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Cataplexy and Its Mimics: Clinical Recognition and Management

  • Sleep Disorders (A Iranzo, Section Editor)
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This review describes the diagnosis and management of cataplexy: attacks of bilateral loss of muscle tone, triggered by emotions and with preserved consciousness. Although cataplexy is rare, its recognition is important as in most cases, it leads to a diagnosis of narcolepsy, a disorder that still takes a median of 9 years to be diagnosed. The expression of cataplexy varies widely, from partial episodes affecting only the neck muscles to generalized attacks leading to falls. Moreover, childhood cataplexy differs from the presentation in adults, with a prominent facial involvement, already evident without clear emotional triggers (‘cataplectic facies’) and ‘active’ motor phenomena especially of the tongue and perioral muscles. Next to narcolepsy, cataplexy can sometimes be caused by other diseases, such as Niemann-Pick type C, Prader Willi Syndrome, or lesions in the hypothalamic or pontomedullary region. Cataplexy mimics include syncope, epilepsy, hyperekplexia, drop attacks and pseudocataplexy. They can be differentiated from cataplexy using thorough history taking, supplemented with (home)video recordings whenever possible. Childhood narcolepsy, with its profound facial hypotonia, can be confused with neuromuscular disorders, and the active motor phenomenona resemble those found in childhood movement disorders such as Sydenham’s chorea. Currently, the diagnosis of cataplexy is made almost solely on clinical grounds, based on history taking and (home) videos. Cataplexy shows remarkable differences in childhood compared to adults, with profound facial hypotonia and complex active motor phenomena. Over time, these severe symptoms evolve to the milder adult phenotype, and this pattern is crucial to recognize when assessing the outcome of uncontrolled case series with potential treatments such as immunomodulation. Symptomatic treatment is possible with antidepressants and sodium oxybate. Importantly, management also needs to involve sleep hygiene advice, safety measures whenever applicable and guidance with regard to the social sequelae of cataplexy.

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References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: •    Of importance ••   Of major importance

  1. Dauvilliers Y, Arnulf I, Mignot E. Narcolepsy with cataplexy. Lancet. 2007;369:499–511.

    Article  PubMed  Google Scholar 

  2. Taddei RN, Werth E, Poryazova R, Baumann CR, Valko PO. Diagnostic delay in narcolepsy type 1: combining the patients' and the doctors' perspectives. J Sleep Res. 2016;25:709–15.

    Article  PubMed  Google Scholar 

  3. Scammell TE. Narcolepsy. N Engl J Med. 2015;373:2654–62.

    Article  CAS  PubMed  Google Scholar 

  4. Dauvilliers Y, Siegel JM, Lopez R, Torontali ZA, Peever JH. Cataplexy—clinical aspects, pathophysiology and management strategy. Nat Rev Neurol. 2014;10:386–95.

    Article  CAS  PubMed  Google Scholar 

  5. Honda Y. Clinical features of narcolepsy. In: Honda Y, Juiji T, editors. HLA in Narcolepsy. 1988; 24–57.

  6. Overeem S, Lammers GJ, Van Dijk JG. Weak with laughter. Lancet. 1999;354:838.

    Article  CAS  PubMed  Google Scholar 

  7. Lammers GJ, Overeem S, Tijssen MA, Van Dijk JG. Effects of startle and laughter in cataplectic subjects: a neurophysiological study between attacks. Clin Neurophysiol. 2000;111:1276–81.

    Article  CAS  PubMed  Google Scholar 

  8. Mattarozzi K, Bellucci C, Campi C, Cipolli C, Ferri R, Franceschini C, et al. Clinical, behavioural and polysomnographic correlates of cataplexy in patients with narcolepsy/cataplexy. Sleep Med. 2008;9:425–33.

    Article  PubMed  Google Scholar 

  9. •• Overeem S, van Nues SJ, van der Zande WL, Donjacour CE, van Mierlo P, Lammers GJ. The clinical features of cataplexy: a questionnaire study in narcolepsy patients with and without hypocretin-1 deficiency. Sleep Med. 2011;12:12–8. Overview of the clinical features of cataplexy in adults.

