what is Narcolepsy?

Narcolepsy, Symptoms, Causes and Treatment

What is Narcolepsy?

Narcolepsy is a chronic sleep disorder that usually begins in infancy or adolescence. Patients suffer severe effects as a result of excessive sleepiness. Social stigma, bodily and mental comorbidities, difficulty acquiring an education and holding a career, and a lower quality of life are issues. Narcolepsy has significant socioeconomic implications. Severe, uncontrollable daytime sleepiness, disconnected REM sleep experiences, interrupted nighttime sleep, and rapid changes in REM sleep are all indications of narcolepsy. 

Cataplexy, a particular symptom of narcolepsy, affects about two-thirds of confirmed narcoleptic individuals. Cataplexy is characterized by a rapid loss of muscle effect caused by feelings such as laughing, joking, and wrath on rare occasions. Narcolepsy is also linked to “automatic conduct,” when you do anything without thinking about it. Narcolepsy affects about one out of every 2,000 people, but max researchers think that many people with the illness go untreated or are misdiagnosed. 

Narcolepsy appears to be an autoimmune condition, according to growing research. When the body’s immune system targets healthy tissue or cells, autoimmune diseases develop. The immune system breaks particular brain cells that produce the peptide hypocretin in narcolepsy. Hypocretin affects various brain activities, but the exact nature of its effects is unknown. It’s unclear why the immune system targets healthy cells in narcolepsy, and other environmental and genetic variables could play a significant role in the disorder’s evolution.

Symptoms of Narcolepsy

The progression and intensity of narcolepsy symptoms differ widely from one person to another. The signs happen at a time initially; new symptoms may occur years apart, with tiredness usually coming before cataplexy. Narcolepsy commonly starts in adolescence, with minor symptoms that increase with age. Symptoms may not alter for months at the moment, or they may change quite fast. Narcolepsy can significantly impact a person’s daily routine, interrupting all facets of their lives based on the danger of the illness.

The problem with Narcolepsy can also be sleep schedules at night. People affected wake up during the night and maybe fully awake for long periods. Despite their interrupted sleeping habits, people with narcolepsy have a typical overall sleep duration of 24 hours because they sleep for a brief time throughout the day and night. Some patients with narcolepsy do not experience cataplexy, so cataplexy is not required for a narcolepsy diagnosis. 

Cataplexy commonly appears many weeks to months once excessive daytime tiredness appears. In rare instances, cataplexy may lead to difficulties in managing wakefulness. For a short period, People with narcolepsy may have “sleep paralysis.” They would be unable to use their muscles or talk for a short time. Sleep paralysis lasts for a few minutes and usually occurs when trying to sleep or awake. Following these brief instances, the people with this disorder regain all of their motions.

Some people may encounter additional symptoms, including weariness, sadness, trouble focusing, and memory issues. Individuals with narcolepsy have also been found to experience periodic body movement and sleep apnea.

Causes of Narcolepsy

Low levels of a brain substance called hypocretin have been associated with narcolepsy with cataplexy, also called orexin. This molecule is crucial in the regulation of sleep as well as other bodily activities. Hypocretin is a neurotransmitter, a substance that changes, amplifies, or transfers nerve impulses through one nerve cell to another, allowing nerve cells to interact. According to research, the number of hidden neurons that generate hypocretin decreases in those with narcolepsy.

The hypothalamus, a brain area that controls several activities such as sleep, hunger, and temperature, contains hypocretin-producing neurons. 80-90 % of hypocretin-producing neurons in the hypothalamus are destroyed in certain people. Cataplexy-related narcolepsy patients had the lowest rate of hypocretin.

Several occurrences of narcolepsy are firmly linked to a collection of genes of human chromosome 6 stating that human leukocyte antigen (HLA) complex. Several genes have a role in controlling the immune system’s accurate method. The precise role and importance of these HLAs in narcolepsy are unknown. Most HLA complex illnesses include an immunological component, either due to autoimmunity or an inappropriate immune system reaction to a foreign substance. According to researchers, HLA and the mutant T cell present in people with narcolepsy are thought to interact in a way that destroys brain cells that generate hypocretin.

