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Review Article

Narcolepsy: Clinical Decision Making for the Primary Care Physician

Faisal Khan MD, Ribhi Hazin MD, Mohsin Iqbal MD
Volume: 102 Issue: 12 December, 2009

Abstract:

Abstract:Narcolepsy is a serious neurological condition in which patients are overcome by persistent, excessive feelings of fatigue and drowsiness. In addition to chronic fatigue, patients with narcolepsy often succumb to intermittent, uncontrollable periods where they abruptly fall asleep during waking hours. In addition to episodic bouts of daytime sleeping, narcoleptics also exhibit cataplexy, sleep paralysis, and hypnagogic and hypnopompic hallucinations. Unfortunately, many individuals with narcolepsy remain undiagnosed and therefore, untreated, posing a risk to themselves and those around them. There is currently no cure for this lifelong disease. Nonetheless, narcolepsy can be effectively managed with medications, lifestyle changes, and the peripheral support of individuals such as family members, coworkers, and other casual relations.

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References:

References1. Rye DB, Dihenia B, Weissman JD, et al. Presentation of narcolepsy after 40. Neurology 1998;50:459–465.RyeDB]]DiheniaB]]WeissmanJD&etal;Presentation of narcolepsy after 40.Neurology199850459-4652. Nishino S, Okura M, Mignot E. Narcolepsy: genetic predisposition and neuropharmacological mechanisms. REVIEW ARTICLE. Sleep Med Rev 2000;4:57–99.NishinoS]]OkuraM]]MignotENarcolepsy: genetic predisposition and neuropharmacological mechanisms. REVIEW ARTICLE.Sleep Med Rev2000457-993. Ocasio-Tascón ME, Alicea-Colón E, Torres-Palacios A, et al. The veteran population: one at high risk for sleep-disordered breathing. Sleep Breath 2006;10:70–75.Ocasio-TascónME]]Alicea-ColónE]]Torres-PalaciosA&etal;The veteran population: one at high risk for sleep-disordered breathing.Sleep Breath20061070-754. Mustafa M, Erokwu N, Ebose I, et al. Sleep problems and the risk for sleep disorders in an outpatient veteran population. Sleep Breath 2005;9:57–63.MustafaM]]ErokwuN]]EboseI&etal;Sleep problems and the risk for sleep disorders in an outpatient veteran population.Sleep Breath2005957-635. Nevsimalova S, Prihodova I, Kemlink D, et al. REM behavior disorder (RBD) can be one of the first symptoms of childhood narcolepsy. Sleep Med 2007;8:784–786.NevsimalovaS]]PrihodovaI]]KemlinkD&etal;REM behavior disorder (RBD) can be one of the first symptoms of childhood narcolepsy.Sleep Med20078784-7866. Ohayon MM, Ferini-Strambi L, Plazzi G, et al. Frequency of narcolepsy symptoms and other sleep disorders in narcoleptic patients and their first-degree relatives. J Sleep Res 2005;14:437–445.OhayonMM]]Ferini-StrambiL]]PlazziG&etal;Frequency of narcolepsy symptoms and other sleep disorders in narcoleptic patients and their first-degree relatives.J Sleep Res200514437-4457. Faull KF, Thiemann S, King RJ, et al. Monoamine interactions in narcolepsy and hypersomnia: a preliminary report. Sleep 1986;9:246–249.FaullKF]]ThiemannS]]KingRJ&etal;Monoamine interactions in narcolepsy and hypersomnia: a preliminary report.Sleep19869246-2498. Gugger JJ, Wagner ML. Rapid eye movement sleep behavior disorder. Ann Pharmacother 2007;41:1833–1841.GuggerJJ]]WagnerMLRapid eye movement sleep behavior disorder.Ann Pharmacother2007411833-18419. Nishino S. The hypocretin/orexin system in health and disease. Biol Psychiatry 2003;54:87–95.NishinoSThe hypocretin/orexin system in health and disease.Biol Psychiatry20035487-9510. Alaez C, Lin L, Flores-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.AlaezC]]LinL]]Flores-AH&etal;Association of narcolepsy-cataplexy with HLA-DRB1 and DQB1 in Mexican patients: a relationship between HLA and gender is suggested.BMC Med Genet200897911. Serra L, Montagna P, Mignot E, et al. Cataplexy features in childhood narcolepsy. Mov Disord 2008;23:858–865.SerraL]]MontagnaP]]MignotE&etal;Cataplexy features in