    Article  PubMed  Google Scholar 

  10. Vetrugno R, D'Angelo R, Moghadam KK, Vandi S, Franceschini C, Mignot E, et al. Behavioural and neurophysiological correlates of human cataplexy: a video-polygraphic study. Clin Neurophysiol. 2010;121:153–62.

    Article  PubMed  Google Scholar 

  11. Guilleminault C, Wilson RA, Dement WC. A study on cataplexy. Arch Neurol. 1974;31:255–61.

    Article  CAS  PubMed  Google Scholar 

  12. American Academy of Sleep Medicine. International classification of sleep disorders—third edition (ICSD-3). AASM Resource Library, 2014.

  13. Matos N, Gaig C, Santamaria J, Iranzo A. Tricks to rapidly terminate episodes of cataplexy in narcolepsy. Sleep Med. 2016;20:129–30.

    Article  PubMed  Google Scholar 

  14. Panda S. Status cataplecticus as initial presentation of late onset narcolepsy. J Clin Sleep Med. 2014;10:207–9.

    PubMed  PubMed Central  Google Scholar 

  15. Simon DK, Nishino S, Scammell TE. Mistaken diagnosis of psychogenic gait disorder in a man with status cataplecticus (“limp man syndrome”). Mov Disord. 2004;19:838–40.

    Article  PubMed  Google Scholar 

  16. Calabrò RS, Savica R, Laganà A, Magaudda A, Imbesi D, Gallitto G, et al. Status cataplecticus misdiagnosed as recurrent syncope. Neurol Sci. 2007;28:336–8.

    Article  PubMed  Google Scholar 

  17. Wang J, Greenberg H. Status cataplecticus precipitated by abrupt withdrawal of venlafaxine. J Clin Sleep Med. 2013;9:715–6.

    PubMed  PubMed Central  Google Scholar 

  18. Chabas D, Habert M-O, Maksud P, Tourbah A, Minz M, Willer J-C, et al. Functional imaging of cataplexy during status cataplecticus. Sleep. 2007;30:153–6.

    Article  PubMed  Google Scholar 

  19. Krahn LE, Lymp JF, Moore WR, Slocumb N, Silber MH. Characterizing the emotions that trigger cataplexy. J Neuropsychiatry Clin Neurosci. 2005;17:45–50.

    Article  PubMed  Google Scholar 

  20. Serra L, Montagna P, Mignot E, Lugaresi E, Plazzi G. Cataplexy features in childhood narcolepsy. Mov Disord. 2008;23:858–65.

    Article  PubMed  Google Scholar 

  21. •• Plazzi G, Pizza F, Palaia V, Franceschini C, Poli F, Moghadam KK, et al. Complex movement disorders at disease onset in childhood narcolepsy with cataplexy. Brain Oxford University Press. 2011;134:3480–92. This article describes the typical movement disorders seen in childhood cataplexy.

    Google Scholar 

  22. Pizza F, Franceschini C, Peltola H, Vandi S, Finotti E, Ingravallo F, et al. Clinical and polysomnographic course of childhood narcolepsy with cataplexy. Brain. 2013;136:3787–95.

    Article  PubMed  PubMed Central  Google Scholar 

  23. Luca G, Haba Rubio J, Dauvilliers Y, Lammers GJ, Overeem S, Donjacour CE, et al. Clinical, polysomnographic and genome-wide association analyses of narcolepsy with cataplexy: a European Narcolepsy Network study. J Sleep Res. 2013;22:482–95.

    Article  PubMed  Google Scholar 

  24. Thorpy MJ, Krieger AC. Delayed diagnosis of narcolepsy: characterization and impact. Sleep Med. 2014;15:502–7.

    Article  PubMed  Google Scholar 

  25. Partinen M, Saarenpää-Heikkilä O, Ilveskoski I, Hublin C, Linna M, Olsén P, et al. Increased incidence and clinical picture of childhood narcolepsy following the 2009 H1N1 pandemic vaccination campaign in Finland. PLoS One. 2012;7:e33723.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Heier MS, Gautvik KM, Wannag E, Bronder KH, Midtlyng E, Kamaleri Y, et al. Incidence of narcolepsy in Norwegian children and adolescents after vaccination against H1N1 influenza A. Sleep Med. 2013;14:867–71.

