CME Topic

Myasthenia Gravis Crisis

Eric M. Bershad,

MD,

Eliahu S. Feen,

MD,

and Jose I. Suarez,

Abstract: Myasthenia gravis (MG) is an autoimmune disorder resulting from the production of antibodies against acetylcholine receptors leading to the destruction of the postsynaptic membrane at
the neuromuscular junction. In the US there are about 18,000 people
with MG. Myasthenia gravis crisis (MGC) is defined as any MG
exacerbation necessitating mechanical ventilation. Most patients presenting with MGC have an identifiable risk factor. The diagnosis of
MGC should be suspected in all patients with respiratory failure,
particularly those with unclear etiology. Acute management of MGC
requires supportive general and ventilatory therapy and institution of
measures to improve the neuromuscular blockade. The latter includes plasma exchange or IV immunoglobulin, and removal of the
offending trigger. The outcome of patients with MGC has improved
significantly and the current mortality rate is about 4 to 8%.
Key Words: autoimmune disorders, mechanical ventilation, myasthenia gravis, myasthenia gravis crisis, respiratory failure

Epidemiology

he incidence of MG is 3 to 4 per million per year, and the

prevalence is about 60 per million.1 Thus, in the United
States there are about 18,000 people with MG. The incidence
of MG is twice as high in women compared with men. The
peak age of onset in women is in the childbearing years. In
men there is no clear peak age of onset.1 Overall, 15 to 20%
of patients with MG will experience a myasthenic crisis, and
it usually occurs within the first 2 years after diagnosis of MG
in most patients (74%).2

From the Division of Neurocritical Care, The Neurological Institute, University Hospitals Case Medical Center, Departments of Neurology and
Neurological Surgery, Case Western Reserve University, Cleveland,
Ohio; and the Department of Neurology, Baylor College of Medicine,
Houston, Texas.
Reprint requests to Dr. Jose I. Suarez, Director, Vascular Neurology and
Neurocritical Care, Department of Neurology, Baylor College of Medicine, One Baylor Plaza, NB 302, Houston, TX 77030. Email: jisuarez@
bcm.tmc.edu
Dr. Eliahu S. Feen has received an honorarium from Boehringer-Ingelheim.
Drs. Jose I. Suarez and Eric M. Bershad have no financial disclosures to
declare.
The authors have no commercial or proprietary interest in any drug, device,
or equipment mentioned in this article
Accepted March 20, 2007.
Copyright 2008 by The Southern Medical Association
0038-4348/02000/10100-0063

Southern Medical Journal Volume 101, Number 1, January 2008

MD

Risk Factors
Most patients that develop MGC have an identifiable
precipitating event; however, in 30 to 40% of patients, no
triggering factor can be found (Table 1).2,3 Furthermore, in
some patients, MGC may be the initial manifestation of MG.2
The most common identifiable precipitant of MGC is an infection (40%), usually in the upper airway (such as pneumonia). Another 10% of patients have aspiration pneumonitis as
the triggering event. Other important predisposing factors include the initiation of new medications or a change in medications, recent surgery, trauma, botulinum injections, and
thymoma.25 The presence of thymoma is higher among patients with MG who have a MGC than patients with MG but
no history of MGC (30% versus 15%).6
The most common medications that may trigger MGC
include the aminoglycoside and quinolone antibiotics, antiarrhythmics such as quinidine and procainamide, antihypertensives including -blockers and calcium channel blockers,
magnesium sulfate, and neuromuscular blocking agents such
as succinylcholine and curare derivatives.7 The initiation of
high-dose steroids in patients with MG leads to a paradoxical
worsening of muscle weakness in almost 50% of patients
(Table 2).8

Pathophysiology
MG is an autoimmune disease of the nicotinic acetylcholine receptor of skeletal muscle at the neuromuscular junc-

Key Points
Myasthenia gravis crisis (MGC) is defined as any myasthenia gravis (MG) exacerbation necessitating mechanical ventilation.
About 1520% of patients with myasthenia gravis will
experience MGC, typically within the first 2 years of
MG diagnosis.
Most patients with myasthenia gravis crisis have an
identifiable precipitating event; however in 30 40%
of patients, no triggering factor is found.
Plasma exchange or intravenous immunoglobulins are
indicated to help facilitate recovery of neuromuscular
blockade.
The overall outcome for patients with myasthenia gravis crisis is excellent if therapeutic and supportive
measures are instituted promptly.

