvic pain/penetration disorder is only given to women. There is relatively new research concerning urological chronic pelvic pain syn- drome in men, suggesting that men may expe rience some similar problems. The research and clinical experience are not sufficiently de veloped yet to justify the application of this diagnosis to men. Other specified sexual dy sfunction or unspecified sexual dysfunction may be diagnosed in men appearing to fit this pattern. Functional Consequences of Genito-Pelvic Pain/Penetration Disorder
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Functional Consequences of Genito-Pelvic Pain/Penetration Disorder Functional difficulties in ge nito-pelvic pain/pen etration disorder are often associated with interference in relationship satisfacti on and sometimes with the ability to conceive via penile/vaginal intercourse.
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440 Sexual Dysfunctions Differential Diagnosis Another medical condition. In many instances, women with genito-pelvic pain/pene- tration disorder will also be diagnosed with another medical condition (e.g., lichen scle- rosus, endometriosis, pelvic inflammatory disease, vulvovaginal atrophy). In some cases, treating the medical condition may alleviate the genito-pelvic pain/penetration disorder. Much of the time, this is not the case. There are no reliable tools or diagnostic methods to allow clinicians to know whether the medica l condition or genito-pelvic pain/penetration disorder is primary. Often, the associated medi cal conditions are difficult to diagnose and treat. For example, the increased incidence of postmenopausal pain during intercourse may sometimes be attributable to vaginal dr yness or vulvova ginal atrophy as sociated with declining estrogen levels. The relationship, however, between vulv ovaginal atrophy/dry- ness, estrogen, and pain is not well understood. Somatic symptom and related disorders. Some women with genito-pelvic pain/pene- tration disorder may also be diagnosable with somatic symptom disorder. Since both genito-pelvic pain/penetration disorder and the somatic symptom and related disorders are new diagnoses, it is not yet clear whet her they can be reliably differentiated. Some women diagnosed with genito-pelvic pain/penetration disorder will also be diagnosed with a specific phobia. Inadequate sexual stimuli. It is important that the clinician, in considering differential diag- noses, assess the adequacy of se xual stimuli within the woman’s sexual experience. Sexual sit- uations in which there is inadequate foreplay or arousal may lead to difficulties in penetration,
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uations in which there is inadequate foreplay or arousal may lead to difficulties in penetration, pain, or avoidance. Erectile dysfunction or premature ejacul ation in the male partner may result in difficulties with penetration. These conditions should be carefully assessed. In some situations, a diagnosis of genito-pelvic pain/p enetration disorder may not be appropriate. Comorbidity Comorbidity between genito-pel vic pain/penetration disorder and other sexual difficul- ties appears to be common. Comorbidity with relationship distress is also common. This is not surprising, since in Western cultures the inability to have (pain-free) intercourse with a desired partner and the avoidance of sexual opportunities may be either a contributing factor to or the result of other sexual or re lationship problems. Because pelvic floor symp- toms are implicated in the diagnosis of geni to-pelvic pain/penetrati on disorder, there is likely to be a higher prevalence of other diso rders related to the pelvic floor or reproduc- tive organs (e.g., interstitial cystitis, constipation, vaginal infection, endometriosis, irrita- ble bowel syndrome). Male Hypoactive Sexual Desire Disorder Diagnostic Criteria 302.71 (F52.0) A. Persistently or recurrently deficient (or absent) sexual/erotic thoughts or fantasies and desire for sexual activity. The judgment of deficiency is made by the clinician, taking into account factors that affect sexual functioning, such as age and general and socio- cultural contexts of the individual’s life. B. The symptoms in Criterion A have persist ed for a minimum duration of approximately 6 months. C. The symptoms in Criterion A cause clinically significant distress in the individual. D. The sexual dysfunction is not better explained by a nonsexual mental disorder or as a
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D. The sexual dysfunction is not better explained by a nonsexual mental disorder or as a consequence of severe relationship distress or other significant stressors and is not at- tributable to thes effects of a substance/medication or another medical condition.
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Male Hypoactive Sexual Desire Disorder 441 Specify whether: Lifelong: The disturbance has been present since the individual became sexually active. Acquired: The disturbance began after a period of relatively normal sexual function. Specify whether: Generalized: Not limited to certain types of stimulation, situations, or partners. Situational: Only occurs with certain types of stimulation, situations, or partners. Specify current severity: Mild: Evidence of mild distress over the symptoms in Criterion A. Moderate: Evidence of moderate distress ov er the symptoms in Criterion A. Severe: Evidence of severe or extreme distress over the symptoms in Criterion A. Diagnostic Features When an assessment for male hypoactive sexu al desire disorder is being made, inter- personal context must be take n into account. A “desire discrepancy,” in which a man has lower desire for sexual activity than his partne r, is not sufficient to diagnose male hypo- active sexual desire d isorder. Both low/absent desire fo r sex and deficient/absent sexual thoughts or fantasies are required for a diag nosis of the disorder. There may be variation across men in how sexual desire is expressed. The lack of desire for sex and deficient/absent erotic though ts or fantasies must be per- sistent or recurrent and must occur for a mi nimum duration of approximately 6 months. The inclusion of this duration criterion is me ant to safeguard against making a diagnosis in cases in which a man’s low sexual desire may represent an adaptive response to adverse life conditions (e.g., concern about a partner’s pregnancy when the man is considering ter- minating the relationship). The introduction of “approximately” in Criterion B allows for clinician judgment in cases in which sympto m duration does not meet the recommended 6-month threshold.
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6-month threshold. Associated Features Supporting Diagnosis Male hypoactive sexual desire disorder is some times associated with erectile and/or ejac- ulatory concerns. For example, persistent difficulties obtaining an erection may lead a man to lose interest in sexual activity. Men with hypoactive sexual desire disorder often report that they no longer initiate sexual activity and that they are minima lly receptive to a part- ner’s attempt to initiate. Sexual activities (e.g ., masturbation or part nered sexual activity) may sometimes occur even in the presence of low sexual desire. Relationship-specific pref- erences regarding patterns of sexual initiation must be taken into account when making a diagnosis of male hypoactive sexual desire di sorder. Although men are more likely to ini- tiate sexual activity, and thus low desire may be characterized by a pattern of non-initiation, many men may prefer to have their partner init iate sexual activity. In such situations, the man’s lack of receptivity to a partner’s initiati on should be considered when evaluating low desire. In addition to the subtypes “lifelong/acquired” and “generalized/situational,” the fol- lowing five factors must be considered duri ng assessment and diagnosis of male hypo- active sexual desire disorder given that they may be relevant to et iology and/or treatment: 1) partner factors (e.g., partne r’s sexual problems, partner’s health status); 2) relationship factors (e.g., poor communication , discrepancies in desire for sexual activity); 3) individ- ual vulnerability factors (e.g., po or body image, history of se xual or emotional abuse), psy- chiatric comorbidity (e.g., depression, anxiety), or stressors (e.g., job loss, bereavement);
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