    Article  CAS  PubMed  Google Scholar 

  27. Pizza F, Peltola H, Sarkanen T, Moghadam KK, Plazzi G, Partinen M. Childhood narcolepsy with cataplexy: comparison between post-H1N1 vaccination and sporadic cases. Sleep Med. 2014;15:262–5.

    Article  PubMed  Google Scholar 

  28. Lecendreux M, Maret S, Bassetti C, Mouren M-C, Tafti M. Clinical efficacy of high-dose intravenous immunoglobulins near the onset of narcolepsy in a 10-year-old boy. J Sleep Res. 2003;12:347–8.

    Article  PubMed  Google Scholar 

  29. Lecendreux M, Berthier J, Corny J, Bourdon O, Dossier C, Delclaux C. Intravenous immunoglobulin therapy in pediatric narcolepsy: a nonrandomized, open-label, controlled, longitudinal observational study. J Clin Sleep Med. 2017;13:441–53.

    Article  PubMed  Google Scholar 

  30. Valko PO, Khatami R, Baumann CR, Bassetti CL. No persistent effect of intravenous immunoglobulins in patients with narcolepsy with cataplexy. J Neurol. 2008;255:1900–3.

    Article  PubMed  Google Scholar 

  31. Nishino S, Mignot E. Pharmacological aspects of human and canine narcolepsy. Prog Neurobiol. 1997;52:27–78.

    Article  CAS  PubMed  Google Scholar 

  32. Chemelli RM, Willie JT, Sinton CM, Elmquist JK, Scammell T, Lee C, et al. Narcolepsy in orexin knockout mice: molecular genetics of sleep regulation. Cell. 1999;98:437–51.

    Article  CAS  PubMed  Google Scholar 

  33. Tabuchi S, Tsunematsu T, Black SW, Tominaga M, Maruyama M, Takagi K, et al. Conditional ablation of orexin/hypocretin neurons: a new mouse model for the study of narcolepsy and orexin system function. J Neurosci. 2014;34:6495–509.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  34. Scammell TE, Willie JT, Guilleminault C, Siegel JM. A con-sensus definition of cataplexy in mouse models of narcolepsy. Sleep. 2009;32:111–6.

    Article  PubMed  PubMed Central  Google Scholar 

  35. • Oishi Y, Williams RH, Agostinelli L, Arrigoni E, Fuller PM, Mochizuki T, et al. Role of the medial prefrontal cortex in cataplexy. J Neurosci. 2013;33:9743–51. The medial prefrontal cortex plays important roles in mediating behavioral responses to emotional stimuli, and inactivation of this region reduces cataplexy.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  36. España RA, McCormack SL, Mochizuki T, Scammell TE. Running promotes wakefulness and increases cataplexy in orexin knockout mice. Sleep. 2007;30:1417–25.

    Article  PubMed  PubMed Central  Google Scholar 

  37. Willie JT, Chemelli RM, Sinton CM, Tokita S, Williams SC, Kisanuki YY, et al. Distinct narcolepsy syndromes in orexin receptor-2 and orexin null mice. Neuron. 2003;38:715–30.

    Article  CAS  PubMed  Google Scholar 

  38. Schmidt C, Leibiger J, Fendt M. The norepinephrine reuptake inhibitor reboxetine is more potent in treating murine narcoleptic episodes than the serotonin reuptake inhibitor escitalopram. Behav Brain Res. 2016;308:205–10.

    Article  CAS  PubMed  Google Scholar 

  39. Burgess CR, Peever JH. A noradrenergic mechanism functions to couple motor behavior with arousal state. Curr Biol. 2013;23:1719–25.