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Bershad et al Myasthenia Gravis Crisis

Table 1. Triggers of myasthenia gravis crisis

Infections (40%) pneumonia, upper airway infection
Aspiration (10%)
Medications - Botulinum toxin, prednisone
Stress Surgery, trauma
Idiopathic (30%)

tion.9 In a normal individual, an action potential travels down

a motor nerve axon to the axon terminals and stimulates the
release of vesicles containing acetylcholine. The acetylcholine diffuses across the synapse at the neuromuscular
junction and stimulates the postsynaptic membrane by
binding to acetylcholine receptors. The stimulation of multiple acetylcholine receptors results in the production of
excitatory postsynaptic potentials which trigger muscle fiber action potentials. Subsequently, the muscle fiber action
potentials result in skeletal muscle contraction and generation of muscle power. Acetylcholine is rapidly inactivated
by acetylcholinesterase present in the postsynaptic membrane folds. In a patient with MG, an autoimmune attack
against acetylcholine receptors results in destruction of the
postsynaptic membrane (Fig.). With a reduced number of
available binding sites for acetylcholine, inconsistent generation of muscle fiber action potentials occurs, which
manifests as skeletal muscle weakness.9,10
Acetylcholine receptor antibodies are detected in approximately 90% of patients with generalized MG symptoms, and
in about 70% of patients with predominant involvement of
the ocular muscles.11,12 Three commercially available tests
for detection of acetylcholine receptor antibodies are the binding, modulating, and blocking assays. These antibodies provide a specific marker for MG. In those patients with negative
acetylcholine receptor antibody assays (approximately 10%), antibodies against other proteins have been implicated including
the following: muscle-specific kinase (MuSK); calcium and potassium channels in the motor nerve terminal; and titin and ryanodine receptor intracellularly in the muscle cell. Of these,

Table 2. Drugs that may exacerbate myasthenia gravis

Class
Immunosuppressants
Antibiotics
Antiarrhythmics
Antihypertensives
Neuromuscular blocking
agents
Neuropsychiatric
Others

64

Drug
Steroids
Aminoglycosides, ciprofloxacin, clindamycin,
erythromycin
Procainamide, quinidine
-Blockers (propranolol, timolol), calciumchannel blockers (verapamil)
Succinylcholine, curare derivatives, botulinum
toxin
Phenytoin, lithium
Magnesium sulfate

Fig. Depiction of the neuromuscular junction of a normal individual (A) and a patient with myasthenia gravis (B).

MuSK, a receptor tyrosine kinase, is the most clinically relevant,

and has been shown to cause phosphorylation of the acetylcholine receptors.

Clinical Presentation
MG usually presents with one of three different forms:
ocular, bulbar, or generalized.1,2,9 The hallmark presenting
characteristics are weakness and muscle fatigability. Depending on the predominant initial form, patients with MG can
complain of a plethora of symptoms including diplopia, eyelid ptosis, difficulty chewing and swallowing, dysarthria,
proximal limb weakness, generalized fatigue, and shortness
of breath. Characteristically the weakness of MG patients
worsens by prolonged or repetitive activity (eg, it is worse at
the end of the day), and improves with rest (eg, it is better in
the early morning hours).
Patients presenting with MGC are normally admitted to an
intensive care unit (ICU) because of the acute respiratory failure.
It is important for the examiner to have a high index of suspicion
for MGC as the initial manifestations may be subtle. In some
patients, MGC occurs as their initial presentation of MG.13 Because of this, MGC should be strongly considered in any patient
2008 Southern Medical Association

CME Topic

Table 3. Differential diagnosis of myasthenic crisis

Localization
Brainstem
Spinal cord
Motor neuron
Roots and peripheral
nerves
Neuromuscular junction

Muscles

Disease
Infarction, hemorrhage, compressive mass
lesion
Cervical cord compression
Amyotrophic lateral sclerosis, poliomyelitis,
West Nile virus
Guillain-Barre syndrome, acute intermittent
porphyria
Lambert-Eaton myasthenic syndrome,
botulinum poisoning, organophosphate
poisoning, spider or snake envenomation
Critical illness, myopathy/neuropathy,
polymyositis, acid-maltase deficiency,
hyper/hypokalemic periodic paralysis,
myopathy secondary to electrolyte
disturbances

