Skip to main content
SpringerLink
Log in

Das primäre Hypoventilationssyndrom

Primary hypoventilation syndrome (ondine's curse syndrome)

  • Originalien
  • Published:
Klinische Wochenschrift Aims and scope Submit manuscript

Summary

Primary hypoventilation syndrome is an extremely rare type of impairment of respiratory control. Termed “Ondine's Curse Syndrome”, it may be opposed to the more frequent “Pickwick Syndrome” which is found only in very obese patients. Giving a case report as an example, pathogenesis and clinical symptomatology of primary hypoventilation syndrome are discussed in detail. It is demonstrated that this syndrome may serve in an ideal way as a natural model for studying the peripheral chemoreceptor function, the pathogenesis of pulmonary hypertension and the therapeutic mechanism of respiratory stimulants.

Zusammenfassung

Das primäre Hypoventilationssyndrom ist eine bei Normalgewichtigen sehr selten beobachteteAtemregulationsstörung. Man kann es unter der Bezeichnung„Ondine's Curse“-Syndrome dem funktionell verwandten, aber ausschließlich bei extrem Adipösen vorkommendenPickwick-Syndrom zur Seite stellen. Anhand eines eigenen Falles mit primärem Hypoventilationssyndrom werden Pathogenese und klinische Symptomatik des bis heute ätiologisch unklaren Krankheitsbildes dargestellt. Die an diesem Patienten durchgeführten Untersuchungen stellen das primäre Hypoventilationssyndrom als einsellenes Naturmodell vor, an dem sich die Funktionsweise der peripheren Chemoreceptoren, die Pathogenese des pulmonalen Hochdrucks und der Wirkungsmechanismus von Atemanaleptika in idealer Weise am Menschen studieren läßt.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

Literatur

  1. Atlan, G., Hatzfeld, C., Brille, D.: Hypoventilation alvéolaire primitive ou d'origine centrale chez le sujet non obése. Presse méd.75, 2650 (1967)

    Google Scholar 

  2. Bartels, H., Witzleb, E.: Der Einfluß des arteriellen CO2-Druckes auf die chemorezeptorischen Aktionspotentiale im Carotissinusnerven. Pflügers Arch. ges. Physiol.262, 466 (1956)

    Google Scholar 

  3. Biscoe, T. J., Purves, M. J., Sampson, S. R.: The frequency of nerve impulses in single carotid body chemoreceptor afferent fibers recorded in vivo with intact circulation. J. Physiol. (Lond.)208, 121 (1970)

    PubMed  Google Scholar 

  4. Burwell, C. S., Robin, E. D., Whaley, R. D., Bickelmann, A. G.: Extreme obesity associated with alveolar hypoventilation—a Pickwickian syndrome. Amer. J. Med.21, 811 (1956)

    PubMed  Google Scholar 

  5. Chiesa, A., Balbi, L., Dolcetti, A., Motta, P. E.: Un caso di ipoventilazione alveolare primitiva in soggetto non obeso. Minerva med.61, 1755 (1970)

    PubMed  Google Scholar 

  6. Coccagna, G., Mantovani, M., Ceroni, G. B., Pazzaglia, P., Petrella, A., Lugaresi, E.: Sindromi ipersonniche-ipoventilatorie. Minerva med.61, 1073 (1970)

    PubMed  Google Scholar 

  7. Cohn, J. E., Kuida, H.: Primary alvolar hypoveontilation associated with Western equine encephalitis. Ann. intern. Med.56, 633 (1962)

    PubMed  Google Scholar 

  8. Comroe, J. H.: Physiologie der Atmung. Stuttgart: Schattauer 1968

    Google Scholar 

  9. Fishman, L. S., Samson, J. H., Sperling, D. R.: Primary alveolar hypoventilation syndrome (Ondine's curse). Amer. J. Dis. Child.110, 155 (1965)

    PubMed  Google Scholar 

  10. Fraser, R. S., Sproule, B. J., Dvorkin, J.: Hypoventilation, cyanosis and polycythemia in thin man. Canad. med. Ass. J.89, 1178 (1963)

    PubMed  Google Scholar 

  11. Fruhmann, G.: Hypersomnia with primary hypoventilation syndrome and following cor pulmonale (Ondine's curse Syndrome). Bull. Physio-path. resp.8, 1173 (1972)

    Google Scholar 

  12. Fruhmann, G., Blömer, H., Kolb, P.: Primäre alveoläre Hypoventilation infolge einer Funktionsstörung des Atemzentrums unbekannter Genese. Klin. Wschr.39, 618 (1961)

    PubMed  Google Scholar 

  13. Garlind, T., Linderholm, H.: Hypoventilation syndrome in a case of chronic epidemic encephalitis. Acta med. Scand.162, 333 (1958)

    PubMed  Google Scholar 

  14. Geisler, L., Herberg, D., Schlierf, G.: Primäre alveolare Hypoventilation. Dtsch. med. Wschr.92, 2056 (1967)

    PubMed  Google Scholar 

  15. Gerardy, W., Herberg, D., Kuhn, H. M.: Chronische primäre alveoläre Hypoventilation. Klin. Wschr.38, 583 (1960)

    PubMed  Google Scholar 

  16. Grant, J. L., Arnold, W.: Idiopathic hypoventilation. J. Amer. med. Ass.194, 99 (1956)

    Google Scholar 

  17. Hadorn, W., Scherrer, M.: Essentielle alveoläre Hypoventilation mit Cor pulmonale. Schweiz. med. Wschr.89, 647 (1959)

