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PARASOMNIAS BY AHMAD YOUNES PROFESSOR OF THORACIC MEDICINE Mansoura Faculty of Medicine Two major types of primary sleep disorders are dyssomnias and parasomnias • Primary sleep disorder is a malady of sleep that does not appear to be secondary to a physical or mental illness and is not substance-induced. • Dyssomnias are characterized by insomnias and excessive sleepiness (abnormal sleep quality, including initiation, maintenance, duration, timing, and amount of sleep) • parasomnias are distinguished by deviant behavioral and/or physiologic events • parasomnias manifest by activation of systems, such as the autonomic nervous system, or programs, such as cognitive, behavioral, or motor program stimulation Parasomnias Parasomnias are disorders characterized by abnormal behavioral or physiological events occurring in association with sleep, specific sleep stages, or sleep-wake transitions. parasomnias do not involve abnormalities of the mechanisms generating sleep-wake states, nor of the timing of sleep and wakefulness. Individuals with parasomnias usually present with complaints of unusual behavior during sleep rather than complaints of insomnia or excessive daytime sleepiness Parasomnias • Most of the symptoms are manifestations of central nervous system activation, specifically motor and autonomic discharge. • All parasomnias are more common in males than in females, and persons with one type of parasomnia are more likely to manifest symptoms of another. • Children with sleep terrors usually sleepwalk when they reach an older age. Positive family histories of parasomnias are common. Pathophysiology • The concept that wakefullness ,NREM and REM sleep can occur simultaneously or oscillate rapidly is the key to the understanding of primary sleep parasomnias • The mixture of wakefullness and NREM sleep would explain confusional arousals • THE tonic and phasic REM sleep can become dissociated ,intruding or persisting into wakefullness, explaining cataplexy ,wakefull dreaming and persistence of motor activity during REM sleep ( REM sleep behaviour disorder) Pathogenesis 1- State dissociation (two state of being ovelap or occur simultaneously ) 2- Locomotor centers present in multiple sites in CNS which represent motor activity that is dissociated from waking consciosness . 3- Sleep inertia (sleep drunkness) is a period of impaired performance and reduced vigilance followig awakening from sleep episode or naps (gradual disengagment from sleep to wakfullness ). 4- Sleep state instability( cyclic alternating pattern of NREM sleep which correlte with arousal oscillations ) Types of parasomnias Primary parasomnias (disorders of the sleep state per se) 1- NREM sleep Normal (exploding head syndrome ,exlosive tinnitus) Abnormal(confusional arousals ,sleep walking ,sleep terrors) 2- REM sleep Normal (sleep paralysis,hypnagogic and hypnopompic hallucinations) Abnormal REM sleep behavior disorders ,REM related painfull erections ,nightmares) Types of parasomnias Miscellaneous Nocturnal catathrenia (groaning),bruxism,enuresis,rhthmic movement disorder,sleep talking(somniloquy),myoclonus Secondary parasomnias (diorders of other systems that emerge during sleep ) 1-CNS (hypnic headach ,tinnitus ,seizers) 2- Cardiopulmonary (arrhthmias,nocturnal angina pectoris,nocturnal asthma ,respiratory dyskinesias ,coughing ,hiccup ,choking ) 3-Gastrointestinal(GERD,esophageal spasm ,abnormal swallowing ) 4- Medications and substance abuse (Beta adrenergic blocker,SSRI,TCA,cocaine) 5- Functional (nocturnal panic attacks, PTSD ,maligering ) Epidemiology • The disorders of arousal are most common of NREM sleep parasomnias ,commonly in children ,positive family history, febril illness ,sleep deprivation and emotional stress. • Numerous sleep disorders that result in arousals (OSA ,PLMS,nocturnal seizures )can provoke NREM sleep parasomnias . • Sleep disordered breathing has been found to be more prevalent in children and adults with disorders of arousal ( sleep disorder within sleep disorder).This explain common improvement of disorders of arousal following treatment of OSA NREM sleep parasonias Disorders of arousals • Abnormal arousal (motor activity is restored without an accompanying full consciousness ) • Occur during NREM sleep stage N3 • Predisposing factors (febrile illness ,sleep derivation irregular sleep wake schedules ,stress,alcohol ,distended bladder ,OSA ,PLMS, minor tranquilizers ,neuroleptics ,hypnotics ,stimulants) Types of disorders of arousals 1-confusional arousals 2- sleepwalking 3-sleep terrors. Confusional Arousals • Episodes of confusion following spontaneous or forced arousals from sleep, typically from NREM stages N3 • Disorientation, confusion, (moving around in bed, crying, or sleep talking), diminished vigilance and blunted response to questions or external stimuli. • Signs of fear or autonomic hyperactivity are minimal or absent. last from several minutes to hours, with most cases spontaneously resolving within 5 to 15 minutes and amnesia for the event. Sleep walking (somnambulism) • Sleepwalking, refers to ambulation that occurs during sleep. • Sleepwalking is associated with an altered state of consciousness, diminished arousability, impaired judgment and inappropriate behavior (eg, shouting or climbing out of a window). • The behavior can either be calm or agitated and violent • Each episode varies widely from several minutes to over an hour • sleepwalker’s eyes are usually open (described as a blank stare), but attempts to communicate with the sleepwalker are generally unsuccessful Sleep Terrors • Sleep terrors consist of abrupt awakenings with profound fear usually from NREM stages N3. • Sleep terrors suddenly bolt upright from their beds with a loud cry, or scream, and in rare instances, sleepwalking or running • Associated clinical features include misperception of the environment, confusion, amnesia for the episode, autonomic and behavioral manifestations of intense fear (tachycardia, elevated blood pressure, tachypnea, dilated pupils, and profuse sweating), vocalizations, or urinary incontinence • Persons with sleep terrors then spontaneously calm down and return rapidly to sleep. REM sleep parasomnias Rapid Eye Movement Sleep Behavior Disorder • Abnormal behaviors develop during REM sleep and are accompanied by loss of REM-related muscle atonia or hypotonia. • These dream-enacting behaviors can result in sleep disruption or injury to the sleeper or bed partner. • There is often no history of violent or aggressive behavior during the day while awake. • Range from simple motions to highly elaborate activities (eg, screaming, punching, kicking, jumping, or running). • Affected individuals appear to be “acting out their dreams. dream content often involving defense of the sleeper against attack. • The eyes are usually closed, in contrast to the sleepwalker, whose eyes are open during the episode. Rapid Eye Movement Sleep Behavior Disorder • Episodes end with a rapid awakening and full alertness. • Associated features include good dream recall on awakening. • Most forms of RBD are idiopathic (approximately 60% of cases). • RBD can also be associated with Parkinson disease, dementia with Lewy bodies,and multiple system atrophy. during withdrawal from alcohol or REM sleep suppressants. Clinical subtypes of rapid eye movement sleep behavior disorder • Subclinical RBD Polysomnographic features consistent with RBD without clinical manifestations of the disorder • Parasomnia overlap syndrome Elements of disorders of arousal (confusional arousals, sleep terrors, and sleepwalking) and RBD are present. • Status dissociatus Admixture of the different states of wakefulness, NREM sleep, and REM sleep.Abnormal sleep and dream-related behaviors closely resembling RBD in the absence of identifi able sleep stages during polysomnography. Sleep-Related Painful Erections • Painful penile erections occurring during REM sleep can give rise to repetitive awakenings, and, in some cases, insomnia and/or excessive daytime sleepiness. • Begin after the fourth decade of life and progressively worsens with advancing age. It is not associated with any physical abnormality, or any penile disorder or pain during sexual erections while awake. • Sexual function while awake is generally normal Nightmares • Nightmares are frightening dreams, often involving threats to life or security, that occur during REM sleep and that commonly abruptly awaken the sleeper from sleep. • Once awakened, the person is fully alert and profoundly fearful and anxious, can recall vividly the preceding dream, and has difficulty returning to sleep. Some minor autonomic activation, such as tachycardia and tachypnea, is evident. • Nightmares can be precipitated by illness, traumatic experiences, acute alcohol ingestion,and medications. Differences between nightmares and sleep terrors Characteristics Nightmares Time of night Latter half of night First half of night Sleep stage REM sleep