INTRODUCTION

Myasthenia gravis (MG) is a complex, autoimmune

disorder in which antibodies destroy neuromuscular
connections.

Causes problems with the nerves that communicate

with muscles.

Affects the voluntary muscles of the body, especially

the eyes, mouth, throat, and limbs.
Characterized by weakness and rapid fatigue of any
of the muscles under the voluntary control.

The cause of myasthenia gravis is a breakdown in

the normal communication between nerves and
muscles.

No cure for myasthenia gravis, but treatment can

help relieve signs and symptoms – such as
weakness of arm or leg muscles, double vision,
drooping eyelids, and difficulties with speech,
chewing, swallowing and breathing.
 TYPES OF MYASTHENIA GRAVIS
Three types of MG in children:
Congenital MG - Very rare non-immune form of MG that is
inherited as an autosomal recessive disease.
Symptoms of congenital MG usually begin in the baby's first
year and are life-long.
Transient neonatal MG - Between 10 and 20 percent of
babies born to mothers with MG may have a temporary form
of MG.
Neonatal MG usually lasts only a few weeks, and babies are
not at greater risk for developing MG later in life.
Juvenile MG - This auto-immune disorder develops typically
in female adolescents.
 It is a life-long condition that may go in and out of remission.
About 10 percent of MG cases are juvenile-onset.
 SYMPTOMS
Babies with neonatal MG may be weak, with a poor suck,
and may have respiratory difficulty. A few babies may need
the help of a mechanical breathing machine if their
respiratory muscles are too weak to breathe on their own.

Congenital MG symptoms may begin in the first year, with

generalized weakness in the arms and legs, and delays in
motor skills such as crawling, sitting, and walking

Juvenile MG symptoms may begin gradually over weeks or

months. The child may become excessively tired after very
little activity, and begin to have problems chewing and
swallowing. Drooping eyelids may be so severe that the child
cannot see.
 Eye muscles
In more than half the people who develop MG, their
first signs and symptoms involve eye problems:
Drooping of one or both eyelids (ptosis)
Double vision (diplopia), which may be horizontal or
vertical
Blurred vision, which may come and go
 Face and throat muscles
In about 15 percent of people with myasthenia gravis, the first
symptoms involve face and throat muscles, which can cause
difficulties with:
Speaking. The speech may be very soft or sound nasal,
depending upon which muscles have been affected.
Swallowing. May choke very easily, which makes it difficult
to eat, drink or take pills. In some cases, liquids may come
out of the nose.
Chewing. The muscles used for chewing may wear out
halfway through a meal, particularly if eating
something hard to chew, such as sugarcane.
Facial expressions. Family members may
note "lost smile" if the muscles that control
facial expressions are affected.
 Arm and leg muscles
Myasthenia gravis can cause weakness in arms and
legs, but this usually happens in conjunction with
muscle weakness in other parts of the body – such
as eyes, face or throat.
The disorder usually affects arms more often than
legs.
If it affects legs, may waddle when walking.

Normal dumbbell Weakness dumbbell

 When to see a doctor
If having trouble with:
Breathing
Seeing
Swallowing
Chewing
Walking
 CAUSES
Myasthenia gravis may be inherited, genetic
disease, acquired by babies born to mothers with
MG
Nerves communicate with the muscles by releasing
chemicals, called neurotransmitters, which fit
precisely into receptor sites on the muscle cells.
In myasthenia gravis, immune system produces
antibodies that block or destroy many of the
muscles' receptor sites for a neurotransmitter called
acetylcholine.
With fewer receptor sites available, muscles receive
fewer nerve signals, resulting in weakness.
 Chemicals messengers, called neurotransmitters, fit
precisely into receptor sites on your muscle cells. In
myasthenia gravis, certain receptor sites are blocked
or destroyed, causing muscle weakness.
 It's believed that the thymus gland, a part of the
immune system located in the upper chest beneath
the breastbone, may trigger or maintain the
production of these antibodies.
Large in infancy, the thymus is small in healthy
adults. But, in some adults with myasthenia gravis,
the thymus is abnormally large.
Some people also have tumors of the thymus.
Usually, thymus gland tumors are noncancerous.
Thymus gland, a part of your immune system located
in the upper chest beneath the breastbone, may
trigger or maintain the production of antibodies that
result in the muscle weakness common in MG.
Factors worsening MG
Fatigue
Illness
Stress
Extreme heat
Medications – such as beta blockers, calcium

channel blockers, quinine and some antibiotics

 COMPLICATIONS
Myasthenic crisis: A life-threatening condition,
which occurs when the muscles that control
breathing become too weak to do their jobs.
Emergency treatment is needed to provide
mechanical assistance with breathing.

