In Practice

Myasthenia Gravis in Pregnancy

CHERYL K. ROTH
SARAH DENT

I
Imagine that youre caring for a 34-year-old pri-
migravida at 36 4/7 weeks gestation for preterm
premature rupture of membranes (PPROM) and
breech presentation. She was diagnosed with
myasthenia gravis (MG) 3 years prior to her
pregnancy. She is medicated with pyridostig-
mine, prednisone and azathioprine to treat her
disease at this time. Symptoms initially pro-
gressed in her pregnancy, but disappeared com-
KYLE MCDEVITT
persistent nonproductive cough. Her lungs are
clear to auscultation bilaterally.
Regional spinal anesthesia is performed
without incident. She is in the obstetric postan-
esthesia care unit (PACU) post cesarean surgi-
cal birth. Her first hour in the PACU is unevent-
ful and she and her baby are showing no signs
of muscle weakness. Maternal vital signs are
Photo iStock Collection / thinkstockphotos.com

pletely in the second trimester after adjusting 122/78, 78, 18 with oxygen saturation 99 percent
her medications. She reports an upper respira- on room air.
tory infection over the past 3 to 4 days with a During the second hour of recovery, her

Abstract Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disease and is the most common disorder of
neuromuscular transmission. MG is caused by a defect in the transmission of nerve impulses to muscles in which communi-
cation from nerves to muscles is interrupted at the neuromuscular junction. This interruption can cause significant impact
to muscle functions, which can have serious consequences for a pregnant woman, especially during labor. This brief article,
which is meant to be used as an easy-reference tool in the clinical setting, examines the disease process and its eff ect on the
antepartum, intrapartum and postpartum periods. DOI: 10.1111/1751-486X.12206
Keywords autoimmune disorder | myasthenia gravis | neuromuscular | pregnancy

