Italian recommendations for the diagnosis and treatment of myasthenia gravis

Neurol Sci. 2019 Jun;40(6):1111-1124. doi: 10.1007/s10072-019-03746-1. Epub 2019 Feb 18.


Myasthenia gravis is a well-treatable disease, in which a prompt diagnosis and an adequate management can achieve satisfactory control of symptoms in the great majority of patients. Improved knowledge of the disease pathogenesis has led to recognition of patient subgroups, according to associated antibodies, age at onset and thymus pathology, and to a more personalized treatment. When myasthenia gravis is suspected on clinical grounds, diagnostic confirmation relies mainly on the detection of specific antibodies. Neurophysiological studies and, to a lesser extent, clinical response to cholinesterase inhibitors support the diagnosis in seronegative patients. In these cases, the differentiation from congenital myasthenia can be challenging. Treatment planning must consider weakness extension and severity, disease subtype, thymus pathology, together with patient characteristics and comorbidities. Since most subjects with myasthenia gravis require long-term immunosuppressive therapy, surveillance of expected and potential adverse events is critical. For patients refractory to conventional immunosuppression, the use of biologic agents is highly promising. These recommendations are addressed to non-experts on neuromuscular transmission disorders. The diagnostic procedures and therapeutic approaches hereafter described are largely accessible in Italy.

Keywords: Immunosuppressive therapy; Myasthenia gravis; Plasma exchange; Rituximab; Thymectomy.

MeSH terms

  • Female
  • Humans
  • Italy / epidemiology
  • Male
  • Myasthenia Gravis / diagnosis*
  • Myasthenia Gravis / epidemiology
  • Myasthenia Gravis / therapy*
  • Practice Guidelines as Topic