Thymus tumors: About 15 percent of the people

who have myasthenia gravis have a tumor in their
thymus, a gland under the breastbone that is
involved with the immune system. Most of these
tumors are noncancerous.
 Other disorders
Underactive or overactive thyroid. The thyroid
gland, located in the neck, secretes hormones that
regulate metabolism. If thyroid is underactive, body
uses energy more slowly. An overactive thyroid
makes body use energy too quickly.
Lupus. Disease of immune system. Common
symptoms include painful or swollen joints, hair loss,
extreme fatigue and a red rash on the face.
Rheumatoid arthritis. Caused by problems with
immune system. It is most conspicuous in the wrists
and fingers, and can result in joint deformities that
make it difficult to use hands.
 Diagnostic tests
Blood tests
Genetic tests - diagnostic tests that evaluate for
conditions that have a tendency to run in families.
Electromyogram (EMG) - a test that measures the
electrical activity of a muscle or a group of muscles.
An EMG can detect abnormal electrical muscle
activity due to diseases and neuromuscular
conditions.
Muscle biopsy - a small sample of the muscle is
removed and examined to determine and confirm a
diagnosis or condition.
Reflexes
Muscle strength
Muscle tone
Senses of touch and sight
Coordination
Balance
Edrophonium test: Injection of the chemical
edrophonium (Tensilon) may result in a sudden,
although temporary, improvement in muscle
strength — an indication that you may have
myasthenia gravis. Edrophonium acts to block an
enzyme that breaks down acetylcholine, the
chemical that transmits signals from nerve endings
to muscle receptor sites.
Blood analysis: A blood test may reveal the
presence of abnormal antibodies that disrupt the
receptor sites where nerve impulses signal muscles
to move.
 Repetitive nerve stimulation: Is a type of nerve
conduction study, in which electrodes are attached
to skin over the muscles to be tested. To diagnose
MG, the nerve will be tested many times to see if its
ability to send signals worsens with fatigue.
Single-fiber electromyography (EMG): EMG
measures the electrical activity traveling between
brain and muscle. It involves inserting a very fine
wire electrode through skin and into a muscle. In
single-fiber EMGs, a single muscle fiber is tested.
Imaging scans: CT scan or an MRI to confirm a
tumor or other abnormality in thymus.
 TREATMENTS & DRUGS
Specific treatment to age, overall health, and
medical history and extent of the condition
No cure for MG, but the symptoms can be
controlled.
MG is a life-long medical condition and the key to
medically managing MG is early detection.
The goal of treatment is to prevent respiratory
problems and provide adequate nutritional care to
the child since the swallowing and breathing
muscles are affected by this condition.
 Medications
Cholinesterase inhibitors. Drugs like
pyridostigmine (Mestinon) enhance communication
between nerves and muscles. These drugs don't
cure, but improves muscle contraction and strength.
Corticosteroids. These types of drugs inhibit the
immune system, limiting antibody production.
Prolonged use of corticosteroids, can lead to serious
side effects, like bone thinning, weight gain,
diabetes, increased risk of some infections, and
increase and redistribution of body fat.
Immunosuppressants. Doctor may also prescribe
other medications that alter immune system, like
azathioprine (Imuran), cyclosporine (Sandimmune,
Neoral) or mycophenolate (CellCept).
 How antibodies against
acetylcholine receptor
block impulse conduction

in synapse
 Therapy
Plasmapheresis. This procedure uses a filtering
process similar to dialysis. Blood is routed through a
machine that removes the antibodies that are
blocking transmission of signals from nerve endings
to muscles' receptor sites. However, the beneficial
effects usually last only a few weeks.
Intravenous immune globulin. This therapy
provides body with normal antibodies, which alters
immune system response. It has a lower risk of side
effects than do plasmapheresis and immune-
suppressing therapy, but it can take a week or two to
start working and the benefits usually last less than
a month or two.
 Surgery
Thymectomy - surgical removal of the thymus
gland. The role of the thymus gland in MG is not
fully understood, and the thymectomy may or may
not improve a child's symptoms.
Plasmapheresis - a procedure that removes
abnormal antibodies from the blood and replaces
the child's blood with normal antibodies through
donated blood.
Extent of the problems is dependent on the severity
of the condition and the presence of other problems
that could affect the child.
 In severe cases, a breathing machine may be
required to help the child breathe easier.
It is important to allow the child as much
independent function and self care, especially with
juvenile MG, as possible and to promote age-
appropriate activities to ensure a sense of normalcy.
About 15 percent of the people who have MG have
a tumor in their thymus
For people with MG who don't have a tumor in the
thymus, it's unclear whether the potential benefit of
removing the thymus outweighs the risks of surgery.
This is an individualized decision between patient
and the doctor, but most doctors don't recommend
surgery if:
Symptoms are mild
Symptoms involve only the eyes
Patients over 60 years old
 Nutrition
Along with exercises & breathing practices eating
habits should also be altered.
Simple, nourishing, no stimulating foods, including
plenty of fresh fruits & lightly cooked vegetable,
particularly greens.
Asparagus is considered excellent since it contains
certain natural steroid-like nutritious elements, which
help strengthen the weakened muscles caused by
MG.
Whole meal grains, sprouts & pulses in places eggs
and meats.
Food should have a blend of all necessary vitamins.