2019意大利建议_重症肌无力的诊断和治疗.pdfVIP

Neurological Sciences /10.1007/s10072-019-03746-1 REVIEW ARTICLE Italian recommendations for the diagnosis and treatment of myasthenia gravis Amelia Evoli 1,2 Giovanni Antonini3 Carlo Antozzi4 Antonio DiMuzio 5 Francesco Habetswallner6 Cesare Iani 7 Maurizio Inghilleri8 Rocco Liguori9,10 Renato Mantegazza4 Roberto Massa 11 Elena Pegoraro 12 Roberta Ricciardi 13 Carmelo Rodolico 14 Received: 16 November 2018 /Accepted: 31 January 2019 # Fondazione Società Italiana di Neurologia 2019 Abstract 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 ex- pected 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 Myasthenia gravis . Thymectomy . Immunosuppressive therapy . Plasma exchange . Rit