NREM sleep consciousness Alert Confused Memory of episode Full recall Subsequent return to sleep Delayed Sleep terrors Partial or complete amnesia Rapid Miscellaneous parasomnias Sleep-Related Groaning (Catathrenia) • Catathrenia consists of expiratory groaning during sleep. • Episodes occur predominantly or exclusively during REM sleep. • A rare condition that is more common among males. The individual is asymptomatic with no evident distress and is unaware of the events. • Polysomnography demonstrate episodes of bradypnea associated with loud expiratory groaning sounds that occur in clusters recurring several times throughout the night mainly during REM sleep. unusual movements or cardiac arrhythmias are typically absent. Sleep architecture and oxygen saturation remain normal. Sleep Bruxism – Sleep bruxism is characterized by repetitive grinding of the teeth, caused by contractions of the masticatory muscles (eg, masseter and temporalis) during sleep. – Bruxism during sleep can give rise to arousals, unpleasant noises that might disrupt the bed partner’s sleep or causing abnormal dental damage – chronic bruxism was present in 8% of adults – can either be isolated and sustained, or repetitive (rhythmic masticatory muscle activity [RMMA]) – The risk of developing bruxism is increased among smokers, restless legs syndrome , stress, dental disease such as malocclusion or mandibular malformation, caffeine, alcohol, primary sleep disorders (eg, OSA or REM sleep behavior disorder), personality subtypes (eg, vigilant and highly motivated), and medication use (eg, levodopa or SSRIs). Enuresis – Recurrent involuntary bed-wetting occurring during sleep after 5 years of age. It can arise throughout the night and during any stage of sleep, although most tend to occur early during sleep in the first third of the evening. – Children with enuresis may report guilt about their problem – Pathophysiology include failure to arouse in response to a sensation of bladder fullness, impaired ability to transiently delay bladder contraction when a need to void develops, greater urine production during sleep in relation to agerelated nocturnal bladder capacity, or a maturational delay in bladder development resulting in a smaller bladder capacity. Enuresis • Primary if recurrent sleep-related micturition occurring at least twice a week persists in children older than 5 years of age who have not been consistently dry during sleep • Secondary (5-10%)if bed-wetting recurs at least twice a week for at least 3 months after the child or adult has maintained dryness for at least six consecutive months • Increased production of urine due to the use of diuretics, ingestion of caffeine, or impairment in the ability to concentrate urine (eg, diabetes mellitus or diabetes insipidus); urinary tract infection;pelvic abnormalities (eg, anomalies of the bladder); psychosocial stressors (eg, birth of a sibling); depression; OSA; congestive heart failure;dementia; seizures; and chronic constipation. Enuresis • Nocturia involves frequent awakenings from sleep to urinate in the bathroom. ( diuretic therapy, diabetes mellitus,diabetes insipidus ) • Evaluation includes an extensive medical, neurologic, psychiatric, and sleep history. • polysomnography or EEG to rule out the presence of OSA or seizure disorder, respectively. • Spontaneous cure rate in children with primary sleep enuresis is estimated at 15% annually. • Treatment consists of pharmacotherapy (Desmopressin ,Tricyclic antidepressants )or behavioral therapy( sleep hygiene). A secondary cause of enuresis, if identified, should be addressed and corrected. • Sleep hygiene ( restricting fluid intake after dinner and voiding prior to going to bed. Rewards for dry nights are preferable to punishing the child for bedwetting). Rhythmic Movement Disorder • Head banging (jactatio capitis nocturna) repeatedly lifting and banging the head back onto the bed, head rolling (lateral movements of the head), body rolling (side-to-side motions of the body), body rocking (entire body is rocked while positioned on hands and knees), leg rolling or banging. • complications of head banging, in addition to sleeponset insomnia, include eye and cranial injuries, such as fractures or soft tissue trauma. • Often affects normal infants younger than 18 months of age. Typically self-limited; spontaneous resolution before 4 years of age is characteristic. Sleep Paralysis Generalized transient inability to move the head,body, and extremities,with sparing of the