with unexplained respiratory failure, particularly in those requiring prolonged mechanical ventilation.
On initial physical examination, the patient may appear
anxious. Rapid and shallow breathing may occur due to fatigue of respiratory muscles. There may be paradoxical chest
movements due to diaphragmatic weakness, manifested by
inward movement of the abdomen during inspiration. It is
important to assess the strength of neck muscles as there is a
correlation between these and diaphragm strength. The patients ability to speak can help to determine the integrity of
bulbar muscles. A wet, gurgling voice, dysarthric speech, and
stridor indicate poor airway protection.
It is paramount to bear in mind that other diseases may
mimic MGC. The etiologies that mimic MGC are diverse and
may affect the brainstem, spinal cord, motor neurons, peripheral nerves, neuromuscular junction or muscle (Table 3).
The mechanisms leading to respiratory compromise in
MGC stem from combined weakness of respiratory and bulbar musculature. Weakness of respiratory muscles including
the diaphragm and intercostals results in a progressive decrease in forced vital capacity (FVC), with specific clinical
manifestations (Table 4). Initially, a weak cough reflex results in accumulation of secretions. Next, an impaired sigh
reflex results in atelectasis. Hypoxia develops, along with
Table 4. Respiratory dysfunction associated with
decreasing forced vital capacity
Forced vital capacity
65 mL/kg
30 mL/kg
20 mL/kg
15 mL/kg
10 mL/kg
510 mL/kg

Manifestation
Normal respiratory function
Weak cough, accumulation of secretions
Atelectasis
Shunting of alveolar blood flow
Hypoventilation
Hypercapnia

Southern Medical Journal Volume 101, Number 1, January 2008

shunting of pulmonary blood flow. Finally, hypoventilation

occurs with resulting hypercarbia and acidosis. A simple bedside test to estimate FVC involves having the patient count numbers as high as possible on one breath; the number the patient
reaches multiplied by 100 mL approximates the FVC. For example, if the patient counts up to 10 on one breath, the FVC is
about 1000 mL. It is important to obtain bedside pulmonary
function tests (PFTs) promptly, to more formally evaluate the
FVC, as well as the positive expiratory force (PEF), and negative inspiratory force (NIF). Indications for mechanical ventilation in MG patients include an FVC 15 mL/kg (normal 60
mL/kg), a NIF 20 cm H2O (normal 70 cm H2O), or
PEF 40 cm H2O (normal 100 cm H2O). An arterial blood
gas showing a Pa02 60 mm Hg or PaCO2 60 mm Hg warrants immediate intubation of the patient; however, the clinician
should not wait for the arterial blood gas to become abnormal as
this occurs late in the course of MGC, once the patient has
already decompensated. Bedside PFTs should be performed serially as the clinical course of MG patients may deteriorate even
in patients who initially appear stable.
In addition to the compromise of respiratory function, bulbar muscle dysfunction including oropharynx and laryngeal musculature may contribute to respiratory failure. Normally, the oropharyngeal muscles maintain patency of the upper airway by
regulating its cross-sectional area. Dysfunction of these muscles
may result in increased airflow resistance in the upper airway.14
Swallowing function and the cough reflex may become impaired, resulting in an inability to protect the upper airway from
secretions. This may result in aspiration pneumonia and further
deterioration in respiratory status. The tongue may become weak
and obstruct the airway. Weakness of laryngeal muscles may
result in the vocal cords remaining adducted during inspiration,
further obstructing airflow. The latter is also called the sail
phenomenon since it simulates the wind blowing against the
sail of a boat.2

Management
Once the patient with suspected MGC has been identified, immediate steps should be taken to intubate the patient.
This should occur via a rapid sequence oral intubation.2 The
patient should be prepped by bag-masking to an arterial oxygen saturation 97%. IV normal saline should be infused
wide-open to help avoid hypotension associated with intubation. Continuous blood pressure monitoring is ideal. Etomidate is a commonly used anesthetic agent at a bolus IV dose
of 0.2 to 0.3 mg/kg. Paralytic agents should be avoided unless
absolutely necessary as MG patients are sensitive to their
effects.2,15 If necessary, a nondepolarizing agent such as vecuronium is preferred.
The initial ventilator settings should be optimized to allow for rest of the fatigued patient and promotion of lung
expansion. We recommend starting with assist control (AC)
with positive end expiratory pressure (PEEP) of 5 cm H2O,

