The implications of gender and age at onset of first symptoms in narcoleptic patients in Germany—results from retrospective evaluation of hospital records

  • Geert Mayer
  • Karl Kesper
  • Helga Peter
  • Thomas Ploch
  • Thomas Leinweber
  • Jörg Hermann Peter
Original Articles

Summary

Objective

The aim is to create a matrix of relevant information about narcolepsy in Germany for a narcolepsy register.

Method

A retrospective study of randomly selected hospital records of 106 narcoleptic patients, all presenting cataplexies and excessive daytime sleepiness plus HLA DR 2 positive typing, was performed.

Results

First symptoms of narcolepsy evolved mainly between the ages 10–20 and 30–45. In patients with early manifestation the latency between onset of excessive daytime sleepiness and cataplexy was much longer than in those with late manifestation (19.4 vs 7.9 years, p<0.001). Since the chance of narcolepsy to be diagnosed increases significantly after the onset of cataplexies the latency between manifestation of first symptoms and diagnosis is much shorter in the group with later manifestation. Women showed a slightly earlier manifestation (22.0 years) than men (23.7 years).

Conclusion

Our findings may implicate the existence of two different types of narcolepsy, depending on the age of manifestation of first symptoms.

Keywords

narcolepsy onset on first symptoms narcolepsy register gender differences 

Die Bedeutung von Geschlecht und Alter beim Auftreten der ersten Symptome von Narkolepsiepatienten in Deutschland—Ergebnisse einer retrospektiven Auswertung von Krankenakten

Zusammenfassung

Einführung

Ziel ist die Erstellung einer Matrix relevanter Daten von Narkolepsiepatienten für ein deutsches Narkolepsieregister.

Methode

In einer retrospektiven Untersuchung zufällig ausgewählter Krankenakten wurden Daten von 106 Patienten mit Kataplexien, Tagesschläfrigkeit und positiver HLA-DR-2 Typisierung erfaßt.

Ergebnisse

Erste Narkolepsiesymptome entwickeln sich meist im Alter zwischen 10–20 und 30–45. Bei Patienten mit früher Symptommanifestation war die Latenz zwischen dem Auftreten von Tagesschläfrigkeit und Kataplexien viel länger als bei Patienten mit einer späten Symptommanifestation (19,4 vs 7,9 Jahre, p<0,001). Da die Diagnose signifikant häufiger gestellt wird, wenn Kataplexien auftauchen, als wenn Tagesschläfrigkeit als einziges Hauptsymptom vorhanden ist, ist die Latenz vom Auftreten erster Symptome bis zur Diagnosestellung bei der Gruppe mit später Erstmanifestation sehr viel kürzer als bei früher Symptommanifestation. Bei Frauen scheinen sich erste Symptome etwas früher zu manifestieren (22,0 Jahre) als bei Männern (23,7 Jahre).

Schlussfolgerung

Unsere Befunde legen die Existenz von zwei unterschiedlichen Formen der Narkolepsie nahe, die altersabhängig sind.

Schlüsselwörter

Narkolepsie Erstmanifestation narkoleptischer Symptome Narkolepsie Register Geschlechtsunterschiede 
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References

