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Description

Development of respiratory failure in a patient with myasthenic gravis is termed a crisis, since it leads to death if untreated. A crisis may occur due to different mechanisms, which need to be understood if effective therapy is to be given.


 

Types of crisis:

(1) myasthenic: severe weakness due to accelerated disease activity, which may be precipitated by infection, stress, drugs or trauma. This type is responsive to increasing the dose of anticholinesterase agents.

(2) cholinergic: relative drug overdosage, often due to a spontaneous reduction in disease activity, with nicotinic ganglionic blockade. This requires reducing or stopping drug therapy.

(3) brittle: associated with severe bulbar involvement, often in patients with thymomas, with alternating myasthenic and cholingergic states.

Stage

Group

Findings

I

incipient crisis

obvious respiratory difficulty may not be present; bulbar symptoms may be prominent; spirometry may show a decrease in tidal volume and vital capacity

II

objective

evidence of ventilatory distress present

III

apneic

patient stops breathing

 

Bulbar signs:

(1) dysarthria

(2) dysphagia

(3) dysphonia (from paresis of vocal cords)

(4) pooling of secretions

 

Objective signs of ventilatory distress:

(1) decreased vital capacity

(2) increased blood pressure

(3) cyanosis

(4) decreased respirations

 

Management:

(1) In cholinergic and brittle crisis medications are stopped, while in myasthenic crisis an anticholinesterase agent is given.

(2) Intubation or tracheostomy with respiratory support.

 


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