childhood narcolepsy.Mov Disord200823858-86512. Ton TG, Longstreth WT Jr, Koepsell T. Active and passive smoking and risk of narcolepsy in people with HLA DQB1*0602: a population-based case-control study. Neuroepidemiology 2008;32:114–121.TonTG]]LongstrethWTJr]]KoepsellTActive and passive smoking and risk of narcolepsy in people with HLA DQB1*0602: a population-based case-control study.Neuroepidemiology200832114-12113. Mignot E. Genetic and familial aspects of narcolepsy. Neurology 1998;50(2 suppl 1):S16–S22.MignotEGenetic and familial aspects of narcolepsy.Neurology199850S16-S2214. Nishino S, Ripley B, Overeem S, et al. Low cerebrospinal fluid hypocretin (Orexin) and altered energy homeostasis in human narcolepsy. Ann Neurol 2001;50:381–388.NishinoS]]RipleyB]]OvereemS&etal;Low cerebrospinal fluid hypocretin (Orexin) and altered energy homeostasis in human narcolepsy.Ann Neurol200150381-38815. Heier MS, Evsiukova T, Vilming S, et al. CSF hypocretin-1 levels and clinical profiles in narcolepsy and idiopathic CNS hypersomnia in Norway. Sleep 2007;30:969–973.HeierMS]]EvsiukovaT]]VilmingS&etal;CSF hypocretin-1 levels and clinical profiles in narcolepsy and idiopathic CNS hypersomnia in Norway.Sleep200730969-97316. Mignot E, Lammers GJ, Ripley B, et al. The role of cerebrospinal fluid hypocretin measurement in the diagnosis of narcolepsy and other hypersomnias. Arch Neurol 2002;59:1553–1562.MignotE]]LammersGJ]]RipleyB&etal;The role of cerebrospinal fluid hypocretin measurement in the diagnosis of narcolepsy and other hypersomnias.Arch Neurol2002591553-156217. Liu M, Thankachan S, Kaur S, et al. Orexin (hypocretin) gene transfer diminishes narcoleptic sleep behavior in mice. Eur J Neurosci 2008 Oct;28:1382–139318. Taheri S, Zeitzer JM, Mignot E. The role of hypocretins (orexins) in sleep regulation and narcolepsy. Annu Rev Neurosci 2002;25:283–313.TaheriS]]ZeitzerJM]]MignotEThe role of hypocretins (orexins) in sleep regulation and narcolepsy.Annu Rev Neurosci200225283-31319. Wurtman RJ. Narcolepsy and the hypocretins. Metabolism 2006;55:S36–S39.WurtmanRJNarcolepsy and the hypocretins.Metabolism200655S36-S3920. Nishino S. Clinical and neurobiological aspects of narcolepsy. Sleep Med 2007;8:373–399.NishinoSClinical and neurobiological aspects of narcolepsy.Sleep Med20078373-39921. Nishino S, Tafti M, Reid MS, et al. Muscle atonia is triggered by cholinergic stimulation of the basal forebrain: implication for the pathophysiology of canine narcolepsy. J Neurosci 1995;15:4806–4814.NishinoS]]TaftiM]]ReidMS&etal;Muscle atonia is triggered by cholinergic stimulation of the basal forebrain: implication for the pathophysiology of canine narcolepsy.J Neurosci1995154806-481422. Nishino S, Mignot E. Pharmacological aspects of human and canine narcolepsy. Prog Neurobiol 1997;52:27–78.NishinoS]]MignotEPharmacological aspects of human and canine narcolepsy.Prog Neurobiol19975227-7823. Ebadi M, Brown-Borg H, Ren J, et al. Therapeutic efficacy of selegiline in neurodegenerative disorders and neurological diseases. Curr Drug Targets 2006;7:1513–1529.EbadiM]]Brown-BorgH]]RenJ&etal;Therapeutic efficacy of selegiline in neurodegenerative disorders and neurological diseases.Curr Drug Targets200671513-152924. Mayer G, Ewert Meier K, Hephata K. Selegeline hydrochloride treatment in narcolepsy. A double-blind, placebo-controlled study. Clin Neuropharmacol 1995;18:306–319.MayerG]]Ewert MeierK]]HephataKSelegeline hydrochloride treatment in narcolepsy. A double-blind, placebo-controlled study.Clin Neuropharmacol199518306-31925. Reid MS, Tafti M, Nishino S, et al. Cholinergic regulation of cataplexy in canine narcolepsy in the pontine reticular formation is mediated by M2 muscarinic receptors. Sleep 1994;17:424–435.ReidMS]]TaftiM]]NishinoS&etal;Cholinergic regulation of cataplexy in canine narcolepsy in the pontine reticular formation is mediated by M2 muscarinic receptors.Sleep199417424-43526. D’Alessandro R, Rinaldi R, Cristina E, et al. Prevalence of excessive