narcolepsy
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Etiology
- cataplexy
- altered sleep rapid-eye movement patterns
- defects in noradrenergic or dopamine receptors is suggested from research in dogs[2]
Epidemiology
- HLA linkage: HLA DR-2 & Dqw1
- onset in adolescence or young adulthood
- effects both sexes equally
- occurs in 0.05% of US population
Genetics
- 60-fold increased incidence in children of narcoleptics, but no clear Mendelian pattern of inheritance
- association with HLA-DR2, DQw1 & Dw2
Clinical manifestations
- sleepiness
- sleep attacks (generally initial manifestation)
- total sleep in 24 hour period is not increased
- daytime naps usually refreshing
- automatic behaviors
- microsleep periods invading awakened activities
- patient with diminished awareness
- cataplexy (80%)
- pathognomonic for narcolepsy
- may develop several years after initial sleep attacks
- sleep paralysis
- hypnagogic hallucinations (68%)
- vivid dream-like experiences which the patient cannot distinguish from reality
- may occur during daytime naps
- may occur as patient is falling off to sleep
- disturbed nocturnal sleep (87%)
- memory impairment (50%)
Laboratory
- HLA typing indicated only in cases that do not fit standard pattern
- thyroid function tests
- narcolepsy-associated Ag in serum/plasma
- see ARUP consult[2]
Diagnostic procedures
- overnight sleep study (polysomnography)
- may be evidence of REM sleep disorder
- multiple sleep latency test (MSLT)
Radiology
Complications
- stroke (RR=2.5)
- myocardial infarction (RR=1.6)
- cardiac arrest (RR=1.6)
- heart failure (RR=2.6)
- comorbidities (metabolic syndrome) common
Differential diagnosis
- sleep apnea
- nocturnal myoclonus
- idiopathic hypersomnolence
- psychiatric disorder
- antisocial personality disorder
- hypothyroidism
- hydrocephalus
- absence seizures
- vertebrobasilar insufficiency
Management
- general recommendations
- brief daytime naps as needed
- avoid sleep deprivation
- analeptics
- modafinil (drug of choice)[5]
- pemoline (Cylert) 37.5-150 mg QD or divided BID
- methylphenidate (Ritalin) 20-60 mg QD divided BID/TID
- dextroamphetamine or methamphetamine 20-60 mg QD or divided BID
- symptoms of cataplexy
- tricyclic antidepressants
- clomipramine (Anafranil) 25-75 mg QD
- protriptyline (Vivactil) 5-20 mg QD
- imipramine (Tofranil) 50-75 mg QHS
- may be synergistic with protriptyline
- fluoxetine (Prozac) 20-80 mg QD
- tricyclic antidepressants
- experimental agents
- prognosis
More general terms
Additional terms
- cataplexy
- hypersomnia (hypersomnolence, excessive sleepiness)
- multiple sleep latency test (MSLT)
- sleep paralysis
References
- ↑ Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 1038-39
- ↑ 2.0 2.1 2.2 Medical Knowledge Self Assessment Program (MKSAP) 11, American College of Physicians, Philadelphia 1998
- ↑ Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 155
- ↑ Nightingale S, Orgill JC, Ebrahim IO, de Lacy SF, Agrawal S, Williams AJ. The association between narcolepsy and REM behavior disorder (RBD). Sleep Med. 2005 May;6(3):253-8. PMID: https://www.ncbi.nlm.nih.gov/pubmed/15854856
- ↑ 5.0 5.1 Morgenthaler TI et al, Practice parameters for the treatment of narcolepsy and other hypersomnias of central origin. Sleep 2007, 30:1705 PMID: https://www.ncbi.nlm.nih.gov/pubmed/18246980
- ↑ ARUP Consult: Narcolepsy The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/narcolepsy
- ↑ Scammell TE Narcolepsy. N Engl J Med 2015; 373:2654-2662. December 31, 2015. PMID: https://www.ncbi.nlm.nih.gov/pubmed/26716917
- ↑ 8.0 8.1 WebMD Professional Clinical Update. Nov 3, 2020 Narcolepsy and Prevalence of Comorbidities.
Ohayon MM. Narcolepsy is complicated by high medical and psychiatric comorbidities: a comparison with the general population. Sleep Med. 2013;14(6):488-492. PMID: https://www.ncbi.nlm.nih.gov/pubmed/23643648
Black J, Reaven NL, Funk SE, et al. Medical comorbidity in narcolepsy: findings from the Burden of Narcolepsy Disease (BOND) study. Sleep Med. 2017;33:13-18. PMID: https://www.ncbi.nlm.nih.gov/pubmed/28449892
Cohen A, Mandrekar J, St Louis EK, Silber MH, Kotagal S. Comorbidities in a community sample of narcolepsy. Sleep Med. 2018;43:14-18. PMID: https://www.ncbi.nlm.nih.gov/pubmed/29482805 Free PMC article. - ↑ Facts about Narcolepy http://www.nhlbi.nih.gov/health/public/sleep/narcolep.htm
NINDS Narcolepsy Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Narcolepsy-Information-Page