Skip to main content

Stiff-man syndrome with central and peripheral nervous manifestations

Summary

A case of stiff-man syndrome with characteristic anamnestic and clinical data is reported. Electrophysiological and histopathological results are referred and indicate that both central and peripheral nervous lesions may be present.

Zusammenfassung

Es wird über einen Fall von Stiff-man-Syndrom mit typischer Anamnese und charakteristischen Befunden berichtet. Aufgrund der elektrophysiologischen und histopathologischen Befunde wird gefolgert, daß sowohl eine Läsion des Zentral- wie des peripheren Nervensystems vorliegen dürfte.

This is a preview of subscription content, access via your institution.

References

  1. 1.

    Dubowitz, V., Brooke, M. H.: Muscle biopsy: A modern approach. London, Philadelphia, Toronto: Saunders 1973

    Google Scholar 

  2. 2.

    Gordon, E. E., Januszko, D. M., Kaufman, L.: A critical survey of the stiff-man syndrome. Am. J. Med. 42, 582–599 (1966)

    Google Scholar 

  3. 3.

    Guilleminault, C., Sigwald, J., Castaigne, P.: Sleep studies and therapeutical trial with L-Dopa in a case of stiff-man syndrome. Europ. Neurol. 10, 89–96 (1973)

    Google Scholar 

  4. 4.

    Haefely, W., Pieri, L., Polc, P., Schaffner, R.: Benzodiazepines and GABA. Nature 263, 173–174 (1976)

    Google Scholar 

  5. 5.

    Howard, F. M.: A new and effective drug in the treatment of the stiff-man syndrome. Mayo Clin. Proc. 38, 203–212 (1963)

    Google Scholar 

  6. 6.

    Isaacs, H.: Syndrome of continuous muscle fiber activity. J. Neurol. Neurosurg. Psychiat. 24, 319–325 (1961)

    Google Scholar 

  7. 7.

    Isaacs, H.: Continuous muscle fiber activity in an Indian male with additional evidence of terminal motor fiber activity. J. Neurol. Neurosurg. Psychiat. 30, 126–131 (1967)

    Google Scholar 

  8. 8.

    Kasparek, S., Zebrowski, S.: Stiff-man syndrome and encephalomyelitis—report of a case. Arch. Neurol. 24, 22–30 (1971)

    Google Scholar 

  9. 9.

    Kendall, V.: Disseminated sclerosis and diabetes. Lancet 1953 II, 202

    Google Scholar 

  10. 10.

    Mamoli, B., Heiss, W.-D., Maida, E., Podreka, I.: Electrophysiological studies on the “stiffman” syndrome. J. Neurol. 217, 111–121 (1977)

    Google Scholar 

  11. 11.

    Mertens, H. G., Ricker, K.: Übererregbarkeit der gamma-Motoneurone beim Stiff-Man-Syndrom. Klin. Wschr. 46, 33–42 (1968).

    Google Scholar 

  12. 12.

    Moersch, F. P., Woltman, H. W.: Progressive fluctuating muscular rigidity and spasms (“stiff-man” syndrome): report of a case and some other observations in 13 other cases. Proc. Mayo Clin. 31, 421–428 (1956)

    Google Scholar 

  13. 13.

    Olafson, R. A., Mulder, D. W., Howard, F. M.: Stiff-man syndrome, a review of the literature, and report of three additional cases and discussion of the pathophysiology and therapy. Mayo Clin. Proc. 39, 131–144 (1964)

    Google Scholar 

  14. 14.

    Sawaya, M. C. G., Horton, R. W., Meldrum, B. S.: Effects of anticonvulsant drugs on the cerebral enzymes metabolizing GABA. Epilepsia 4, 649–655 (1966)

    Google Scholar 

  15. 15.

    Schmidt, R. T., Stahl, S. M., Spellmann, R.: A pharmacologic study of the stiff-man syndrome. Neurology 25, 622–626 (1975)

    Google Scholar 

  16. 16.

    Sigwald, J., Guilleminault, C.: Syndrome de contracture permanente. Rev. Neurol. 124, 191–212 (1971)

    Google Scholar 

  17. 17.

    Thorén, C.: Diabetes mellitus in Friedreich's ataxia. Acta paediatr. Suppl. 135, 239–242 (1962)

    Google Scholar 

  18. 18.

    Wallis, W. E., Poznak, A. V., Plum, F.: Generalized muscular stiffness, fasciculations, and myokimia of peripheral nerve origin. Arch. Neurol. 22, 430–439 (1970)

    Google Scholar 

  19. 19.

    Westblom, U.: Stiff-man syndrome and clonazepam. J. Am. med. Ass. 237, 1930 (1977)

    Google Scholar 

  20. 20.

    Whiteley, A. M., Swash, M., Urich, H.: Progressive encephalomyelitis with rigidity. Brain 99, 27–42 (1976)

    Google Scholar 

Download references

Author information

Affiliations

Authors

Corresponding author

Correspondence to S. I. Mellgren.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Nyland, H., Mellgren, S.I. & Frövig, A.G. Stiff-man syndrome with central and peripheral nervous manifestations. J. Neurol. 219, 171–176 (1978). https://doi.org/10.1007/BF00314532

Download citation

Key words

  • Stiff-man syndrome
  • EMG
  • Pharmacology
  • Muscle biopsy