daytime sleepiness an open epidemiological problem. Sleep 1995;18:389–391.D’AlessandroR]]RinaldiR]]CristinaE&etal;Prevalence of excessive daytime sleepiness an open epidemiological problem.Sleep199518389-39127. Franceschi M, Zamproni P, Crippa D, et al. Excessive daytime sleepiness: a 1-year study in an unselected inpatient population. Sleep 1982;5:239–247.FranceschiM]]ZamproniP]]CrippaD&etal;Excessive daytime sleepiness: a 1-year study in an unselected inpatient population.Sleep19825239-24728. Silber MH, Krahn LE, Olson EJ, et al. The epidemiology of narcolepsy in Olmsted County, Minnesota: a population-based study. Sleep 2002;25:197–202.SilberMH]]KrahnLE]]OlsonEJ&etal;The epidemiology of narcolepsy in Olmsted County, Minnesota: a population-based study.Sleep200225197-20229. Benca RM. Narcolepsy and excessive daytime sleepiness: diagnostic considerations, epidemiology, and comorbidities. J Clin Psychiatry 2007;68:5–8.BencaRMNarcolepsy and excessive daytime sleepiness: diagnostic considerations, epidemiology, and comorbidities.J Clin Psychiatry2007685-830. Aldrich MS. The clinical spectrum of narcolepsy and idiopathic hypersomnia. Neurology 1996;46:393–401.AldrichMSThe clinical spectrum of narcolepsy and idiopathic hypersomnia.Neurology199646393-40131. Silber MH. Narcolepsy, head injury, and the problem of causality. J Clin Sleep Med 2005;1:157–158.SilberMHNarcolepsy, head injury, and the problem of causality.J Clin Sleep Med20051157-15832. Ebrahim IO, Peacock KW, Williams AJ. Posttraumatic narcolepsy–two case reports and a mini review. J Clin Sleep Med 2005;1:153–156.EbrahimIO]]PeacockKW]]WilliamsAJPosttraumatic narcolepsy–two case reports and a mini review.J Clin Sleep Med20051153-15633. Donadio V, Plazzi G, Vandi S, et al. Sympathetic and cardiovascular activity during cataplexy in narcolepsy. J Sleep Res 2008;17:458–463.DonadioV]]PlazziG]]VandiS&etal;Sympathetic and cardiovascular activity during cataplexy in narcolepsy.J Sleep Res200817458-46334. Sandyk R. Resolution of partial cataplexy in multiple sclerosis by treatment with weak electromagnetic fields. Int J Neurosci 1996;84:157–164.SandykRResolution of partial cataplexy in multiple sclerosis by treatment with weak electromagnetic fields.Int J Neurosci199684157-16435. Moore WR, Silber MH, Decker PA, et al. Cataplexy Emotional Trigger Questionnaire (CETQ)—a brief patient screen to identify cataplexy in patients with narcolepsy. J Clin Sleep Med 2007;3:37–40.MooreWR]]SilberMH]]DeckerPA&etal;Cataplexy Emotional Trigger Questionnaire (CETQ)—a brief patient screen to identify cataplexy in patients with narcolepsy.J Clin Sleep Med2007337-4036. Krahn LE, Boeve BF, Olson EJ, et al. A standardized test for cataplexy. Sleep Med 2000;1:125–130.KrahnLE]]BoeveBF]]OlsonEJ&etal;A standardized test for cataplexy.Sleep Med20001125-13037. Anic-Labat S, Guilleminault C, Kraemer HC, et al. Validation of a cataplexy questionnaire in 983 sleep-disorders patients. Sleep 1999;22:77–87.Anic-LabatS]]GuilleminaultC]]KraemerHC&etal;Validation of a cataplexy questionnaire in 983 sleep-disorders patients.Sleep19992277-8738. Mattarozzi K, Bellucci C, Campi C, et al. Clinical, behavioural and polysomnographic correlates of cataplexy in patients with narcolepsy/cataplexy. Sleep Med 2008;9:425–433.MattarozziK]]BellucciC]]CampiC&etal;Clinical, behavioural and polysomnographic correlates of cataplexy in patients with narcolepsy/cataplexy.Sleep Med20089425-43339. McNally RJ, Clancy SA. Sleep paralysis in adults reporting repressed, recovered, or continuous memories of childhood sexual abuse. J Anxiety Disord 2005;19:595–602.McNallyRJ]]ClancySASleep paralysis in adults reporting repressed, recovered, or continuous memories of childhood sexual abuse.J Anxiety Disord200519595-60240. Szklo-Coxe M, Young T, Finn L, et al. Depression: relationships to sleep paralysis and other sleep disturbances in a community sample. J Sleep Res 2007;16:297–312.Szklo-CoxeM]]YoungT]]FinnL&etal;Depression: relationships to