    Article  CAS  PubMed  Google Scholar 

  40. •• Burgess CR, Scammell TE. Narcolepsy: neural mechanisms of sleepiness and cataplexy. J Neurosci. 2012;32:12305–11. Overview of the neural mechanisms of narcolepsy and cataplexy.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  41. • Hasegawa E, Yanagisawa M, Sakurai T, Mieda M. Orexin neurons suppress narcolepsy via 2 distinct efferent pathways. J Clin Invest. 2014;124:604–16. Mice lacking orexin receptors have cataplexy, but restoration of OX2 receptors to serotonergic neurons of the dorsal raphe nucleus strongly suppresses cataplexy.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  42. Burgess CR, Oishi Y, Mochizuki T, Peever JH, Scammell TE. Amygdala lesions reduce cataplexy in orexin knock-out mice. J Neurosci. 2013;33:9734–42.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  43. Gulyani S, Wu M-F, Nienhuis R, John J, Siegel JM. Cataplexy-related neurons in the amygdala of the narcoleptic dog. Neuroscience. 2002;112:355–65.

    Article  CAS  PubMed  Google Scholar 

  44. • Mahoney C, Agostinelli L, Brooks J, Lowell B, Scammell T. GABAergic neurons of the central amygdala promote cataplexy. J Neurosci. 2017; doi:10.1523/JNEUROSCI.4065-15.2017. GABAergic neurons of the central nucleus of the amygdala mediate behavioral responses to emotional stimuli and innervate brainstem regions that regulate atonia. This paper shows that activation of these neurons increases cataplexy, whereas inactivation reduces cataplexy.

    Google Scholar 

  45. Meletti S, Vaudano AE, Pizza F, Ruggieri A, Vandi S, Teggi A, et al. The brain correlates of laugh and cataplexy in childhood narcolepsy. J Neurosci. 2015;35:11583–94.

    Article  CAS  PubMed  Google Scholar 

  46. Abela L, Plecko B, Palla A, Burda P, Nuoffer J-M, Ballhausen D, et al. Early co-occurrence of a neurologic-psychiatric disease pattern in Niemann-Pick type C disease: a retrospective Swiss cohort study. Orphanet J Rare Dis. 2014;9:176.

    Article  PubMed  PubMed Central  Google Scholar 

  47. Nevsimalova S, Malinova V. Cataplexy and sleep disorders in Niemann-Pick type C disease. Curr Neurol. 2015;15:522.

    Article  CAS  Google Scholar 

  48. Parkes JD. Genetic factors in human sleep disorders with special reference to Norrie disease, Prader–Willi syndrome and Moebius syndrome. J Sleep Res. 1999;8:14–22.

    Article  PubMed  Google Scholar 

  49. Granild Bie Mertz L, Christensen R, Vogel I, Hertz JM, Østergaard JR. Epilepsy and cataplexy in Angelman syndrome. Genotype-phenotype correlations. Res Dev Disabil. 2016;56:177–\.

    Article  PubMed  Google Scholar 

  50. Winkelmann J, Lin L, Schormair B, Kornum BR, Faraco J, Plazzi G, et al. Mutations in DNMT1 cause autosomal dominant cerebellar ataxia, deafness and narcolepsy. Hum Mol Genet. 2012;21:2205–10.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  51. Fronczek R, Lammers GJ, Balesar R, Unmehopa UA, Swaab DF. The number of hypothalamic hypocretin (orexin) neurons is not affected in Prader-Willi syndrome. J Clin Endocrinol Metab. 2005;90:5466–70.

    Article  CAS  PubMed  Google Scholar 

  52. Plazzi G, Montagna P, Provini F, Bizzi A, Cohen M, Lugaresi E. Pontine lesions in idiopathic narcolepsy. Neurology. 1996;46:1250–4.

    Article  CAS  PubMed  Google Scholar 

  53. Sinsioco C, Silver K, Forrest KM, Gray J, Nechay A, Sheldon S, et al. Narcolepsy with cataplexy as presenting symptom of occult neuroblastoma. Pediatr Neurol. 2013;49:64–7.