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Bershad et al Myasthenia Gravis Crisis

low tidal volumes (6 mL/kg ideal body weight), and respiratory rate 12 to 16/min. Although in the past, large tidal volumes (12 mL/kg) have been recommended for MG patients,6
newer literature suggests that lower tidal volumes (6 mL/kg)
and faster respiratory rates (12-16 breaths/min) may help
avoid lung injury in ventilated patients.16 Adding intermittent
sighs (1.5 tidal volume) 3 to 4 times per hour, and PEEP
of 5 cm H2O can help prevent atelectasis.16
Once the patient has been intubated and stabilized, it is
essential to search for the trigger of MGC as it may require
additional treatment (Table 1). Routine diagnostic workup
should include obtaining a chest x-ray, cultures of sputum,
urine and blood, complete blood count, coagulation screen,
and comprehensive chemistry panel. If there is any doubt as
to whether the patient has MGC, electrodiagnostic studies (ie,
nerve conduction studies and electromyography) should be
performed. A decremental response of the amplitude of motor
action potentials following repetitive nerve stimulation is
highly suggestive of MG. A more definitive diagnosis of MG
depends on the identification of one of the pathogenic antibodies. In patients with suspected MGC the three acetylcholine receptor antibodies assays should be performed. If these
are negative and there is still a high index of suspicion then
MuSK antibody testing should be carried out. However, it is
important to bear in mind that results for the latter may take
weeks to months.
The next step in management of the MGC patient is to
institute acute therapy to help facilitate acute recovery of
neuromuscular blockade (Table 5). Acute therapy of MGC
usually includes either plasma exchange therapy or IV immunoglobulins (IVIg). There is generally no role for acetylcholinesterase inhibitors in the acute setting of MGC. Al-

though these agents may work quickly to improve neuromuscular

transmission, they may promote excessive secretions which
can lead to mucous plugging. Furthermore, cardiac arrhythmias, which are especially common in patients with MGC,
may be triggered by IV acetylcholinesterase inhibitors.17 Another issue that may be important to explore is the possibility
that patients may have an overdose of the acetylcholinesterase inhibitors. The clinical presentation of such overdose may
mimic MGC. The easiest manner to elicit this information is
asking the patient or relatives whether there has been a recent
increase in the usual dose of these medications. Management
of acetylcholinesterase inhibitors overdose remains supportive.
Plasma exchange therapy involves removing plasma containing acetylcholine receptor antibodies. Patients usually respond well to plasma exchange with an efficacy reported at
75%.6 Improvement is rapid and usually seen after two to
three sessions, but patients usually receive five sessions of
plasma exchange regardless of improvement. Although efficacious, plasma exchange has drawbacks, relating to the need
for a large-bore indwelling vascular access, and the hemodynamics of plasma exchange. Some of the serious adverse
events associated with the indwelling catheter include bleeding, pneumothorax, venous thrombosis, line infection and sepsis. The plasma exchange process may result in coagulopathy,
thrombocytopenia, electrolyte disturbances, arrhythmias, and
hypotension. The coagulopathy is frequently due to removal
of coagulant proteins along with the antibodies during plasma
exchange. Therefore daily monitoring of coagulation tests,
complete blood count, and serum electrolytes is warranted.
As an alternative to plasma exchange, one may consider
using IVIg. The therapeutic effect of IVIg starts about 5 days
after initiation of therapy.2 The efficacy of IVIg compared

Table 5. Short- and long-term therapies for myasthenia gravis crisis

Drug

Dosing

Time to onset

Adverse effects

11.5 plasma volume during each of

5 treatments qd or qod

After 23 sessions

400 mg/kg/d over 46 hours over a

5-day course

About 5 days after

initiation

Coagulopathy, thrombocytopenia, electrolytedisturbances,

cardiac arrhythmias, hypotension, and central-line
related complications
Headache, fever, chills, myalgias, aseptic meningitis, fluid
overload, renal failure, and thromboembolic events

3060 mg q 36 hours

Immediate
24 weeks

Azathioprine

Start at 20 mg daily and increase by

5 mg every 3 days until at
6080 mg daily
13 mg/kg/d

Cyclosporine

23 mg/kg/d

Several months

Mycophenolate mofetil

1000 mg bid

Months

Short-term
PE

IVIg
Long-term
Pyridostigmine
Prednisone

39 months

GI disturbances, sialorrhea, cramps, fasciculations,

bradycardia
Osteoporosis, weight gain, glaucoma, cataracts,
hypertension, psychiatric changes, easy bruising,
glucose intolerance, immunosupression
Flu-like reaction, leucopenia, hepatotoxicity, alopecia,
teratogen, neoplasia (rare)
Renal insufficiency, hypertension, multiple drug
interactions
Anemia, leucopenia, GI discomfort, diarrhea

PE, Plasma exchange; IVIg, intravenous immunoglobulins; qd, daily; qod, every other day; GI, gastrointestinal.

