Myasthenia gravis treatment

In severely ill patients, the first priority is to protect respiration by intubation and, if necessary, ventilation. Anticholinesterase drugs

This is the longest established form of treatment (1930s).

Anticholinesterase drugs inhibit cholinesterase, the enzyme responsible for the breakdown of acetylcholine, allowing enhanced receptor stimulation. As a result, more acetylcholine is available to effect neuromuscular transmission.

ANTICHOLINESTERASES DURATION OF ACTION METHOD OF ADMINISTRATION

Edrophonium— I__- Intravenous

Neostigmine-- ~ *mravenous> >mramuscu'ar> oral

4 min 2 hours 4 hours

A muscarinic inhibitor, atropine, may be required to counter side effects, (nausea, vomiting, diarrhoea, muscle fasciculations and increasing weakness). Anticholinesterases rarely give complete symptomatic relief and large doses can result in a cholinergic crisis

- worsening weakness

- increased sweating, saliva and bronchial secretions

- small pupils (miosis)

- eventual respiratory failure.

Atropine may mask early warning symptoms of this potential life-threatening state.

Steroids

Because this disorder is immune-mediated steroids are a logical choice in generalised and occasionally severe ocular disease. Prednisone 60 mg/day is initially used. Deterioration may briefly occur before improvement. Once a response occurs, dosage is reduced to alternate days.

Immunosuppressants other than steroids

These drugs (azathioprine, cyclophosphamide and cyclosporine) are considered in patients who do not respond to steroids or who require an unacceptably high steroid maintenance dose.

Thymectomy

There are two indications for this:

1. When thymoma is present

2. When myasthenia is generalised and benefits of surgery outweigh risks. Trans-sternal is preferred to supra-sternal approach giving better chance of total clearance. Within 5 yrs of surgery 80% of patients are in remission.

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