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


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.


Stiff-man syndrome steroid drugs 


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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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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