    PubMed  Google Scholar 

  18. Hornbein, T. F.: The relation between stimulus to chemoreceptors and their response. In: Arterial chemoreceptors (Hrsg. R. W. Torrance). Oxford 1968

  19. Joels, N., Neil, E.: Chemoreceptor impulse activity evoked by perfusion of the glomus at various pCO2 and pH values. J. Physiol. (Lond.)154, 7 (1960)

    Google Scholar 

  20. Kafer, E. R., Leigh, J.: Recurrent respiratory failure associated with the absence of ventilatory response to hypercapnia and hypoxemia. Amer. Rev. resp. Dis.106, 100 (1972)

    PubMed  Google Scholar 

  21. Lawrence, L. T.: Idiopathic hypoventilation, polycythemia and cor pulmonale. Amer. Rev. resp. Dis.80, 575 (1959)

    PubMed  Google Scholar 

  22. Lugaresi, E., Coccagna, G., Mantovani, M., Ceroni, B. Pazzaglia, P.: Ipersonnie exenziali associate a respirazione periodica. Sist. nerv.21, 18 (1969)

    Google Scholar 

  23. Mürtz, R., Ashba, J., Strahringer, B., Gymnich, B.: Essentielle alveoläre Hypoventilation bei Hyperpnoe. Med. thorac. (Basel)23, 294 (1966)

    PubMed  Google Scholar 

  24. Naeye, R. L.: Alveolar hypoventilation and cor pulmonale secondary to damage to respiratory center. Amer. J. Cardiol.8, 416 (1961)

    PubMed  Google Scholar 

  25. Naughton, J., Block, R., Welch, M.: Central alveolar hypoventilation. A case report. Amer. Rev. resp. Dis.103, 500 (1971)

    Google Scholar 

  26. Nolte, D., Streller, S. E., Schütterle, G.: Die CO2-abhängige Atemregulation bei Kranken mit chronischer metabolischer Azidose infolge Niereninsuffizienz. Verh. dtsch. Ges. inn. Med.76, 700 (1970)

    Google Scholar 

  27. Oliva, P. B., Williams, M. H., Park, S. S.: Alveolar hypoventilation syndrome: Case of clinical recovery despite continued absence of ventilatory response to inhaled carbon dioxide. Amer. Rev. resp. Dis.96, 805 (1967)

    PubMed  Google Scholar 

  28. Overholt, E., Richert, J., Gilliland, P.: Idiopathic central hypoventilation. Dis. Chest52, 553 (1967)

    PubMed  Google Scholar 

  29. Paré, P., Lowenstein, L.: Polycythemia associated with disturbed function of the respiratory center. Blood11, 1077 (1956)

    PubMed  Google Scholar 

  30. Richter, T., West, J. R., Fishman, A. P.: The syndrome of alveolar hypoventilation and diminished sensitivity of the respiratory center. New Engl. J. Med.256, 1165 (1957)

    PubMed  Google Scholar 

  31. Rodman, T., Resnick, M. E., Berkowith, R., Fennelly, J. F., Olivia, J.: Alveolar hypoventilation due to involvement of the respiratory center by disease of the central nervous system. Amer. J. Med.32, 208 (1962)

    PubMed  Google Scholar 

  32. Rodman, T., Close, H. P.: The primary hypoventilation syndrome. Amer. J. Med.26, 808 (1959)

    PubMed  Google Scholar 

  33. Seriff, N. S.: Alveolar hypoventilation with normal lungs: The syndrome of primary or central alveolar hypoventilation. Ann. N.Y. Acad. Sci.121, 691 (1965)

    PubMed  Google Scholar 

  34. Severinghaus, J. W., Mitchell, R. A.: Ondine's curse — failure of respiratory center automaticity while awake. Clin. Res.10, 122 (1962)

    Google Scholar 

  35. Solliday, N. H., Gaensler, E. A., Schwaber, J. R., Parker, T. F.: Impaired central chemoreceptor function and chronic hypoventilation many years following poliomyelitis. Respiration31, 177 (1974)

    PubMed  Google Scholar 

  36. Strieder, D. J., Baker, W. G., Baringer, J. R., Kazemi, H.: Chronic hypoventilation of central origin: A case of encephalitis lethargica and Parkinson's syndrome. Amer. Rev. resp. Dis.96, 501 (1967)

    PubMed  Google Scholar 

  37. Tsitouris, G., Fertakis, A.: Alveolar hypoventilation due to respiratory center dysfunction of unknown cause. Amer. J. Med.39, 173 (1965)

    PubMed  Google Scholar 

  38. Witzleb, E., Bartels, H., Budda, H., Mochizucki, M.: Der Einfluß des arteriellen O2-Drucks auf die chemoreceptorischen Aktionspotentiale im Carotissinusnerven. Pflügers Arch. ges. Physiol.261, 211 (1955)

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Zentrum für Innere Medizin am Klinikum der Justus Liebig-Universität Gießen, Germany

    D. Nolte & H. -G. Lasch

Authors
  1. D. Nolte
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. H. -G. Lasch
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Nolte, D., Lasch, H.G. Das primäre Hypoventilationssyndrom. Klin Wochenschr 52, 781–786 (1974). https://doi.org/10.1007/BF01468738

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01468738

Key words

Schlüsselwörter

Search

Navigation