248 2015, AWHONN http://nwh.awhonn.org

oxygen saturation levels suddenly drop into the
70s and 80s. She is reporting marked shortness
of breath, stating, I cant breathe! cent present with proximal limb weakness alone
What Is MG?
MG is a chronic autoimmune neuromuscular dis-
ease, and is the most common disorder of neu-
romuscular transmission. Two-thirds of affected
individuals are female, with common onset in the
second and third decade of life (Bird, Stafford, &
Dildy, 2014). Its reported to have an incidence
from one in 10,000 to one in 50,000 among wom-
en of reproductive age (Varner, 2013).
MG is caused by a defect to the transmission
of nerve impulses to muscles. The communica-
tion from the nerve to the muscle is interrupted
at the neuromuscular junction. Normally, when
impulses travel down a nerve, acetylcholine is
released; it binds with acetylcholine receptors,
generating muscle contraction. In MG, anti-
bodies that are produced by the bodys immune
system block, alter or destroy the receptors for
acetylcholine at the neuromuscular junction
(National Institute of Neurological Disorders
and Stroke [NINDS], 2014). This prevents mus-
cle contraction from occurring.
Some people with MG develop thymomas
(tumors of the thymus gland). These thymomas
are generally benign, but they can become ma-
lignant (Wheatley-Price, Jonker, Jonker, Shamji,
& Gomes, 2014). The relationship between the
thymus gland and MG isnt yet fully under-
stood. Its believed that the thymus gland may
give incorrect instructions to developing im-
mune cells, resulting in autoimmunity and the
production of the acetylcholine receptor anti-
bodies (NINDS, 2014). The production of these
antibodies sets the stage for the attack on neuro-
muscular transmission seen in MG.
Symptoms
MG is characterized by varying degrees of
weakness to the muscles of the body, and most
commonly affects the muscles that control the
eyes, facial expressions, chewing, talking and
swallowing. The disease can also affect mus-
cles associated with breathing and neck and
limb movement (Tllez-Zenteno, Hernndez-
Ronquillo, Salinas, Estanol, & da Silva, 2004).
Approximately 50 percent of people with MG
present with ocular symptoms of ptosis and/or
diplopia. About 15 percent present with bulbar
June | July 2015
symptoms, which include dysarthria, dyspha-
In Practice
gia and fatigable chewing. Fewer than 5 per-
(Bird et al., 2014).
A myasthenic crisis occurs when muscles
that control breathing weaken to the point that
ventilation is inadequate, creating a medical
emergency and requiring assisted ventilation.
In individuals whose respiratory muscles are af-
fected by their disease, a crisis may be triggered
by infection, fever, surgery, emotional stress or
an adverse reaction to medication.
Treatment
There are several therapies used to reduce and
improve the muscle weakness associated with
MG. Medications used to treat the disease in-
clude acetylcholinesterase inhibitors, such as
neostigmine (Prostigmin, Vagostigmin) and
MG is characterized by varying degrees
of weakness to the muscles of the body,
and most commonly affects the muscles
that control the eyes, facial expressions,
chewing, talking and swallowing
pyridostigmine (generic only). These drugs help
improve muscle weakness and strength and are
the first-line treatment for MG. Immunosup-
pressive glucocorticoid drugs, such as predni-
sone, azathioprine and cyclosporine (all generic
only), may also be used, which improve strength
by suppressing the production of antibodies.
Thymectomy, the surgical removal of the
thymus gland, can reduce symptoms and may
cure up to 50 percent of people with the condi-
tion. Thymectomy is recommended for patients
with thymoma.
Other therapies used to treat MG include
plasmapheresis, a procedure in which the ab-
normal antibodies are removed from the blood,
and high-dose intravenous immune globulin,
which temporarily modifies the immune sys-
tem. This therapy may be used to help individu-
als during especially difficult periods of weak-
ness (NINDS, 2014).
Antepartum Considerations
Antepartum considerations include the
following:
Nursing for Womens Health 249
 In Practice
The first trimester and the immediate
postpartum period are the most common
times for MG symptom exacerbation
Cheryl K. Roth, PhD,
WHNP-BC, RNC-OB,
RNFA, is a nurse practi-
tioner at Honor Health
Scottsdale Shea Medical
Center in Scottsdale, AZ.
Sarah Dent, MSN, RNC-
OB, is a clinical director at
Honor Health Scottsdale
Shea Medical Center
in Scottsdale, AZ. Kyle
McDevitt, MSN, RN, is a
student at Arizona State
University in Phoenix,
AZ. The authors report
no conflicts of interest or
relevant financial relation-
ships. Address correspon-
dence to: cheryl.roth@
honorhealth.com.
250 Nursing for Womens Health
Preconception planning is important. Preg-
nancy is not recommended in the first 2
years following diagnosis, as maternal mor-
bidity is higher during this period (Kalidindi
et al., 2007).
Women with MG should have an informed
discussion with their health care provider
regarding the medical management of their
disease throughout their pregnancy and
should understand the fetal and maternal
risks.
During pregnancy, two-thirds of women
with MG experience no changes to the clini-
cal course of their disease, while one-third
have an exacerbation of their disease. The
first trimester and the immediate postpar-
tum period are the most common times for
MG symptom exacerbation. Symptoms im-
prove in the second and third trimester in 20
percent to 40 percent of patients. Complete
remission can occur in some people.
Acetylcholinesterase inhibitor dose adjust-
ment may be required in pregnancy as a
result of increased renal clearance, expanded
maternal blood volume and delayed gastric
