Management of myasthenia gravis in daily practice for general neurologists and healthcare professionals

Abstract

A characteristic feature of myasthenia gravis (MG) is the fluctuating weakness of the muscles. MG is an autoimmune neuromuscular junction disorder mediated by autoantibodies against nicotinic acetylcholine receptor and muscle-specific tyrosine kinase. If the commercially available acetylcholine receptor and muscle-specific tyrosine kinase antibody test is negative, MG patients are termed as having double-seronegative MG. Although the clinical phenotype is not yet defined, antibodies for lipoprotein receptor-related protein 4 and agrin, involved in clustering of acetylcholine receptors within the postsynaptic membrane and in structural maintenance of the neuromuscular synapse, have been found. The updated 2014 Guidelines in Japan state that the management of MG should be individualized, and earlier aggressive treatment using steroid pulse, plasma exchange and intravenous immunoglobulin therapy, before long-term steroid therapy, should be used in order to minimize manifestation, with fewer adverse effects achieved by co-administering immunosuppressants. In 2016, thymectomy was shown to be efficacious in non-thymomatous MG in a single-arm randomized clinical trial. The continuous update and modification in MG management makes it difficult for the general neurologist to decide on a treatment strategy. The present review aims to improve the understanding of current MG diagnosis, specific immunotherapy and clinical management tips, not only for the general neurologist, but also for other medical staff.

Using a scenario approach to assess for the current and future demand of immunoglobulins: An interview and literature study from The Netherlands

“…The effect of the latter could be hampered if there was standardisation of dosing based on ideal or adjusted body weight but wide variations in practice still persist. 96 , 97 , 98 Arguably, Ig need not be the first line of treatment options, 4 , 99 but the broadened EMA definition for SIDs 28 provides allowance for it to be.…”