Skip to main content
Log in

Stiff man syndrome: neurophysiological findings in eight patients

  • Original Communication
  • Published:
Journal of Neurology Aims and scope Submit manuscript

Abstract

The neurophysiological findings in eight patients with the stiff man syndrome (SMS), including four of six tested with autoantibodies against glutamic acid decarboxylase, are presented. Neurophysiological findings did not make it possible to discriminate between patients with and those without autoimmunity against GABAergic neurons. Investigation of mono-and polysynaptic reflexes revealed abnormal results in a variable number of SMS patients, the abnormalities largely corresponding to those seen in spastic paresis. A stereotyped motor response to electrical stimulation of peripheral nerves was recorded from the trunk muscles of all patients investigated. This response was termed spasmodic reflex myoclonus and consisted of a sequence of 1–3 synchronous myoclonic bursts, 60–70 ms after median nerve stimulation, followed by a tonic decrescendo activity over a number of seconds. The recruitment order of muscles along the neuraxis in spasmodic reflex myoclonus suggested that the latter was generated in the spinal cord and conveyed via propriospinal tracts. It is thought that spasmodic reflex myoclonus may serve not only as a diagnostic tool, but also as a key to understanding some aspects of the pathophysiology of both spasms and stiffness in SMS. It is speculated that stiffness is a fragment of spasms, both being generated by common neuronal mechanisms tentatively ascribed to interneurons in the spinal grey matter.

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

Access this article

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

References

  1. Baldissera F, Hultborn H, Illert M (1981) Integration in the spinal cord. In: Brookhart JM, Mountcastle VB, Brooks VB, Geiger SR (eds) Handbook of physiology, section 1, vol II. American Physiological Society, Bethesda, pp 509–595

    Google Scholar 

  2. Brown P, Rothwell JC, Thompson PD, Britton TC, Day BL, Marsden CD (1991) The hyperekplexias and their relationship to the normal startle reflex. 114:1903–1928 Brain

    Google Scholar 

  3. Brown P, Thompson PD, Rothwell JC, Day BL, Marsden CD (1991) Axial myoclonus of propriospinal origin. Brain 114:197–214

    Google Scholar 

  4. Chokroverty S, Walters A, Zimmerman T, Picone M (1992) Propriospinal myoclonus: a neurophysiologic analysis. Neurology 42:1591–1595

    Google Scholar 

  5. Delwaide PJ (1973) Human monosynaptic reflexes and presynaptic inhibition. In: Desmedt JE (ed) New developments in electromyography and clinical neurophysiology, vol 3. Karger, Basel, pp 508–522

    Google Scholar 

  6. Deuschl G, Lücking CH (1990) Physiology and clinical applications of hand muscle reflexes. In: Rossini PM, Mauguiere F (eds) New trends and advanced techniques in clinical neurophysiology. Elsevier, Amsterdam, pp 84–101

    Google Scholar 

  7. Gordon EE, Januszko DM, Kaufman L (1966) A critical survey of stiff-man syndrome. Am J Med 42:582–599

    Google Scholar 

  8. Hallett M (1985) Myoclonus: relation to epilepsy. Epilepsia [Suppl] 126:S567–5577

    Google Scholar 

  9. Hallett M, Chadwick D, Adam J, Marsden CD (1977) Reticular reflex myoclonus: a physiological type of human post-hypoxic myoclonus. J Neurol Neurosurg Psychiatry 40:253–264

    Google Scholar 

  10. Hallett M, Chadwick D, Marsden CD (1977) Ballistic movement overflow myoclonus. A form of essential myoclonus. Brain 100:299–312

    Google Scholar 

  11. Howard FM (1963) A new and effective drug in the treatment of stiff-man syndrome: preliminary report. Mayo Clin Proc 38:203–212

    Google Scholar 

  12. Isaacs H (1971) Stiff man syndrome in a black girl. J Neurol Neuosurg Psychiatry 42:988–994

    Google Scholar 

  13. Ishikawa K, Ott K, Porter RW, Stuart D (1966) Low frequency depression of the H wave in normal and spinal man. Exp Neurol 15:140–156

    Google Scholar 

  14. Markand ON, Garg BP, Weaver DD (1984) Familial startle disease (hyperekplexia): electrophysiologic studies. Arch Neurol 41:71–74

    Google Scholar 

  15. Martinelli P, Pazzaglia P, Montagna P, Coccagna G, Rizzuto N, Simonati S, Lugaresi E (1978) Stiff-man syndrome associated with nocturnal myoclonus and epilepsy. J Neurol Neurosurg Psychiatry 41:458–462

    Google Scholar 

  16. Meinck H-M (1991) Das Stiff-manSyndrom: eine Immunopathie? Nervenarzt 62:762–765

    Google Scholar 

  17. Meinck H-M (1991) Spasmodic polymodal reflex myoclonus: characteristic for the stiff man syndrome. Z EEG-EMG 21:131

    Google Scholar 

  18. Meinck H-M, Conrad B (1986) Neuropharmacological investigations in the stiff man syndrome. J Neurol 233:340–347

    Google Scholar 

  19. Meinck H-M, Ricker K, Conrad B (1984) The stiff-man syndrome: new pathophysiological aspects from abnormal exteroceptive reflexes and the response to clomipramine, clonidine, and tizanidine. J Neurol Neurosurg Psychiatry 47:280–287

    Google Scholar 

  20. Meinck HM, Benecke R, Conrad B (1985) Cutaneo-muscular control mechanisms in health and disease: possible implications on spasticity. In: Struppler A, Weindl A (eds) Electromyography and evoked potentials. Springer, Berlin, pp 75–83

    Google Scholar 

  21. Meinck HM, Küster S, Benecke R, Conrad B (1985) The flexor reflex-influence of stimulus parameters on the reflex response. Electroencephalogr Clin Neurophysiol 61:287–298

    Google Scholar 

  22. Meinck HM, Ricker K, Hülser PJ, Schmid E, Peiffer J, Solimena M (1994) Stiff man syndrome: clinical and laboratory findings in 8 patients. J Neurol 241:157–166

    Google Scholar 

  23. Moersch FP, Woltman HW (1956) Progressive fluctuating muscular rigidity and spasm (“stiff-man” syndrome): report of a case and some observations in 13 other cases. Mayo Clin Proc 31:421–427

    Google Scholar 

  24. Obeso JA, Rothwell JC, Marsden CD (1985) The spectrum of cortical myoclonus. Brain 108:193–224

    Google Scholar 

  25. Solimena M, De Camilli P (1991) Autoimmunity to glutamic acid decarboxylase (GAD) in stiff man syndrome and insulin-dependent diabetes mellitus. Trends Neurosci 14:452–457

    Google Scholar 

  26. Thompson PD, Colebatch JG, Brown P, Rothwell JC, Day BL, Obeso JA, Marsden CD (1992) Voluntary stimulus-sensitive jerks and jumps mimicking myoclonus or pathological startle syndromes. Mov Disord 7:257–262

    Google Scholar 

  27. Wilkins DE, Hallett M, Wess MM (1986) Audiogenic startle reflex of man and its relationship to startle syndromes. Brain 109:561–573

    Google Scholar 

  28. Williams AC, Nutt JG, Hare T (1988) Autoimmunity in stiff man syndrome. Lancet II:222

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Meinck, H.M., Ricker, K., Hülser, P.J. et al. Stiff man syndrome: neurophysiological findings in eight patients. J Neurol 242, 134–142 (1995). https://doi.org/10.1007/BF00936885

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00936885

Key words

Navigation