emptying. Glucocorticoids may be used if
anticholinesterases dont control symptoms.
Glucocorticoid medications have been well-
studied in pregnancy, and have been found
to be relatively safe. However, high doses of
cyclosporine and azathioprine have been
linked to spontaneous abortion, preterm la-
bor, low birth weight, chromosomal damage
and hematologic suppression. The risk of us-
ing these medications in pregnancy should
be weighed against the benefit of controlling
myasthenic symptoms (Bird et al., 2014).
The benefit versus risk of intravenous immu-
noglobulins in pregnancy must be consid-
ered. These interventions should be reserved
for cases in which other therapies have failed
and respiratory failure or profound dyspha-
Photos iStock Collection / thinkstockphotos.com
gia and weakness threatens a mother and
fetus (Ellison, Thomson, Walker, & Greer,
2000).
Diligent assessment and prompt treatment
of infections in pregnant women with MG
is indicated, as infection may precipitate a
flare. In one study of 65 pregnant women
with MG, four women had infection (pyelo-
nephritis, endometritis or mastitis) and all
developed exacerbations (Djelmis, Sostarko,
Volume 19 Issue 3
 Mayer, &Ivanisevic, 2002). Complaints of
dyspnea or cough call for prompt evalua-
tion for possible myasthenic flare with dia-
phragm and respiratory muscle weakness
(Stafford & Dildy, 2005).
An anesthesia consult is recommended be-
fore labor and birth as the plan of care must
be individualized. Care will need to be indi-
vidualized based on a womans disease his-
tory. Regional anesthesia is recommended
for mild to moderate disease when vaginal
birth is anticipated. General anesthesia is
recommended for women with severe dis-
ease (Bird et al., 2014).
Intrapartum Considerations
Intrapartum considerations include the
following:
Magnesium sulfate is contraindicated for
women with MG since it can precipitate a
severe crisis (Bird et al., 2014). Severe hyper-
tension can be treated with methyldopa or
hydralazine, while calcium channel blockers
and beta blockers, such as labetalol, should
be avoided if possible. Seizure prophylax-
is may need to be managed in coordination
with neurology providers.
Continuous fetal monitoring is indicated
during a myasthenic crisis if the fetus is at
a viable gestational age. Birth during a my-
asthenic crisis is not optimal, as its an addi-
tional stressor for the woman. The decision
to initiate birth must be based on the total
obstetric picture.
The first stage of labor is not affected be-
cause the uterus is made of smooth muscle,
which doesnt have the acetylcholine recep-
tors affected by MG. The second stage of
labor may be affected because the muscles
used during pushing may easily weaken
(Chaudhry, Vignarajah, & Koren, 2012). An
instrument-assisted birth should be consid-
ered to reduce the effects of maternal fatigue
on birth. Stress and exertion can precipi-
tate a myasthenic crisis. Because the uterus
is made of smooth muscle and not affected
by the presence of acetylcholine receptor an-
tibodies, theres not a higher risk of uterine
atony during crisis, but surgery can worsen
a myasthenic crisis (Chaudhry et al., 2012).
Decisions regarding cesarean surgical birth
June | July 2015
The first stage of labor is not affected
In Practice
because the uterus is made of smooth
muscle, which doesnt have the
acetylcholine receptors affected by MG
during a crisis must be individualized based
on benefit versus risk.
Sedatives, opioids and tranquilizers can ex-
acerbate respiratory depression and should
be used with caution (Berlit, Tuschy, Spaich,
Suttenlin, & Schaffelder, 2012). These might
include morphine and other anesthetic
agents (Hopkins, Alshaeri, Akst, & Berger,
2014).
Mortality risk is highest risk in the first year,
with risk decreasing after 7 years (Chaudhry
et al., 2012).
Fetal Considerations
Fetal considerations include the following:
Fetal risks include neonatal MG, prematu-
rity, severe malformation and death (Bird et
al., 2014).
The development of fetal abnormalities re-
lated to transplacental passage of antiacetyl-
choline antibodies is a major concern dur-
ing pregnancy. This can occur in 10 percent
to 20 percent of pregnancies (Varner, 2013).
Abnormal sonographic findings in affected
pregnancies may include polyhydramnios
due to impaired fetal swallowing.
Symptoms of respiratory distress, poor feed-
ing and flaccid tone usually appear within 48
hours of birth and may be ongoing for up to
3 months. All infants born to women with
MG should be observed in a special care
nursery for the first 48 to 72 hours of life.
In subsequent pregnancies the risk of recur-
rence of neonatal MG is approximately 75
percent (Bird et al., 2014).
Postpartum Considerations
Postpartum considerations include the
following:
Breastfeeding isnt contraindicated in wom-
en with MG. Glucocorticoids can be used
safely in lactation, but breastfeeding isnt
Nursing for Womens Health 251

Fetal risks include neonatal MG, prematurity,
severe malformation and death
recommended for women tak-
ing azathioprine, cyclosporine and
methotrexate.
The Rest of the Story
You quickly called for help, anesthe-
sia arrived to the room immediately
and intubated the woman, performing
bag and mask ventilation until she was
transferred to the intensive care unit for
mechanical ventilation. She was treated
with pyridostigmine, azathioprine and
corticosteroids and received intrave-
nous immunoglobulin and five treat-
ments of plasmapheresis. After 2 days
she was extubated and transferred to
252 Nursing for Womens Health
postpartum, and was discharged home
in good clinical condition on the fifth
postpartum day. NWH
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Volume 19 Issue 3