    Article  PubMed  Google Scholar 

  54. Landolfi JC, Nadkarni M. Paraneoplastic limbic encephalitis and possible narcolepsy in a patient with testicular cancer: case study. Neuro-Oncology. 2003;5:214–6.

    Article  PubMed  PubMed Central  Google Scholar 

  55. Lagrange AH, Blaivas M, Gomez-Hassan D, Malow BA. Rasmussen's syndrome and new-onset narcolepsy, cataplexy, and epilepsy in an adult. Epilepsy Behav. 2003;4:788–92.

    Article  CAS  PubMed  Google Scholar 

  56. Dhondt K, Verloo P, Verhelst H, Van Coster R, Overeem S. Hypocretin-1 deficiency in a girl with ROHHAD syndrome. Pediatrics. 2013;132:e788–92.

    Article  PubMed  Google Scholar 

  57. Stahl SM, Layzer RB, Aminoff MJ, Townsend JJ, Feldon S. Continuous cataplexy in a patient with a midbrain tumor: the limp man syndrome. Neurology. 1980;30:1115–8.

    Article  CAS  PubMed  Google Scholar 

  58. D’Cruz OF, Vaughn BV, Gold SH, Greenwood RS. Symptomatic cataplexy in pontomedullary lesions. Neurology. 1994;44:2189–91.

    Article  PubMed  Google Scholar 

  59. Rivera VM, Meyer JS, Hata T, Ishikawa Y, Imai A. Narcolepsy following cerebral hypoxic ischemia. Ann Neurol. 1986;19:505–8.

    Article  CAS  PubMed  Google Scholar 

  60. Krahn LE, Hansen MR, Shepard JW. Pseudocataplexy. Psychosomatics. 2001;42:356–8.

    Article  CAS  PubMed  Google Scholar 

  61. Shankar R, Jalihal V, Walker M, Zeman A. Pseudocataplexy and transient functional paralysis: a spectrum of psychogenic motor disorder. J Neuropsychiatry Clin Neurosci. 2010;22:445–50.

    Article  PubMed  Google Scholar 

  62. Plazzi G, Khatami R, Serra L, Pizza F, Bassetti CL. Pseudocataplexy in narcolepsy with cataplexy. Sleep Med. 2010;11:591–4.

    Article  PubMed  Google Scholar 

  63. Pizza F, Vandi S, Poli F, Moghadam KK, Franceschini C, Bellucci C, et al. Narcolepsy with cataplexy mimicry: the strange case of two sisters. J Clin Sleep Med. 2013;9:611–2.

    PubMed  PubMed Central  Google Scholar 

  64. Pizza F, Antelmi E, Vandi S, Meletti S, Erro R, Barone P, et al. The distinguishing motor features of cataplexy: a study from video recorded attacks. J Sleep Res. 2016;25(suppl 1):277.

    Google Scholar 

  65. Thiagarajah PH, Finkielstein D, Granato JE. Sitcom syncope: a case series and literature review of Gelastic (laughter-induced) syncope. Postgraduate Medicine Taylor & Francis. 2015;122:137–44.

    Article  Google Scholar 

  66. Totah AR, Benbadis SR. Gelastic syncope mistaken for cataplexy. Sleep Med. 2002;3:77–8.

    Article  PubMed  Google Scholar 

  67. Schwartz PJ, Priori SG, Spazzolini C, Moss AJ, Vincent GM, Napolitano C, et al. Genotype-phenotype correlation in the long-QT syndrome. Circulation. 2001;103:89–95.

    Article  CAS  PubMed  Google Scholar 

  68. Lane RD, Reis HT, Peterson DR, Zareba W, Moss AJ. Happiness and stress alter susceptibility to cardiac events in long QT syndrome. Ann Noninvasive Electrocardiol. 2009;14:193–200.

