Pediatric Narcolepsy
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Pediatric Narcolepsy
Pediatric narcolepsy refers to conditions of narcolepsy during childhood and adolescence (during the ages 18 years and younger). In a pediatric setting, people with narcolepsy still exhibit the classical tetrad symptoms of narcolepsy, and thus is possible for both type 1 and type 2 narcolepsy to develop in adolescence. Pediatric population characteristics Pediatric narcolepsy cases are cases when patients are diagnosed or experience symptoms onset for narcolepsy before the age of 18. Of patients who obtain a formal diagnosis for narcolepsy, more than 50% report first experiencing symptoms of narcolepsy more than 10 years before their formal diagnosis, with an average age of symptom onset being at age 15 and symptom onset most likely to occur during a person's second decade. The overall prevalence of narcolepsy in a population is around 0.05% and is not impacted by population age. The most common symptom that occurs with symptom onset is excessive daytime sleepiness (EDS). There i ...
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Narcolepsy
Narcolepsy is a chronic neurological disorder that impairs the ability to regulate sleep–wake cycles, and specifically impacts REM (rapid eye movement) sleep. The symptoms of narcolepsy include excessive daytime sleepiness (EDS), sleep-related hallucinations, sleep paralysis, disturbed nocturnal sleep (DNS), and cataplexy. People with narcolepsy typically have poor quality of sleep. There are two recognized forms of narcolepsy, narcolepsy type 1 and type 2. Narcolepsy type 1 (NT1) can be clinically characterized by symptoms of EDS and cataplexy, and/or will have cerebrospinal fluid (CSF) orexin levels of less than 110 pg/ml. Cataplexy are transient episodes of aberrant tone, most typically loss of tone, that can be associated with strong emotion. In pediatric-onset narcolepsy, active motor phenomena are not uncommon. Cataplexy may be mistaken for syncope, tics, or seizures. Narcolepsy type 2 (NT2) does not have features of cataplexy, and CSF orexin levels are normal. ...
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Cataplexy
Cataplexy is a sudden and transient episode of muscle weakness accompanied by full conscious awareness, typically triggered by emotions such as laughing, crying, or terror. Cataplexy is the first symptom to appear in about 10% of cases of narcolepsy, caused by an autoimmune destruction of hypothalamic neurons that produce the neuropeptide hypocretin (also called orexin), which regulates arousal and has a role in stabilization of the transition between wake and sleep states. Cataplexy without narcolepsy is rare and the cause is unknown. The term cataplexy originates from the Greek κατά (''kata'', meaning "down"), and πλῆξις (''plēxis'', meaning "strike") and it was first used around 1880 in German physiology literature to describe the phenomenon of tonic immobility also known as " playing possum" (in reference to the opossum's behavior of feigning death when threatened). In the same year the French neuropsychiatrist Jean-Baptiste Gélineau coined the term 'narcol ...
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Precocious Puberty
In medicine, precocious puberty is puberty occurring at an unusually early age. In most cases, the process is normal in every aspect except the unusually early age and simply represents a variation of normal development. There is early development of secondary sex characters and gametogenesis also starts earlier. Precocious puberty is of two types: true precocious puberty and pseudoprecocious puberty. In a minority of children with precocious puberty, the early development is triggered by a disease such as a tumor or injury of the brain. Even when there is no underlying disease, unusually early puberty can have adverse effects on social behavior and psychological development (having more mature knowledge than one's peers, feeling inadequate, trying to attend and establish friendships with older people, depression). Affected children also face shorter adult height potential and possible lifelong health risks. Central precocious puberty can be treated by suppressing the pituita ...
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Periodic Limb Movement Disorder
Periodic limb movement disorder (PLMD) is a sleep disorder where the patient moves Limb (anatomy), limbs involuntarily and periodically during sleep, and has symptoms or problems related to the movement. PLMD should not be confused with restless legs syndrome (RLS), which is characterized by a voluntary response to an urge to move legs due to discomfort. PLMD on the other hand is involuntary, and the patient is often unaware of these movements altogether. Periodic limb movements (PLMs) occurring during daytime period can be found but are considered as a symptom of RLS; only PLMs during sleep can suggest a diagnosis of PLMD. Periodic limb movement disorder is characterized by recurrent episodes of frequent limb movements while sleeping. It mostly happens in the lower parts of the body like the toes, ankles, knees and hips. It can also, in some cases, appear in the upper extremities of the body. These movements can lead the patient to wake up, and if so, sleep interruption can be the ...
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Rapid Eye Movement Sleep Behavior Disorder
Rapid eye movement sleep behavior disorder or REM sleep behavior disorder (RBD) is a sleep disorder in which people act out their dreams. It involves abnormal behavior during the sleep phase with rapid eye movement (REM) sleep. The major feature of RBD is loss of muscle atonia (i.e., the loss of paralysis) during otherwise intact REM sleep (during which paralysis is not only normal but necessary). The loss of motor inhibition leads to sleep behaviors ranging from simple limb twitches to more complex integrated movements that can be violent or result in injury to either the individual or their bedmates. RBD is a very strong predictor of progression to a synucleinopathy (usually Parkinson's disease or dementia with Lewy bodies). Melatonin is useful in the treatment of RBD. RBD was first described in 1986. Classification RBD is a parasomnia. It is categorized as either idiopathic or symptomatic. ''Idiopathic RBD'' is the term used when RBD is not associated with anot ...
