Narcolepsy DSM-5 347.00 (ICD-10-CM Multiple Codes)
DSM-5 Category: Sleep-Wake Disorders
Introduction
Narcolepsy is a neurologically based, chronic, hypersomnic type of sleep disorder, which typically appears in childhood, adolescence or young adulthood. Despite adequate nocturnal sleep, the individual will experience daytime fatigue, or EDS (Excessive Daytime Sleepiness) (Psychologist World, 2014), and (University of Tennessee, 2014).The individual will experience sudden loss of muscle tonus ranging from isolated muscle weakness to bilateral, full body loss of muscle tonus, and will fall asleep in inappropriate times and places, which may place themselves or others in danger, if they are driving or doing another potentially hazardous activity. Narcolepsy is accompanied by unusually rapid onset of REM sleep (American Psychiatric Association, 2013). The sleep episodes will typically be triggered by a strong emotional response in the individual (Psychologist world, 2014), or a heavy meal, or general psychosocial stressors (Simon, 2012). Their should be an objective laboratory finding of abnormally low levels of hypocretin, a neuropeptide associated with sleep/wake and arousal levels, (Tsujino & Sakurai, 2009), and according to a polysomnography study, unusually low amounts of REM sleep during nocturnal sleep. Hypnogogic hallucinations (vivid dreams upon onset of sleep) hypnopompic hallucination (vivid dreams upon waking) and sleep paralysis (inability to move upon waking) can be part of the clinical presentation (Simon, 2012).
Symptoms of Narcolepsy
According to the DSM-5, (Diagnostic and Statistical Manual of Mental Disorders, fifth edition), narcolepsy is a disorder in which the individual will experience recurrent periods of an irresistible need to sleep, or will fall asleep, or nap within the same day, regardless of whether or not the time and place are appropriate. These episodes must occur at least three times per week over the past three months, accompanied by at least one of the following symptoms:
1. Episodes of cataplexy, or loss of muscle tonus, occurring several times a month, episodes lasting seconds to minutes of sudden full body loss of skeletal muscle tone without LOC (Loss Of Consciousness) that are precipitated by an episode of strong emotion. 2. In children or in individuals “within six months of narcolepsy onset, involuntary grimacing or jaw-opening with tongue thrusting, or hypotonia”, in the absence of clear emotional triggers.3. Objective measure of hypocretin deficiency in the cerebrospinal fluid (CSF) as defined by “less than or equal to 110 pg/mL”, excluding CSF levels of hypocretin-1 in the context of recent TBI . 4. Nocturnal sleep polysomnography study revealing abnormally low Rapid Eye Movement (REM) sleep duration ( < 15 minutes inclusive) or a multiple sleep latency test showing a mean sleep latency ( < eight minutes), and > two REM sleep episodes upon onset of sleep (American Psychiatric Association, 2013).REM sleep typically does not occur until an individual has gone through sleep stages One through Three. Individuals with narcolepsy will plunge into REM sleep upon onset of sleep (Psychologist World, 2014).
The clinician can include several additional specifiers to the narcolepsy diagnosis : “ 347.00 (G47.419) Narcolepsy without cataplexy, but with hypocretin deficiency, low CSF hypocretin-1 levels and positive polysomnography/multiple sleep latency test without cataplexy, 347.01 (G47.411) Narcolepsy with cataplexy but without low hypocretin levels. 347.00 (G47.419) Autosomal dominant cerebellar ataxia, deafness, and narcolepsy: (with low or intermediate CSF hypocretin-1 levels), deafness, cerebellar ataxia, and eventually dementia. 347.00 (G47.419) Autosomal dominant narcolepsy, obesity, and type 2 diabetes: Narcolepsy, obesity, and type 2 diabetes and low CSF hypocretin-1 levels
347.10 (G47.429) “ Narcolepsy can also be secondary to another medical condition: This subtype is for narcolepsy that has developed secondary to infectious, traumatic, or neoplasmic destruction of hypocretinergic neurons (American Psychiatric Association, 2013).
The clinician can also specify the severity of narcolepsy:
Mild: Infrequent cataplexy (< once per week), need for naps once or twice a day, and less disturbed nocturnal sleep. Moderate: Cataplexy once a day or every several days, disturbed nocturnal sleep, and need for daily naps Severe: medication resistant cataplexy with multiple daily episodes, persistent somnolesence and disturbed nocturnal sleep (e.g, excessive movements, insomnia, and vivid dreams).(American Psychiatric Association, 2013).