sleep paralysis and other sleep disturbances in a community sample.J Sleep Res200716297-31241. Cheyne JA, Newby-Clark IR, Rueffer SD. Relations among hypnagogic and hypnopompic experiences associated with sleep paralysis. J Sleep Res 1999;8:313–317.CheyneJA]]Newby-ClarkIR]]RuefferSDRelations among hypnagogic and hypnopompic experiences associated with sleep paralysis.J Sleep Res19998313-31742. Choong C, Hunter MD, Woodruff PW. Auditory hallucinations in those populations that do not suffer from schizophrenia. Curr Psychiatry Rep 2007;9:206–212.ChoongC]]HunterMD]]WoodruffPWAuditory hallucinations in those populations that do not suffer from schizophrenia.Curr Psychiatry Rep20079206-21243. Kishi Y, Konishi S, Koizumi S, et al. Schizophrenia and narcolepsy: a review with a case report. Psychiatry Clin Neurosci 2004;58:117–124.KishiY]]KonishiS]]KoizumiS&etal;Schizophrenia and narcolepsy: a review with a case report.Psychiatry Clin Neurosci200458117-12444. Mahowald MW. What is causing excessive daytime sleepiness? Evaluation to distinguish sleep deprivation from sleep disorders. Postgrad Med 2000;107:108–110, 115–118, 123.MahowaldMWWhat is causing excessive daytime sleepiness? Evaluation to distinguish sleep deprivation from sleep disorders.Postgrad Med2000107108-110, 115-118, 12345. Thorpy M. Therapeutic advances in narcolepsy. Sleep Med 2007;8:427–440.ThorpyMTherapeutic advances in narcolepsy.Sleep Med20078427-44046. Britton T, Douglas N, Hansen A, et al. Guidelines on the Diagnosis and Management of Narcolepsy in Adults and Children. Ashtead, Surrey, Taylor Patten Communications, 2002.BrittonT]]DouglasN]]HansenA&etal;Guidelines on the Diagnosis and Management of Narcolepsy in Adults and Children.Ashtead, SurreyTaylor Patten Communications200247. Hong SC, Lin L, Jeong JH, et al. A study of the diagnostic utility of HLA typing, CSF hypocretin-1 measurements, and MSLT testing for the diagnosis of narcolepsy in 163 Korean patients with unexplained excessive daytime sleepiness. Sleep 2006;29:1429–1438.HongSC]]LinL]]JeongJH&etal;A study of the diagnostic utility of HLA typing, CSF hypocretin-1 measurements, and MSLT testing for the diagnosis of narcolepsy in 163 Korean patients with unexplained excessive daytime sleepiness.Sleep2006291429-143848. Aldrich MS, Chervin RD, Malow BA. Value of the multiple sleep latency test (MSLT) for the diagnosis of narcolepsy. Sleep 1997;20:620–629.AldrichMS]]ChervinRD]]MalowBAValue of the multiple sleep latency test (MSLT) for the diagnosis of narcolepsy.Sleep199720620-62949. American Academy of Sleep Medicine. ICSD-2-International Classification of Sleep Disorders. Diagnostic and Coding Manual. Westchester, IL, American Academy of Sleep Medicine Westchester, 2005, ed 2.50. Bassetti C, Aldrich MS, Quint DJ. MRI findings in narcolepsy. Sleep 1997;20:630–631.BassettiC]]AldrichMS]]QuintDJMRI findings in narcolepsy.Sleep199720630-63151. Keam S, Walker MC. Therapies for narcolepsy with or without cataplexy: evidence-based review. Curr Opin Neurol 2007;20:699–703.KeamS]]WalkerMCTherapies for narcolepsy with or without cataplexy: evidence-based review.Curr Opin Neurol200720699-70352. Valentino RM, Foldvary-Schaefer N. Modafinil in the treatment of excessive daytime sleepiness. Cleve Clin J Med 2007;74:561–566, 568–571.ValentinoRM]]Foldvary-SchaeferNModafinil in the treatment of excessive daytime sleepiness.Cleve Clin J Med200774561-566, 568-57153. Vignatelli L, D’Alessandro R, Candelise L. Antidepressant drugs for narcolepsy. Cochrane Database Syst Rev 2005;3:CD003724.VignatelliL]]D’AlessandroR]]CandeliseLAntidepressant drugs for narcolepsy.Cochrane Database Syst Rev20053CD00372454. Lammers GJ, Arends J, Declerck AC, et al. Gammahydroxybutyrate and narcolepsy: a double-blind placebo-controlled study. Sleep 1993; 16:216–220.LammersGJ]]ArendsJ]]DeclerckAC&etal;Gammahydroxybutyrate and narcolepsy: a double-blind placebo-controlled study.Sleep199316216-220

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