ocular and respiratory muscles. unable to speak during these episodes. It can occur either at sleep onset (hypnagogic)or upon awakening (hypnopompic) • Profound anxiety, and hallucinations (visual, auditory, or tactile) may accompany these attacks, but consciousness and recall are typically unaffected. • Paralysis spontaneously resolves after several seconds to several minutes. Sleep Paralysis • The frequency of episodes ranges from once in a lifetime to almost nightly • Predisposing and precipitating factors include sleep deprivation, irregular sleep patterns (eg, shift work), a supine sleep position, use of anxiolytic agents, and stress. • Most cases are identified in an isolated form; others occur in a familial form (autosomal dominant in some cases) or in persons with narcolepsy. • Isolated sleep paralysis occurs at least once in a lifetime in 40% to 50% of normal individuals, Sleep-Related Hallucinations • Hallucinations can occur during sleep-wake transitions, either at sleep onset (hypnagogic hallucinations)or during awakening (hypnopompic hallucinations). • These hallucinatory experiences can take a variety of forms, visual, auditory, or tactile phenomena, and can last from several seconds to minutes. Sleep paralysis may accompany the hallucinations. • can be encountered in patients with narcolepsy or in an isolated form in otherwise healthy individuals in whom they occur more commonly during adolescence or early adulthood. Sleep-Related Hallucinations • Prevalence decreases with aging. Women are affected more frequently than men. • Reported during administration of β-adrenergic blocking agents and in patients with mood disorders, Parkinson disease, substance or alcohol use and sleep deprivation. • Occurring predominantly during sleep-onset REM periods, but episodes can also arise during NREM sleep Diaphragmatic flutter • Diaphragmatic flutter is a rare disorder characterized by rapid involuntary contractions of the diaphragm superimposed on ordinary respiratory excursions • High frequency bilateral oscillatory movements were observed in chest and upper abdomen during both inspiration and expiration. • Involuntary movements were not seen in hands and legs. • Etiology of this disorder includes abnormal excitation of the central nervous system including cerebrum and the brainstem, direct irritation of the phrenic nerve and irritation of the diaphragm itself. Since the psychosomatic status played a significant role in the development of diaphragmatic flutter, this may be a disorder in the behavioral control of breathing. Palatal myoclonus • Palatal myoclonus is a rapid spasm of the palatal muscles, which results in clicking or popping in the ear. • Chronic clonus is often due to lesions of the central tegmental tract . • Uniquely, the clicking noise does not subside when the patient sleeps. Rapid Eye Movement Sleep-Related Sinus Arrest • Cardiac rhythm disorder is characterized by sinus arrest developing during REM sleep. Episodes often occur in clusters, with periods of asystole lasting up to 9 seconds in duration that usually recur frequently. • It affects apparently healthy young adults without any identifiable cardiac pathology. • Episodes of nocturnal sinus arrest are not accompanied by arousals, OSA or sleep disruption. • Most patients are asymptomatic, although palpitations or vague chest discomfort may occasionally be reported. • Daytime ECG and angiography are usually unremarkable. • Underlying pathophysiology involves abnormal vagal activity, particularly during REM sleep. Evaluation of PARASOMNIAS • Diagnosis for most parasomnias is based on its clinical presentation and seldom requires polysomnographic documentation. • Polysomnographic study is recommended for possible parasomnias associated with very frequent episodes, complaints of excessive sleepiness, unusual presentation or significant sleep disturbance, significant disruption of the bed partner, an underlying seizure activity is suspected, or in cases that have medicolegal implications. • A single normal PSG does not exclude the presence of parasomnias. Evaluation of PARASOMNIAS • Time-synchronized video recording (ie,simultaneous video and sleep monitoring), performed over several nights may be required. • Additional EEG electrodes are required for patients in whom a seizure disorder is being excluded. • Evaluation of patients presenting with violent behavior during sleep should be more comprehensive, and it may include an extensive neurologic and psychiatric assessment.