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CME Topic

with plasma exchange is unclear. A retrospective review reported greater benefit of plasma exchange over IVIg in respiratory status at two weeks and functional outcome at one
month; however, a small prospective randomized trial comparing IVIg and plasma exchange found no significant difference in efficacy of treatment between the groups. Both
studies observed a lower incidence of adverse events in the
IVIg-treated patients.18,19 A larger clinical trial will be needed
to settle the debate between the efficacies of plasma exchange
versus IVIg therapy.
The optimal dose of IVIg for MGC is still unclear. A
recent randomized double-blinded study found that a dose of
IVIg of 2 g/kg of ideal body weight (IBW) was not significantly better than 1 g/kg, although there was a trend toward
improvement in the overall muscle strength in the higher dose
group.20 Thus the former dose is preferred (Table 5). Adverse
events occur in less than 10% of patients treated, and may
include headache, fever, chills, myalgias, aseptic meningitis,
and fluid overload. Rarely, patients may develop acute renal
failure, or thromboembolic events such as ischemic stroke,
pulmonary embolism, deep venous thrombosis, or myocardial
infarction.9,21,22 By slowly infusing IVIg, thromboembolic
complications can be limited. Before instituting IVIg, it is important to obtain a serum IgA level, as anaphylaxis may occur in
IgA-deficient patients.23
Once acute therapy has been instituted to treat MGC, the
clinician must anticipate and treat complications that can develop in the mechanically ventilated MG patient. Empiric
antibiotic use is not recommended as some antibiotics can
interfere with neuromuscular transmission. Also, this may
lead to bacterial resistance, as well as foster a Clostridium
difficile infection which has been associated with prolonged
MGC.2,4 The clinician should be alert for cardiac arrhythmias
as an incidence of up to 17% have been reported in patients
with MGC.15,24 The arrhythmias may be relatively benign,
but may include fatal arrhythmias such as ventricular tachycardia, ventricular fibrillation and asystole.3 While the patient
is intubated, specific precautions must be instituted to prevent
ventilator-associated pneumonia. Some of the routine measures include frequent hand-washing, continuous aspiration
of subglottic secretions, avoiding unnecessary manipulations
of the ventilator circuit, closely monitoring gastric residuals
to avoid gastric distention and subsequent aspiration pneumonia. Placing the patient in a semierect position (45 degree
angle) can help prevent the aspiration of upper-airway secretions.25 It is common practice to employ chlorhexidine 0.12%
oral rinse, 15 mL for 30 seconds twice daily to reduce oral
bacterial colonization. Other important measures include using PEEP 5 cm H2O and intermittent ventilator delivered
sighs. Furthermore, the use of intrapulmonary percussion ventilation (IPV) to help loosen secretions has shown benefit in
the pediatric neuromuscular population.26
In addition to preventing pulmonary complications, routine measures to prevent deep venous thrombosis (DVT) usSouthern Medical Journal Volume 101, Number 1, January 2008

ing a combination of sequential compression devices, compression stockings and subcutaneous heparin should be
employed. The use of all three of these measures has been
reported to be more efficacious than using only one or two in
a high risk population for developing DVT.27 Gastrointestinal
prophylaxis with sucralfate or histamine blockers will help
prevent stress ulcers and gastrointestinal bleeding.
Ultimately, the best way to prevent complications from
mechanical ventilation and prolonged ICU stay in MG patients is to work actively to extubate the patient as soon as
possible. Daily assessments of pulmonary function measuring
the FVC, NIF, PEF, should take place to determine respiratory muscle strength. Ideally, the parameters of pulmonary
function should include a FVC 15 mL/kg, NIF 30 cm
H2O, and PEF 40 cm H2O. An important respiratory index
that can help predict successful extubation is the rapid shallow breathing index (RSBI). This is calculated by dividing
tidal volume by respiratory rate. A RSBI of 100 predicts a
95% chance of failure of extubation.28 Other important prerequisites before weaning from the ventilator include adequate oxygenation and an intact respiratory drive and cough
reflex. The hemodynamic status, electrolyte levels, and nutritional status should be normalized. The patient should have
infections actively treated and controlled. The frequency of
suctioning should be no more than every 2 to 3 hours. Swallowing function should be tested to ensure adequate protection of the airway after extubation.
Some MG patients will require tracheostomy and/or percutaneous gastrotomy tubes if a prolonged recovery phase is
anticipated. Generally, after two weeks of mechanical ventilation, it is unlikely the patient will be extubated in the shortterm and a tracheostomy is recommended.2 Tracheostomy
has some benefits including reducing the risk of tracheolaryngeal stenosis, allows for more effective suctioning of tracheal
secretions, and potentially weaning the patient faster from the
ventilator by reducing dead space and resistance to air flow
from the endotracheal tube.2,6
Once MGC abates, the clinician must institute a longterm plan to prevent further exacerbations. Long-term therapies for MG include acetylcholinesterase inhibitors, corticosteroids, and immune-modulating agents. These therapies
either treat the symptoms of MG, or can help prevent future
MG exacerbations. Each medication has unique time to onset
and side effect profiles that must be carefully considered
(Table 5).29
The main acetylcholinesterase inhibitor used for treatment of symptoms of MG is pyridostigmine. This medication
works by preventing the breakdown of acetylcholine at the
neuromuscular junction, thus increasing the availability of
acetylcholine. Although generally well-tolerated, side effects
may occur including gastrointestinal complaints of nausea,
vomiting, diarrhea and abdominal cramps. Muscle twitching,

