Myasthenia gravis: not so grave anymore

A common theme in JNNP blogs has been the major paradigm shift in neurological practice, with a change from diagnosis (without treatment) to another in which making a diagnosis was a prelude to providing effective treatment. There have been few disorders in which this has been more clearly visible than that of myasthenia gravis, an autoimmune disorder in which there are antibodies directed against the neuromuscular junction. Patients typically report significant muscle weakness, which may affect the eyes, limbs, respiration and muscles that control speech and swallowing.  There is a beautiful diagram in Engel’s textbook on myasthenia which outlines the marked reductions in mortality from myasthenia have paralleled the major advances in critical care medicine, particularly in the provision of assisted ventilation (reproduced here in a review in Muscle and Nerve: http://onlinelibrary.wiley.com/enhanced/doi/10.1002/mus.20030). The diagram also notes that steroids and plasma exchange have been important treatment strategies in combating this illness.

However, one issue that not infrequently rears its head in management of these patients is how to advise and treat a patient with myasthenia who is pregnant. Managing patients with neurological disease who are pregnant is hard and can be quite anxiety-provoking, whether we are talking about epilepsy, multiple sclerosis or myasthenia. As neurologists, we may feel that we need to treat the mother first, but pregnant women are often very understandably concerned about the potential teratogenic effects of the treatments that we use. A lot of my own patients would rather go without treatment than run the risk of congenital anomalies. Of course, this has to be balanced against the risks to the foetus from the mother’s neurological diagnosis itself. Myasthenia at its worst causes respiratory failure, dangerous for the pregnant patient and for her foetus. As neurologists, what should we do?

In this month’s issue of JNNP, Norwood and colleagues have taken on the challenge of providing guidelines for the management of myasthenia and pregnancy http://jnnp.bmj.com/content/85/5/538.abstract . They have done a stellar job in providing guidelines that will assist neurologists around the world. This is an excellent paper, which should be read by all.

We would also be interested in hearing from neurologists around the world about their own experiences in this area. How do you treat these patients and what have you learnt from managing this condition?

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