Populations Affected By Narcolepsy

The actual number of populations in the United States who suffer from narcolepsy is unclear. According to estimates, the frequency in different peoples worldwide ranges from.03 % to.16 % population. According to research, new occurrences of narcolepsy occur at a rate of.74 per 100,000 person-years in the overall population in the U.S.  The condition is thought to afflict one out of every 2,000 persons in the overall population. However, assessing the actual prevalence of narcolepsy in the overall population is challenging since it is frequently misdiagnosed or undiagnosed.

Narcolepsy can appear at any age, from early childhood to 50. There are two prime times: one about  15 years and the other about 36 years of age. A few researchers think that narcolepsy in children is underdiagnosed. Narcolepsy is likely to stay a lifelong condition. The disease is not chronic, even though the kind and degree of symptoms reported by a person affected may change with time.

Related Disruptions

The following conditions have symptoms that are comparable to narcolepsy. For a diagnostic process, similarities may be helpful:

Idiopathic hypersomnia is an uncommon condition marked by periods of excessive sleepiness for no apparent reason. Chronic or recurrent episodes are possible. The illness is distinct from narcolepsy in that patients do not have abrupt sleeping episodes or acquire cataplexy. Furthermore, those who are impacted do not feel fresh after sleep. Idiopathic hypersomnia causes some people to sleep longer than 10 hours, while others sleep for shorter periods than 10 hours. Many parts of life can be disrupted by idiopathic hypersomnia. The condition is treated with behavioural change and some medications.

Sleep apnea is a severe sleep condition marked by frequent, brief pauses in breathing while sleeping. Symptoms of the condition include numerous nighttime disruptions, severe daytime tiredness, loud snoring, irritation,  impaired focus and intellect. Overweight, particularly neck obesity and narrowed airways in the neck, is frequently linked to sleep apnea. Airway closure interrupts laborious breathing in obstructive syndrome, the most prevalent kind of sleep apnea.

Brain tumours, head trauma, blood clot in the brain, psychosis, and excessive protein in the blood owing to renal failure can all cause symptoms similar to narcolepsy. Excessive daytime sleepiness can be caused by hypothyroidism, delayed sleep phase disorder, periodic limb movement disorder, depression, hypoglycemia, and other diseases.

Basic Treatments

The therapy for narcolepsy focuses on the exact symptoms that each person feels. Various narcolepsy medications may assist in easing these symptoms.

Several stimulants, such as modafinil, may be used to treat people who have severe daytime sleepiness and sleep attacks. The Food and Drug Administration (FDA) authorised modafinil to cure narcolepsy’s excessive daytime sleep. For severe daytime sleepiness, modafinil is presently the most commonly recommended medicine. The drug’s mode of action appears to be distinct from other stimulants, as it does not affect alertness or memory.

Furthermore, data shows that modafinil medication is not linked to reliance or side effects, suggesting it might be a viable option to conventional therapies for excessive daytime sleepiness. Modafinil has fewer adverse effects than earlier medicines tried to treat this problem.

Methylphenidate, methamphetamine, and dextroamphetamine were once used to treat excessive daytime drowsiness in narcolepsy. When modafinil fails, these medications stimulate the central nervous system and are still employed. Because such drugs may induce side effects such as anxiousness, sleeplessness, or irritability, physicians must closely monitor their patients to ensure proper dose changes and therapeutic success. If therapy is stopped, doctors may need to keep a careful eye on you and follow up with you for a long time.

Many people have benefitted from a behavioral change in addition to pharmacological therapy. Individuals with narcolepsy should maintain standard sleep patterns by establishing regular bedtime hours and avoiding sleep disturbances. Taking frequent naps during the day, if possible, can help reduce excessive daytime drowsiness. Exercise is also advised regularly. Affected people should talk to their doctors about creating a sleep routine that works for them.

Resources

RareConnect offers a safe patient-hosted online community for patients and caregivers affected by this rare disease.  For more information, visit www.rareconnect.org.

Other Organizations

References

TEXTBOOKS

Goswami M. Narcolepsy. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:561-2.