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Bershad et al Myasthenia Gravis Crisis

fasciculations and cramps may occur. Some patients may

produce excessive and thick saliva. Rarely, bradycardia may
occur.29
Disease modifying agents include corticosteroids and immune-modulators. Prednisone is the mainstay of corticosteroid therapy. It is important to initiate prednisone at a low
dose and gradually titrate upwards, as a large percentage of
patients will have transient worsening of muscle strength in
the first few weeks after initiation. A gradual tapering of
steroids should occur based on the long-term outpatient clinical course.29
Immune-modulatory therapy may help to limit the dose
of steroid therapy. The main immune-modulatory agents used
for MG are azathioprine, cyclosporine, and mycophenolate
mofetil. Generally, cyclosporine works quicker than azathioprine and mycophenolate mofetil, but is generally reserved
for patients with severe MG, not responding to steroids. Azathioprine and mycophenolate mofetil are used to help reduce
steroid requirements.29
In addition to pharmacologic therapy, all patients should
have imaging to assess for thymoma. If a thymoma is found,
resection is mandatory due to the chance of malignant transformation. In addition, a beneficial effect on clinical course
has been observed after resection of a thymoma. In patients
without a thymoma, most MG experts agree based on the
available literature that the thymus gland should be removed
in young healthy patients to increase the chance of remission
or improvement in the future clinical course, although no
good randomized clinical trials exist at this point.29 A practice parameter issued by the American Academy of Neurology recommends thymectomy as an option in patients with
nonthymomatous MG to increase the probability of a remission or improvement in the clinical course.30,31 There is currently an ongoing prospective randomized clinical trial which
will hopefully provide more solid data regarding the efficacy
of thymectomy in patients with nonthymomatous myasthenia
gravis.

Outcome
Overall, the outcome for patients with MGC is good if
therapeutic and supportive measures are instituted effectively.
A large study of MGC patients reports extubation rates of
25%, 50%, and 75% at 7, 13, and 31 days respectively.4 Risk
factors for prolonged intubation include age 50 years, preintubation serum carbon dioxide levels 30 mg/dL, and a
highest vital capacity of 25 mL/kg during the first week of
intubation.4 The median hospitalization time is one month.3
The mortality rate during MGC is about 4 to 8%, a substantial
improvement from the 50% mortality reported in the early
1960s.3 Short-term functional independence is reported to be
correlated with duration of intubation. In one study, 64% of
patients were functionally independent at hospital discharge
if intubated less than two weeks, compared with only 23% if

68

intubated more than 2 weeks.4 About one-third of patients

with MGC will experience a recurrent episode.6

Conclusion
MGC is a neurologic emergency that requires prompt
recognition and treatment. A straight-forward bedside clinical
history and examination using pulmonary function tests can
help with the diagnosis. Effective management with supportive therapy in the ICU setting can help minimize morbidity
and mortality. Triggering factors should be identified and
treated. An active role toward extubating the patient can reduce ICU complications. Short-term treatment with plasma
exchange or IVIg may expedite recovery. Long-term treatment with acetylcholinesterase inhibitors, corticosteroids or
immune-modulating agents to prevent future recurrence should
be initiated as soon as possible.

Acknowledgments
The authors thank Elena Dupont for her assistance with
Figure 1.

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To conquer fear is the beginning of wisdom.

Bertrand Russell

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