Myasthenic Crisis
System Diseases     All Diseases     eMedicine
General Info HPI/Physical

     Autoimmune disorder of the peripheral nerves.  Antibodies are formed against the acetylcholine postsynaptic receptors at the myoneural junction. The ACh receptors are destroyed resulting progressively reduced muscle strength with repeated use of the muscle with recovery of muscle strength after rest.
     The bulbar muscles are most common and most severely affected. The bulbar muscles are the ones that deal with speech and swallowing. Patients can often have normal strength but have airway compromise, but most patients will have generalized weakness at some time.
     Exacerbation of disease can be due to noncompliance, infection, and other stressors
Some medications are known for causing exacerbations

  • Antibiotics: Macrolides, fluoroquinolones, aminoglycosides
  • Antidysrhythmics: Beta blockers, CCBs, lidocaine, procainamide
  • Miscellaneous: muscle relaxants, levothyroxine, and corticosteroids

Look for signs of cholinergic crisis from excess of cholinesterase inhibitors which can mimic myasthenic crisis. Both present with muscle weakness and can be indistinguishable. Look for signs of Miosis and the SLUDGE syndrome

History: Patients usually complain of difficulty breathing or difficulty swallowing.

Symptoms:
Inability to clear secretions leading to rales, rhonchi, wheezes, evidence of pneumonia, cough, fever. Can have mild distress, anxious and rapid and shallow breathing.

Severe exacerbation: expressionless face, unable to support head, nasally voice, limp body, absent gag reflex and respiratory distress.

Signs:
The most important thing to assess in the patients in the ability to adequately ventilation and clear secretions




Tests/Diagnosis
Management
Disposition

CBC
Comprehensive Panel
PT/PTT
ABG
TSH

Chest x-ray for pneumonia or aspiration
Chest CT or MRI for thymoma

The Tensilon (edrophonium) challenge test useful but not 100% specific

Ice over eyelids with improvement of ptosis:  sensitivity 82% and specificity 96%

ED evaluation of pulmonary function:
- Peak expiratory flow
- FEV1
- Vital capacity

High-flow oxygen
pulse oximetry
Suction
BiPAP

Pyridostigmine 60mg q6-8hrs
Prednisone 30-60mg

RSI:
- Avoid succinylcholine
- Rocuronium or Vecuronium preferred

Consult Neurology


Admit to Neurology or ICU for observation

repeated vital capicity q4-6hrs, if <1L patient will need BiPAP and possible intubation

Pyridostigmine
Prednisone
IVIG

Plasmapheresis can be of benefit

Inpatient exams: electromyography, repetitive nerve stimulation, ACh receptor antibody

Clinical improvement takes days to weeks.[14] Because of the delayed onset of beneficial effects, plasmapheresis has limited utility in the ED setting.

Thymectomy if indicated