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Facies (medical)
In medical contexts, a facies is a distinctive facial expression or appearance associated with a specific medical condition. The term comes from Latin Latin ( or ) is a classical language belonging to the Italic languages, Italic branch of the Indo-European languages. Latin was originally spoken by the Latins (Italic tribe), Latins in Latium (now known as Lazio), the lower Tiber area aroun ... for "face". As a fifth declension noun, ''facies'' can be both singular and plural. Types Examples include: * Hippocratic facies – eyes are sunken, temples collapsed, nose is pinched with crusts on the lips, and the forehead is clammy * Moon face (also known as "Cushingoid facies") – Cushing's syndrome * Elfin facies – Williams syndrome, Donohue syndrome * Potter facies – oligohydramnios * Mask like facies – parkinsonism * Leonine facies – lepromatous leprosy or craniometaphyseal dysplasia * Mitral facies – mitral stenosis * Amiodarone facies (deep blu ...
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Narcoleptic Minute 006 0001
Narcolepsy is a chronic neurological disorder that impairs the ability to regulate sleep–wake cycles, and specifically impacts REM (rapid eye movement) sleep. The symptoms of narcolepsy include excessive daytime sleepiness (EDS), sleep-related hallucinations, sleep paralysis, disturbed nocturnal sleep (DNS), and cataplexy. People with narcolepsy typically have poor quality of sleep. There are two recognized forms of narcolepsy, narcolepsy type 1 and type 2. Narcolepsy type 1 (NT1) can be clinically characterized by symptoms of EDS and cataplexy, and/or will have cerebrospinal fluid (CSF) orexin levels of less than 110 pg/ml. Cataplexy are transient episodes of aberrant tone, most typically loss of tone, that can be associated with strong emotion. In pediatric-onset narcolepsy, active motor phenomena are not uncommon. Cataplexy may be mistaken for syncope, tics, or seizures. Narcolepsy type 2 (NT2) does not have features of cataplexy, and CSF orexin levels are normal. Sl ...
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Sleep Apnea
Sleep apnea (sleep apnoea or sleep apnœa in British English) is a sleep-related breathing disorder in which repetitive Apnea, pauses in breathing, periods of shallow breathing, or collapse of the upper airway during sleep results in poor ventilation and sleep disruption. Each pause in breathing can last for a few seconds to a few minutes and often occurs many times a night. A choking or snorting sound may occur as breathing resumes. Common symptoms include daytime sleepiness, snoring, and non restorative sleep despite adequate sleep time. Because the disorder disrupts normal sleep, those affected may experience sleepiness or feel tired during the day. It is often a chronic condition. Sleep apnea may be categorized as obstructive sleep apnea (OSA), in which breathing is interrupted by a blockage of air flow, central sleep apnea (CSA), in which regular unconscious breath simply stops, or a combination of the two. OSA is the most common form. OSA has four key contributors; these ...
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Sleep Paralysis
Sleep paralysis is a state, during waking up or falling asleep, in which a person is conscious but in a complete state of full-body paralysis. During an episode, the person may hallucinate (hear, feel, or see things that are not there), which often results in fear. Episodes generally last no more than a few minutes. It can reoccur multiple times or occur as a single episode. The condition may occur in those who are otherwise healthy or those with narcolepsy, or it may run in families as a result of specific genetic changes. The condition can be triggered by sleep deprivation, psychological stress, or abnormal sleep cycles. The underlying mechanism is believed to involve a dysfunction in REM sleep. Diagnosis is based on a person's description. Other conditions that can present similarly include narcolepsy, atonic seizure, and hypokalemic periodic paralysis. Treatment options for sleep paralysis have been poorly studied. It is recommended that people be reassured that ...
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Hypnopompia
Hypnopompia (also known as hypnopompic state) is the state of consciousness leading out of sleep, a term coined by the psychical researcher Frederic Myers. Its mirror is the hypnagogic state at sleep onset; though often conflated, the two states are not identical and have a different phenomenological character. Hypnopompic and hypnagogic hallucinations are frequently accompanied by sleep paralysis, which is a state wherein one is consciously aware of one's surroundings but unable to move or speak. Etymology Frederic Myers coined the term "hypnopompic", with its word-ending originating from the Greek word "pompos", meaning "sender", in 1904. Previously, in 1848, Alfred Maury introduced the term "hypnagogic" from the Greek words ύπνος (“hypnos”), meaning “sleep”, and αγωγός (“agōgos”), meaning “conductor” or “leader”. Hallucinations Hallucinations are commonly understood as "sensory perceptions that occur in the absence of an objective sti ...
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Hypnagogia
Hypnagogia is the transitional state from wakefulness to sleep, also defined as the waning state of consciousness during the onset of sleep. Its corresponding state is '' hypnopompia'' sleep to wakefulness. Mental phenomena that may occur during this "threshold consciousness" include hallucinations, lucid dreaming, and sleep paralysis. Etymology In 1848, Alfred Maury introduced the term "hypnagogic" from the Greek words ύπνος (“hypnos”), meaning “sleep”, and αγωγός (“agōgos”), meaning “conductor” or “leader”. Later, in 1904, Frederic Myers coined the term "hypnopompic," with its word-ending originating from the Greek word "pompos," meaning "sender." Definitions The word ''hypnagogia'' is sometimes used in a restricted sense to refer to the onset of sleep, and contrasted with '' hypnopompia'', Frederic Myers's term for waking up. However, ''hypnagogia'' is also regularly employed in a more general sense that covers both falling asleep and wa ...
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