Dr.

Shamanthakamani Narendran

M.D. (Pead), Ph.D. (Yoga Science)

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.
While myasthenia gravis can affect people of any
age from neonatal to above 60 and more common in
women younger than 40 and in men older than 60.

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. This means that
both males and females are equally affected and that two copies
of the gene, one inherited from each parent, are necessary to
have the condition. 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.
This occurs when antibodies common in MG cross the placenta
to the developing fetus. 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 - especially Caucasian females. It is a lifelong 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. Symptoms go
away as the maternal antibodies disappear over time.
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. Babies
may have difficulty feeding and may have weak eyelids and
poor head control.
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 as a rare,
genetic disease, acquired by babies born to mothers
with MG, or the disorder may develop spontaneously
later in childhood.
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. Medications
and blood-filtering therapies help people recover
from myasthenic crisis, so they can again breathe
on their own.
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.

TESTS AND DIAGNOSIS

Diagnosis is made after the sudden or gradual onset
of specific symptoms and after diagnostic testing.
It is confirmed with a Tensilon test. With this test, a
small amount of medicine (Tensilon) is injected into
the child, if the child has MG, an immediate, but
brief, increase in muscle tone is noted.

Other 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. Small pulses of
electricity are sent through the electrodes to measure
the nerve's ability to send a signal to muscle. 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.

CT Chest
Thymom
a

Single-fiber EMG

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 immunesuppressing 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.
The healthcare team educates the family after
hospitalization on how to best care for their child at
home and outlines specific clinical problems that
require immediate medical attention by their
physician.
A child with MG requires frequent medical
evaluations throughout his/her life.
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, a gland under the breastbone that
is involved with the immune system, thymus will be
removed.
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

Myasthenia Gravis through yoga

Yoga Exercises
Help to stretch the body which increase the flexibility
as well as strengthen the muscles of the body, which
become weak due to MG.
Improve blood circulation and remove carbondioxide
from the body & provide oxygenated blood to every
part of the body.

Pranayama yoga breathing

Helpful because the onset of MG often lead to mild
depression but the practice of breathing exercises
like,
Nadi- Shodhan,
Anulom-Vilom,
Deep breathing and
Mild Kapalbhati
Helps to remove depression and relax the mind as
well as the body.
Meditation also brings more positive thinking.

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.

Research Study in NIMH

Background Juvenile myasthenia gravis (JMG) is
an uncommon disease. Unlike adults, clinical
characteristics and outcomes of myasthenia gravis
(MG) are not well studied in children.
Patients and methods Case records of 77
patients with MG who were 15 years of age or less
at disease onset, evaluated over a period of 34
years at the National Institute of Mental Health and
Neurosciences, Bangalore, India, were reviewed.
Their clinical characteristics and response to therapy
was compared with 290 patients with MG onset after
15 years of age.

 Results Median age at onset was 8 years and mean period of follow-up
was 6.2 years (range 6 months to 25 years).
At presentation, 30% of patients had ocular myasthenia and the rest had
generalized disease.
Twenty-one patients (27%) had disease confined to ocular muscles
throughout the course and three had limb girdle myasthenia.
Familial myasthenia was more common than adult onset disease, 10
patients had positive family history.
Unlike adults, none of the patients had associated autoimmune disease.
Fifty-two patients (67%) received corticosteroids, and azathioprine was
added in five patients.
Thymectomy was performed in 11 patients, six below the age of 15 years.
Thymic histology was normal in one and showed hyperplasia in eight and
thymoma in one.
Four patients had crisis.
At the end of follow-up, 25 patients were asymptomatic, 28 had partial
improvement, and nine remained unchanged or worsened and two died.
Ten patients achieved complete stable remission.

 Conclusions This study shows some distinctive

characteristics of JMG, such as higher frequency of
ocular myasthenia, benign course, better long-term
outcome and lack of association of thymoma and
other autoimmune disorders.