    Article  PubMed  PubMed Central  Google Scholar 

  69. Kadari R, Sarche MA, Krantz MJ. Fatal laughter. Ann Intern Med. 2012;157:756.

    Article  PubMed  Google Scholar 

  70. Britton JW. Syncope and seizures-differential diagnosis and evaluation. Clin Auton Res. 2004;14:148–59.

    Article  PubMed  Google Scholar 

  71. Baiardi S, Vandi S, Pizza F, Alvisi L, Toscani L, Zambrelli E, et al. Narcolepsy type 1 and idiopathic generalized epilepsy: diagnostic and therapeutic challenges in dual cases. J Clin Sleep Med. 2015;11:1257–62.

    Article  PubMed  PubMed Central  Google Scholar 

  72. Plazzi G, Tinuper P. Praxis-induced seizures misdiagnosed as cataplexy: a case report. Mov Disord. 2008;23:2105–7.

    PubMed  Google Scholar 

  73. Nordli DR. Atonic Seizures. In: Panayiotopoulos CP, editor. Atlas of epilepsies. London: Springer London; 2010. p. 395–8.

    Chapter  Google Scholar 

  74. Macleod S, Ferrie C, Zuberi SM. Symptoms of narcolepsy in children misinterpreted as epilepsy. Epileptic Disord. 2005;7:13–7.

    PubMed  Google Scholar 

  75. Zifkin BG. Reflex seizures. Atlas of epilepsies. London: Springer; 2010. p. 483–9.

    Book  Google Scholar 

  76. Woods RJ, Gruenthal M. Cognition-induced epilepsy associated with specific emotional precipitants. Epilepsy Behav. 2006;9:360–2.

    Article  PubMed  Google Scholar 

  77. Glass HC, Prieur B, Molnar C, Hamiwka L, Wirrell E. Micturition and emotion-induced reflex epilepsy: case report and review of the literature. Epilepsia. 2006;47:2180–2.

    Article  PubMed  Google Scholar 

  78. Martinez O, Reisin R, Andermann F, Zifkin BG, Sevlever G. Evidence for reflex activation of experiential complex partial seizures. Neurology. 2001;56:121–3.

    Article  CAS  PubMed  Google Scholar 

  79. Brownstein CA, Beggs AH, Rodan L, Shi J, Towne MC, Pelletier R, et al. Clinical heterogeneity associated with KCNA1 mutations include cataplexy and nonataxic presentations. Neurogenetics. 2016;17:11–6.

    Article  CAS  PubMed  Google Scholar 

  80. Sheldon JH. On the natural history of falls in old age. Br Med J. 1960;2:1685–90.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  81. Tumarkin A. The otholithic catastrophe. Br Med J. 1936;2:175–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  82. Crow YJ, Zuberi SM, McWilliam R, Tolmie JL, Hollman A, Pohl K, et al. “cataplexy” and muscle ultrasound abnormalities in Coffin-Lowry syndrome. J Med Genet. 1998;35:94–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  83. Bakker MJ, van Dijk JG, van den Maagdenberg AM, Tijssen MA. Startle syndromes. Lancet Neurol. 2006;5:513–24.

    Article  PubMed  Google Scholar 

  84. Stephenson JBP, Hoffman MC, Russell AJC, Falconer J, Beach RC, Tolmie JL, et al. The movement disorders of Coffin-Lowry syndrome. Brain and Development. 2005;27:108–13.

    Article  PubMed  Google Scholar 

  85. Weber F, Jurkat-Rott K, Lehmann-Horn F. Hyperkalemic periodic paralysis. In: Pagon RA, Adam MP, Ardinger HH, et al. editors. SourceGeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2017. 2003 Jul 18 [updated 2016 Jan 28].

  86. Stedwell RE, Allen KM, Binder LS. Hypokalemic paralyses: a review of the etiologies, pathophysiology, presentation, and therapy. Am J Emerg Med. 1992;10:143–8.

    Article  CAS  PubMed  Google Scholar 

  87. Jandhyala SN, Madireddi J, Belle J, Rau NR, Shetty R. Hypokalaemic periodic paralysis—a prospective study of the underlying etiologies. J Clin Diagn Res. 2015;9:OC17–9.