Onset
The typical onset of narcolepsy is between ages seven and 25. (NINDS, 2013).
Prevalence
About 200,000 Americans are diagnosed with narcolepsy. (Psychologist World, 2014) The DSM-5 notes that Narcolepsy affects both men and women, with slightly more men diagnosed. (American Psychiatric Association, 2013).
Risk Factors
Narcolepsy has a robust genetic component, with an eight to twelve percent incidence among people with first order relatives with narcolepsy (Psychologist World, 2014), or according to one source, a 20-40% higher risk than the general population (Simon, H. 2012).
Comorbidity
The DSM-5 indicates that Narcolepsy can be comorbid with bipolar disorder, depressive disorders, anxiety disorders, and sometimes schizophrenia. Narcolepsy is correlated with increased body mass index or obesity, particularly when left untreated. Rapid weight gain is commonly seen in children with sudden onset of narcolepsy. Sleep apnea should also be ruled out if there is a sudden aggravation of previously diagnosed narcolepsy. (American Psychiatric Association, 2013).
Treatment Options for Narcolepsy
Narcolepsy is a neurologically based disorder which will typically require pharmacological intervention to relieve symptoms. CBT (Cognitive Behavioral Therapy) may be a useful adjunct to deal with associated anxiety, or to assist with making lifestyle changes which could ameliorate symptoms. (American Psychiatric Association, 2013).
Impact on Functioning
Narcolepsy, symptoms will often be of sufficient severity to seriously disrupt social, personal, and occupational functioning, and severely curtail daily activities (Psychologist world, 2014) The DSM-5 notes that Individuals with narcolepsy will incur risk at occupations involving driving vehicles or operating heavy machinery,. When the narcolepsy is appropriately managed, patients can usually drive, though it is ill advised for them to drive for long distances without another adult. Untreated individuals are at risk for social isolation and accidental injury to themselves or others. (American Psychiatric Association, 2013). Narcolepsy is sometimes accompanied by Sleep paralysis and hypnogogic and hypnopompic hallucinations. The hallucinations can be visual, auditory, or tactile (Simon, 2012).When this is combined with a loss of voluntary muscle control, this can be a very distressing experience. Clinicians should be aware that there is a long cross- cultural history of this being attributed to supernatural causes, involving entities called incubi or succubi, or the Hag, or “ alien abduction”. This is mostly attributed to nocturnal sleep paralysis and associated hallucinatory experiences. An individual with rigid beliefs in this attribution will experience additional distress. (“The True Night-Mare”).
References
American Psychiatric Association. (2013). Diagnostic and Statistical Manual of Mental Disorders. (5th Edition). Washington, DC.
NINDS. (2013). Narcolepsy Fact Sheet. National Institute of Neurological Disorders and Stroke. Retrieved February 21, 2014, from: http://www.ninds.nih.gov/disorders/narcolepsy/detail_narcolepsy.htm
Psychologist world. (2014). Narcolepsy. Psychologist World. Retrieved February 22, 2014 from: http://www.psychologistworld.com/issue/narcolepsy.php
Simon, H. (2012). Narcolepsy. University of Maryland Medical Center. Retrieved February 21, 2014 from: https://umm.edu/health/medical/reports/articles/narcolepsy
The True Night-Mare: SP in Myth and Legend. A webpage about Sleep Paralysis and Associated Hypnogogic and Hypnopompic Experiences. Retrieved February 22, 2014 from: http://watarts.uwaterloo.ca/~acheyne/night_mare.html
Tsujino, N. and Sakurai, T. (2009). Orexin/Hypocretin: A Neuropeptide at the Interface of Sleep, Energy Homeostasis, and Reward System. Pharmacological Reviews.6. (2). 162-176. doi: 10.1124/pr.109.001321
University of Tennessee..(2014). Narcolepsy /Other Hypersomnias (Excessive Sleep). The University of Tennessee Medical Center. Retrieved February 21, 2014 from http://www.utsleepdisorders.org/sleep-disorders/narcolepsy-other-hypersomnias-excessive-sleep/
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