The Italian Journal of Neurological Sciences

, Volume 11, Issue 2, pp 177–180 | Cite as

Autoimmune myastenia gravis with thymoma following the spontaneous remission of stiff-man syndrome

  • Piccolo G. 
  • Martino G. 
  • Moglia A. 
  • Arrigo A. 
  • Cosi V. 
Case Reports

Abstract

A patient who developed generalized autoimmune myasthenia gravis six years after the spontaneous remission of a stiff-man syndrome is described. He also suffered from chronic active hepatitis and had radiological evidence of a thymoma. He did not have diabetes mellitus. Besides anti-nicotinic acetylcholine receptor antibodies, anti-nuclear, anti-DNA, anti-mitochondrial and anti-skeletal muscle antibodies were found in his serum, while islet-cell antibodies were absent. Immunocytochemistry studies failed to demonstrate autoantibodies to GABA-ergic nerve terminals, although an aspecific neuronal immunostaining was observed. The clinical and immunological features of this case support the hypothesis of a dysimmune pathogenesis of SMS, also in cases not associated with autoimmunity to GABA-ergic nerve terminals. Furthermore, a relationship between thymoma and the neurological syndromes discussed could be considered.

Key-Words

Stiff-man syndrome autoimmune myasthenia gravis paraneoplastic neurological syndromes 

Sommario

Viene descritto il caso di un paziente che sviluppò una forma di Miastenia Gravis generalizzata autoimmune con evidenza radiologica di timoma sei anni dopo la remissione di una sindrome di contrattura muscolare permanente con caratteristiche elettrofisiologiche e di risposta ai farmaci tipiche della “stiff-man syndrome”, sebbene inizialmente associata a transitori segni di sofferenza troncoencefalica. Oltre ad anticorpi contro il recettore nicotico dell'acetilcolina, nel siero del paziente furono riscontrati anticorpi anti-nucleo, anti-DNA, anti-mitocondrio, anti-muscolo scheletrico. Il paziente non era diabetico e non presentava anticorpi contro le isole pancreatiche. Studi di immunoistochimica non hanno dimostrato anticorpi contro i terminali GABA-ergici, sebbene si sia riscontrato un certo grado di immunoreattività neuronale. Le caratteristiche cliniche ed immunologiche di questo caso suggeriscono l'ipotesi di una patogenesi disimmune di alcuni casi di “stiffman syndrome”, anche se non associati ad autoimmunità contro i terminali GABA-ergici ed inducono a considerare la possibilità di un coinvolgimento paraneoplastico-disimmune in queste forme.

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Copyright information

© Masson Italia periodici s.r.l. 1990

Authors and Affiliations

  • Piccolo G. 
    • 1
  • Martino G. 
    • 2
  • Moglia A. 
    • 1
  • Arrigo A. 
    • 1
  • Cosi V. 
    • 1
  1. 1.Istituto Neurologico C. Mondino, Clinica NeurologicaUniversità di PaviaPavia
  2. 2.Istituto Scientifico San Raffaele, Clinica Neurologica IVUniversità di MilanoMilanoItaly

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