Management

Although the response to edrophonium may be strongly positive in this situation, it would be unwise to rely on adjustment of medication alone to salvage the situation in the first instance. If the patient is in a distressed state with breathing difficulties, it is essential to control the airway first and to provide satisfactory ventilation and oxygenation. This means rapid and efficient intubation. Once ventilation is under control, the situation can be reassessed. An edrophonium test can be performed and suitable anticholinesterase medication prescribed. Pyridostigmine can be administered as an elixir via a nasogastric tube.

Any precipitating factors should be corrected (e.g. treatment of infection). Further treatment of the myasthenia should be reviewed. Plasma exchange, intravenous immunoglobulin, or IgG immunoadsorption should be considered with the aim of producing a rapid improvement in clinical state. Long-term management with steroids and azathioprine should also be assessed.

If extubation is unlikely to be achieved within a few days, tracheostomy should be considered as a means of providing a safe and more comfortable weaning from ventilation.

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