    CAS  PubMed  PubMed Central  Google Scholar 

  88. Dhondt K, Verhelst H, Pevernagie D, Slap F, Van Coster R. Childhood narcolepsy with partial facial cataplexy: a diagnostic dilemma. Sleep Med. 2009;10:797–8.

    Article  PubMed  Google Scholar 

  89. Martinez-Rodriguez JE, Lin L, Iranzo A. Decreased hypocretin-1 (orexin-A) levels in the cerebrospinal fluid of patients with myotonic dystrophy and excessive daytime sleepiness. Sleep. 2003;26:287–90.

    Article  PubMed  Google Scholar 

  90. Smith SV, Lee AG. Update on ocular myasthenia gravis. Neurol Clin. 2017;35:115–23.

    Article  PubMed  Google Scholar 

  91. Akimoto H, Honda Y, Takahashi Y. Pharmacotherapy in narcolepsy. Dis Nerv Syst. 1960;21:704–6.

    CAS  PubMed  Google Scholar 

  92. Schachter M, Parkes JD. Fluvoxamine and clomipramine in the treatment of cataplexy. J Neurol Neurosurg Psychiatry. 1980;43:171–4.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  93. Houghton WC, Scammell TE, Thorpy M. Pharmacotherapy for cataplexy. Sleep Med Rev. 2004;8:355–66.

    Article  PubMed  Google Scholar 

  94. Mignot EJM. A practical guide to the therapy of narcolepsy and hypersomnia syndromes. Neurotherapeutics. 2012;9:739–52.

    Article  PubMed  PubMed Central  Google Scholar 

  95. Barateau L, Lopez R, Dauvilliers Y. Management of narcolepsy. Curr Treat Options Neurol. 2016;18:43.

    Article  PubMed  Google Scholar 

  96. Izzi F, Placidi F, Marciani MG, Zannino S, Torelli F, Corte F, et al. Effective treatment of narcolepsy-cataplexy with duloxetine: a report of three cases. Sleep Med. 2009;10:153–4.

    Article  CAS  PubMed  Google Scholar 

  97. Alshaikh MK, Tricco AC, Tashkandi M, Mamdani M, Straus SE, BaHammam AS. Sodium Oxybate for narcolepsy with cataplexy: systematic review and meta-analysis. J Clin Sleep. 2012;8:451–8.

    Google Scholar 

  98. Boscolo-Berto R, Viel G, Montagnese S, Raduazzo DI, Ferrara SD, Dauvilliers Y. Narcolepsy and effectiveness of gamma-hydroxybutyrate (GHB): a systematic review and meta-analysis of randomized controlled trials. Sleep Med Rev. 2012;16:431–43.

    Article  PubMed  Google Scholar 

  99. Husain AM, Ristanovic RK, Bogan RK. Weight loss in narcolepsy patients treated with sodium oxybate. Sleep Med. 2009;10:661–3.

    Article  PubMed  Google Scholar 

  100. Poli F, Plazzi G, Di Dalmazi G, Ribichini D, Vicennati V, Pizza F, et al. Body mass index-independent metabolic alterations in narcolepsy with cataplexy. Sleep. 2009;32:1491–7.

    Article  PubMed  PubMed Central  Google Scholar 

  101. Poli F, Pizza F, Mignot E, Ferri R, Pagotto U, Taheri S, et al. High prevalence of precocious puberty and obesity in childhood narcolepsy with cataplexy. Sleep. 2013;36:175–81.

    Article  PubMed  PubMed Central  Google Scholar 

  102. Bogan RK, Roth T, Schwartz J, Miloslavsky M. Time to response with sodium oxybate for the treatment of excessive daytime sleepiness and cataplexy in patients with narcolepsy. J Clin Sleep Med. 2015;11:427–32.

    PubMed  PubMed Central  Google Scholar 

  103. Mamelak M, Swick T, Emsellem H, Montplaisir J, Lai C, Black J. A 12-week open-label, multicenter study evaluating the safety and patient-reported efficacy of sodium oxybate in patients with narcolepsy and cataplexy. Sleep Med. 2015;16:52–8.

