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The Italian Journal of Neurological Sciences

, Volume 9, Issue 6, pp 559–566 | Cite as

Steroid-responsive and dependent stiff-man syndrome: A clinical and electrophysiological study of two cases

  • Piccolo G. 
  • Cosi V. 
  • Zandrini C. 
  • Moglia A. 
Original Articles

Abstract

Two female patients with the typical clinical and electrophysiological features of the stiff-man syndrome, both responded to steroid treatment. ACTH infusion produced an immediate clinical relief of muscle contracture and cramps, with parallel marked reduction of the EMG pattern of continuous spontaneous activity in agonist and antagonist muscles. Apart from this effect, a more delayed response to oral prednisone was observed in both cases and steroid-dependence in one of them, who also exhibited instrumental and laboratory findings suggesting an inflammatory process. These data lead us to consider a possible dysimmune pathogenesis of some cases with the stiff-man syndrome.

Key-Words

Stiff-man syndrome steroid drugs 

Sommario

Vengono descritte due pazienti di sesso femminile, ambedue con quadro clinico ed elettrofisiologico compatibile con la diagnosi di “stiff-man syndrome”, ambedue responsive a trattamento steroideo. L'infusione di ACTH ha determinato un'immediata riduzione o scomparsa delle contratture muscolari, con parallela riduzione del “pattern” EMG di attività continua di fibra nei muscoli agonisti ed antagonisti. Oltre a questo effetto acuto, abbiamo osservato un più ritardato effetto risolutivo del prednisone per os, con remissione in un caso e corticodipendenza nel secondo, il quale presentava reperti liquorali e strumentali indicativi di un processo infiammatorio del SNC. Questi dati ci inducono a considerare una possibile patogenesi disimmune almeno di alcuni casi di “stiff-man syndrome”.

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References

  1. [1]
    Moersch F.P., Woltman H.W.:Progressive fluctuating muscular rigidity and spasm (stiff-man syndrome): report of a case and some observations on 13 other cases. Mayo Clin Proc. 31:421–7, 1956.PubMedGoogle Scholar
  2. [2]
    Adams R.D., Victor M.:Principles of neurology. New York: McGraw-Hill Book Company, 1092, 1985.Google Scholar
  3. [3]
    Coërs C., Telerman-Toppet N., Durdu J.:Neurogenic benign fasciculations, pseudomyotonia and pseudotetany. A disease in search of a name. Arch Neurol 38:282–7, 1981.PubMedGoogle Scholar
  4. [4]
    Valli G., Barbieri G., Cappa Pellegrini G., Scarlato G.:Syndromes of abnormal muscular activity: overlap between continuous muscle fibre activity and the stiff man syndrome. J Neurol Neurosurg Psychiatry 46:241–7, 1983.PubMedGoogle Scholar
  5. [5]
    Lhermitte F., Chain F., Escourolles R., Chedru F., Guillelminault C. Francoual M.:Un nouveau cas de contracture tétaniforme distinct du “stiff-man” syndrome. Etude pharmacologique et neuropathologique d'un cas d'encéphalomyélite à prédominance médullaire. Rev Neurol, 128:3–21, 1973PubMedGoogle Scholar
  6. [6]
    Kasperek S., Zebroski S.:Stiff-man syndrome and encephalomyelitis. Arch Neurol 24:21–30, 1971.Google Scholar
  7. [7]
    Whiteley A.M., Swash M., Urich H.:Progressive encephalomyelitis with rigidity. Its relation to “subacute myoclonic spinal neuronitis” and to the “stiff-man syndrome”. Brain 99:27–42, 1976.PubMedGoogle Scholar
  8. [8]
    Prier S. Benoit C., Masson M., Cambier J.:Etude d'une observation de contracture permanente proche d'un syndrome de l'home raide, avec signes neurologiques associés. Encéphalomyélite probable. Rev EEG Neurophysiol 12:79–85, 1982.Google Scholar
  9. [9]
    George T.M., Burke J.M., Sobotka P.A., Greenberg H.S., Vinik A.I.:Resolution of stiff-man syndrome with cortisol replacement in a patient with deficiencies of ACTH, growth hormone and prolactin. New Engl J Med 23:1511–13, 1984.Google Scholar
  10. [10]
    Maida E., Reisner T., Summer K., Eggerth H.:Stiff man syndrome with abnormalities in CSF and computerized tomography findings. Arch Neurol 37:182–3, 1980.PubMedGoogle Scholar
  11. [11]
    Foster J.B.:The clinical features of some miscellaneous neuromuscular disorders. In: Walton JN, ed. Disorders of voluntary muscle. London: Churchill Livingstone, 902, 1974Google Scholar
  12. [12]
    Haynes R.C., Murad F.:Adrenocorticotropic hormone; adrencortical steroids and their synthetic analogs; inhibitors of adrenocortical steroid biosynthesis. In: Goodman Gilman A, Goodman LS, Gilman A, eds. The pharmacological basis of therapeutics. New York: MacMillan Publishing Co., 1466–96, 1980.Google Scholar
  13. [13]
    Martin J.B., Reichlin S., Brown G.M.:Effects of hormones on the brain. In: Clinical endocrinology. Philadelphia: Davis Company, 281–2, 1977.Google Scholar
  14. [14]
    Howell D.A., Less A.J., Toghill P.J.:Spinal internuncial neurones in progressive encephalomyelitis with rigidity. J Neurol Neurosurg Psychiatry 42: 773–85, 1979.PubMedGoogle Scholar
  15. [15]
    Gordon E.E., Janusko D.M., Kaufman L.:A clinical survey of stiff-man syndrome. Am J Med 42: 582–99, 1967.CrossRefPubMedGoogle Scholar
  16. [16]
    Meinck H.M., Conrad B.:Neuropharmacological investigation in the stiff-man syndrome. J Neurol 233:340–7, 1986PubMedGoogle Scholar

Copyright information

© Masson Italia Editori 1988

Authors and Affiliations

  • Piccolo G. 
    • 1
  • Cosi V. 
    • 1
  • Zandrini C. 
    • 1
  • Moglia A. 
    • 1
  1. 1.Istituto Neurologico “C. Mondino” Clinica NeurologicaUniversità di PaviaPavia

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