Fauci AS, et al., eds. Harrison’s Principles of Internal Medicine. 14th ed. New York, NY: McGraw-Hill Companies, Inc.; 1998:155.

Wyngaarden JB, et al., eds. Cecil Textbook of Medicine. 19th ed. Philadelphia, PA: W.B. Saunders Company; 1992:2065-2066.

Adams RD, et al., eds. Principles of Neurology. 4th ed. New York, NY: McGraw-Hill Companies, Inc.; 1989:314-317.

JOURNAL ARTICLES

  • Szakacs Z, Dauvilliers Y, Mikhaylov V, Poverennova I, Krylov S, Jankovic S, et al. Safety and efficacy of pitolisant on cataplexy in patients with narcolepsy: a randomised, double-blind, placebo-controlled trial. Lancet Neurol 2017. doi:10.1016/S1474-4422(16)30333-7.
  • Ruoff C, Swick TJ, Doekel R, Emsellem HA, Feldman NT, Rosenberg R, et al. Effect of Oral JZP-110 (ADX-N05) on Wakefulness and Sleepiness in Adults with Narcolepsy: A Phase 2b Study. Sleep 2016;39:1379–87. doi:10.5665/sleep.5968.
  • Sullivan SS, Guilleminault C. Emerging drugs for common conditions of sleepiness: obstructive sleep apnea and narcolepsy. Expert Opin Emerg Drugs 2015;20:571–82. doi:10.1517/14728214.2015.1115480.
  • Hallmayer J, Faraco J, Lin L, et al. Narcolepsy is strongly associated with the T-cell receptor alpha locus. Nat Genet. 2009;41:708-711.
  • Billiard M. Narcolepsy: current treatment options and future approaches. Neuropsychiatr Dis Treat. 2008;4:557-566.
  • Alaez C, Lin L, Flore A-H, et al. Association of narcolepsy-cataplexy with HLA-DRB1 and DQB1 in Mexican patients: a relationship between HLA and gender is suggested. BMC Med Genet. 2008;9:79.
  • Black J, Duntley SP, Bogan RK, O’Malley MB. Recent advances in the treatment and management of excessive daytime sleepiness. CNS Spectr. 2007;12:1-14.
  • Dauvilliers Y, Arnulf I, Mignot E. Narcolepsy with cataplexy. Lancet. 2007;369:499-511.
  • Wise MS, Arand DL, Auger RR, Brooks SN, Watson NF. Treatment of narcolepsy and other hypersomnias of central origin. Sleep. 2007;30:1712-1727.
  • Nishino S. Clinical and neurobiological aspects of narcolepsy. Sleep Med. 2007;8:373-399.
  • Longstreth Jr. WT, Koepsell TD, Ton TG, Hendrickson AF, van Belle G. The epidemiology of narcolepsy. Sleep. 2007;30:13-26.
  • Dauvilliers Y, Carlander B, Billiard M. Narcolepsy, from Westphal to hypocretin. Presse Med. 2004;33:1593-600.
  • Dauvilliers Y, Carlander B, Rivier F, Touchon J, Tafti M. Successful management of cataplexy with intravenous immunoglobulins at narcolepsy onset. Ann Neurol. 2004;56:905-8.
  • Mignot E. Sleep, sleep disorders and hypocretin (orexin). Sleep Med. 2004;5:S2-8.
  • McKenna L, McNicholas F, Childhood onset narcolepsy. A case report. Eur Child Adolesc Psychiatry. 2003;12:43-7.
  • Hood Bm, Harbord MG, Paediatric narcolepsy: complexities of diagnosis. J Paediatr Child Health. 2002;38:618-21.
  • Randomized trial of modafinil as a treatment for the excessive daytime somnolence of narcolepsy: US Modafinil in Narcolepsy Multicenter Study Group. Neurology. 2000;54:1166-1175.
  • Lin L, et al. The sleep disorder canine narcolepsy is caused by a mutation in the hypocretin (orexin) receptor 2 gene. Cell. 1999;98:365-376. [See comment in Cell. 1999;98:409-412.]

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