  1. [1]
    Aldrich MS: Narcolepsy. Neurology 42 Suppl. 6:34–43, 1992.PubMedGoogle Scholar
  2. [2]
    Anie-Labat S, Gulleminault C, Kraemer HC, Mechan J, Arrigonie J, Mignot E: Validation of a cacaplexy questionnaire in 983 sleep-disorders patients. Sleep, 22 (1): 77–87, 1999.Google Scholar
  3. [3]
    Bassetti C, Aldrich MS, Quint DJ: MRI findings in narcolepsy, Sleep, 20 (8): 630–631, 1997.PubMedGoogle Scholar
  4. [4]
    Billiard M, Besset A, Cadilhac J: The clinical and polygraphic development of narcolepsy. In: Guilleminault C, Lugaresi E: Sleep/Wake Disorders: Natural history, epidemiology and long-term evolution, Raven Press, New York: pp 187–199, 1983.Google Scholar
  5. [5]
    Broughton R, Ghanem Q, Hishikawa Y, Sugita Y, Nevsimalova S: The socio-economic and related life-effects in 180 patients with narcolepsy from North America, Asia and Europe compared to matched controls. In: Karacan I: Psychophysiological aspects of sleep. Park Ridge, New Jersey, USA, pp 96–105, 1981.Google Scholar
  6. [6]
    Broughton R, Ghanem Q, Hishikawa Y, Sugita Y, Névsimalová S., Roth B: Life effects of narcolepsy: Relationships to geographic origin (North American, Asian or European) and other patient and illness variables. Can J Neurol Sci 10: 100–104, 1983.PubMedGoogle Scholar
  7. [7]
    Chemelli RM, Willie JT, Sinton CM, Elmquist JK, Scammel T, Lee C, Richardson JA, Williams SC, Xiong Y, Kisanuki Y, Fitch TE, Nakazato M, Hammer RE, Saper CB, Yanagisawa M: Narcolepsy in orexin knockout mice: molecular genetics of sleep regulation. Cell 98: 365–376, 1999.CrossRefGoogle Scholar
  8. [8]
    Dauvilliers Y, Molinari N, Espa F, Carlander B, Lesperance P, Montplaisir J, Daures JP, Billiard M: Delay of diagnosis of narcolepsy in a european and in a north american population. J Sleep Res 7 (suppl2): 56, 1998.Google Scholar
  9. [9]
    D'Cruz O, Neill F, Vaughn BV, Gold SH, Greewood RS: Symptomatic cataplexy in pontomedullary lesions. Neurology 44: 2189–2191, 1994.PubMedGoogle Scholar
  10. [10]
    Dement WC, Zarcone V, Varner V, Hoddes E, Nassau S, Jacobs B, Brown J, McDonald A, Hora K, Glass R, Gonzales P, Friedman E, Phillips R: The prevalence of narcolepsy. Sleep Res 1; 148, 1972.Google Scholar
  11. [11]
    Ditta SC, George CFP, Singh SM: HLA-D-region genomic DNA restriction fragments in DRw15(DR2) familial narcolepsy. Sleep 15 (1): 48–57, 1992.PubMedGoogle Scholar
  12. [12]
    Godbout R, Montplaisir J: All-day performance variations in normal and narcoleptic subjects. Sleep 9 (1): 200–204, 1986.PubMedGoogle Scholar
  13. [13]
    Goswami M, Pollak CP, Cohen FL, Thorpy MJ, Kavey NB: Psychosocial Aspects of Narcolepsy. New York, The Haworth Press Inc., New York, 1992.Google Scholar
  14. [14]
    Guilleminault C, Dement WC, Passouant P: Narcolepsy (Advances in Sleep Research, vol 3). Spectrum Publication, New York, 1976.Google Scholar
  15. [15]
    Guilleminault C, Grumet C: HLA-DR2 and narcolepsy: Not all narcoleptic-cataplectic patients are DR2, Hum Immunol 17: 1–2, 1986.PubMedCrossRefGoogle Scholar
  16. [16]
    Guilleminault C, Pelayo R: Narcolepsy in prepubertal children. Ann Neurol 43: 135–142, 1998.PubMedCrossRefGoogle Scholar
  17. [17]
    Honda Y, Asaka A, Tanimura M, Furusho T: A genetic study of narcolepsy and excessive daytime sleepiness in 308 families with a narcolepsy or hypersomnia proband. In: Guilleminault C, Lugaresi E: Sleep/wake disorders: natural history, epidemiology and long-term evolution, Raven Press, New York, pp 187–199, 1983.Google Scholar
  18. [18]
    Honda Y: Clinical features of narcolepsy, In HLA in narcolepsy. In: Honda Y, Juji T, Springer Verlag, Berlin, pp 24–57 1988.Google Scholar
  19. [19]
    Hood B, Bruck D: Sleepiness and performance in narcolepsy. J Sleep Res 5: 128–134, 1996.PubMedCrossRefGoogle Scholar
  20. [20]
    Hublin C, Partinen M, Kaprio J, Koskenvuao M, Guilleminault C: Epidemiology of narcolepsy, Sleep 17: 7–12, 1994.Google Scholar
  21. [21]
    Hungs M, Mignot E: Hypocretin/orexin, sleep and narcolepsy. Bio Essays 23: 397–408, 2001.Google Scholar
  22. [22]
    Mayer G, Kotterba S: Diagnose und Therapie der Narkolepsie. Dt Ärztebl 98: A249–245, 2001.Google Scholar
  23. [23]
    Meier-Ewert K, Mueller-Eckhardt G, Schendel DJ: Narcolepsy and HLA in the Federal Republic of Germany: Population and family data. In: Honda Y, Juji T. HLA in narcolepsy. Springer Verlag, Berlin, pp 114–20, 1998.Google Scholar
  24. [24]
    Mignot E, Hayduk R, Black J, Grumet FC, Guilleminault C: HLADQB1*0602 is associated with cataplexy in 509 narcoleptic patients. Sleep 20: 1012–1020, 1997.PubMedGoogle Scholar
  25. [25]
    Mignot E, Lin X, Arrigoni J, Macaubas C, Olive F, Hallmayer J, Underhill P, Guilleminault C, Dement WC, Grumet FC: DQB1*0602 and DQA1*0102 (DQ1) are better markers than DR2 for narcolepsy in caucasian and black americans. Sleep 17 (8): 60–67, 1994.Google Scholar
  26. [26]
    Nishino S, Ripley B, Overeem S, Lammers GJ, Mignot E: Hypocretin (orexin) deficiency in human narcolopsy. Lancet 355: 39–40, 2000.PubMedCrossRefGoogle Scholar
  27. [27]
    Rogers AE: Memory deterioration versus attentional deficits in patients with narcolepsy. Sleep Research 15: 418, 1984.Google Scholar
  28. [28]
    Rye DB, Dihenia B, Weissman JD, Epstein CM, Bliwise DL: Presentation of narcolepsy after 40. Neurology 50: 459–465, 1998.PubMedGoogle Scholar
  29. [29]
    Winkelmann J, Wetter TC, Collado-Seidel U, Gasser T, Dichgans M, Yassouridis A, Trenkwalder C: Clinical characteristics and frequency of the hereditary restless legs syndrome in a population of 300 patients. Sleep 23: 597–602, 2000.PubMedGoogle Scholar
  30. [30]
    Yoss RE, Daly D: Criteria for the diagnosis of the narcoleptic syndrome. Proc Staff Meetings Mayo Clin 32: 320–328, 1957.Google Scholar

Copyright information

© Blackwell Verlag 2002

Authors and Affiliations

  • Geert Mayer
    • 1
  • Karl Kesper
    • 2
  • Helga Peter
    • 1
  • Thomas Ploch
    • 2
  • Thomas Leinweber
    • 2
  • Jörg Hermann Peter
    • 2
  1. 1.Hephata KlinikSchwalmstadt-TreysaGermany
  2. 2.Schlafmedizinisches Labor, Philipps Universität MarburgSchwalmstadt-TreysaGermany

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