    Article  PubMed  Google Scholar 

  104. Shelton J, Nishino S, Vaught J, Dement WC, Mignot E. Comparative effects of modafinil and amphetamine on daytime sleepiness and cataplexy of narcoleptic dogs. Sleep. 1995;18:817–26.

    CAS  PubMed  Google Scholar 

  105. 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. The Lancet Neurology. 2017;16:200–7.

    Article  CAS  PubMed  Google Scholar 

  106. A multicenter study of the efficacy and safety of xyrem with an open- label pharmacokinetic evaluation and safety extension in pediatric subjects with narcolepsy with cataplexy. ClinicalTrials.gov Identifier: NCT02221869.

  107. Lecendreux M, Poli F, Oudiette D, Benazzouz F, Donjacour CEHM, Franceschini C, et al. Tolerance and efficacy of sodium oxybate in childhood narcolepsy with cataplexy: a retrospective study. Sleep. 2012;35:709–11.

    Article  PubMed  PubMed Central  Google Scholar 

  108. Aran A, Einen M, Lin L, Plazzi G, Nishino S, Mignot E. Clinical and therapeutic aspects of childhood narcolepsy-cataplexy: a retrospective study of 51 children. Sleep. 2010;33:1457–64.

    Article  PubMed  PubMed Central  Google Scholar 

  109. Ponziani V, Gennari M, Pizza F, Balsamo A, Bernardi F, Plazzi G. Growing up with type 1 narcolepsy: its anthropometric and endocrine features. J Clin Sleep Med. 2016;12:1649–57.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Sleep Medicine Center Kempenhaeghe, P.O. Box 61, , 5590 AB, Heeze, The Netherlands

    Sigrid Pillen MD PhD & Sebastiaan Overeem MD PhD

  2. Department of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, Bologna, Italy

    Fabio Pizza MD PhD

  3. IRCCS Institute of the Neurological SciencesAUSL di Bologna, Bologna, Italy

    Fabio Pizza MD PhD

  4. Department Pediatrics, Division of Child Neurology & Metabolism, Pediatric Sleep Center, Ghent University Hospital, Ghent, Belgium

    Karlien Dhondt MD PhD

  5. Department of Neurology, Beth Israel Deaconess Medical Center, Boston Children’s Hospital, Boston, MA, USA

    Thomas E. Scammell MD PhD

  6. Eindhoven University of Technology, Eindhoven, The Netherlands

    Sebastiaan Overeem MD PhD

Authors
  1. Sigrid Pillen MD PhD
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  2. Fabio Pizza MD PhD
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  3. Karlien Dhondt MD PhD
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  4. Thomas E. Scammell MD PhD
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  5. Sebastiaan Overeem MD PhD
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Corresponding author

Correspondence to Sigrid Pillen MD PhD.

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Conflict of Interest

Fabio Pizza and Karlien Dhondt declare that they have no conflict of interest.

Sigrid Pillen received speaker’s fees from congress organizations (SCEM and JCC, The Netherlands) for postgraduate education on sleep problems in children.

Thomas E. Scammell is supported by research grants from the NIH (HL095491, DE022912) and the Foundation for Prader Willi Research. He has received consulting fees from Reset Therapeutics, Avadel Pharmaceuticals, Marathon Pharmaceuticals, Merck, Jazz Pharmaceuticals, SK Biopharmaceuticals, Purdue Pharma, Ono Pharmaceuticals and Janssen.

Sebastiaan Overeem is supported by a VIDI research grant from the Netherlands Organization for Scientific Research (grant no. 016.116.371), received an unrestricted research grant from UCB Pharma not related to the present work and received conference travel support from UCB Pharma, Novartis and Boehringer Ingelheim.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

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This article is part of the Topical Collection on Sleep Disorders

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Pillen, S., Pizza, F., Dhondt, K. et al. Cataplexy and Its Mimics: Clinical Recognition and Management. Curr Treat Options Neurol 19, 23 (2017). https://doi.org/10.1007/s11940-017-0459-0

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  • DOI: https://doi.org/10.1007/s11940-017-0459-0

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