Presented by: VIVEK DEV

 Myastheniagravis (MG) is a
neuromuscular disorder characterized by
weakness and fatigability of skeletal
muscles.

 Theunderlying defect is a decrease in the

number of available acetylcholine
receptors (AChRs) at neuromuscular
junctions due to an antibody-mediated
autoimmune attack.
 The hallmark of myasthenia gravis is muscle weakness
that worsen after period of activity and improves after
period of rest.

 As a clinical entity, myasthenia gravis was not

recognized until Samuel Wilks in 1877 described bulbar
and peripheral muscular weakness. In the Lancet of June
2, 1934, the remarkable discovery of physostigmine
treatment by Dr. Mary Walker was published, which was
to become the mainstay of symptomatic treatment.
 Certain muscles such as those that control eye and eyelid
movement, facial expression, chewing, talking, and
swallowing are often (but not always) involved in the
disorder. The muscle that control breathing and neck and
limb movements may also be affected.

 There is no known cure but available treatments can

control the symptoms and often allow people to have a
relatively high quality of life.
 In MG, the receptors at the muscle surface are
destroyed or deformed by antibodies that prevent a
normal muscular reaction from occurring.
 The causative factor is unknown, but the disorder may
have a genetic link.
 The most commonly affected muscles are those of the
EYES, FACE, AND SWALLOWING
 Itcan result in double vision, drooping eyelids,
trouble talking, and trouble walking
 Those affected often have a large thymus gland
or develop a thymoma.
 FACTORS THAT CAN WORSEN
MYASTHENIA GRAVIS
 Fatigue
 Illness
 Stress
 Extremeheat
 Some medications — such as BETA
BLOCKERS, CALCIUM CHANNEL
BLOCKERS, QUININE, & ANTIBIOTICS
 MG is rare, having a prevalence of 2–7 in 10,000
 MYASTHENIA GRAVIS is most common in the
people at the age of 40 and above
 This mainly affects the older people and the middle
aged people.
 There are more women than men and the age of the
participants ranged from 40to 82.
 The prevalence of this disease is found to be more in
China (64,942) followed by India (53,253) and is
found to be least in Bhutan (109).
THE FOLLOWING TABLE ATTEMPTS TO EXTRAPOLATE THE
PREVALENCE
RATE FOR MYASTHENIA GRAVIS TO THE POPULATIONS OF
VARIOUS
COUNTRIES AND REGIONS IN ASIA:
 In MG, antibodies are directed toward the
ACETYLCHOLINE RECEPTOR at the
neuromuscular junction of the skeletal muscle
RESULTING IN:
 Decreased number of acetylcholine receptors at the
post synaptic muscle membrane
 Postsynaptic folds are simplified or flattened
 Decreased neuromuscular transmission
 Weakness of muscle contraction
 Therefore,although Ach is released normally, it
produces small end-plate potentials that may fail to
trigger muscle action potentials.
 NEUROMUSCULAR
 Failure of transmission at many junctions
results in weakness of muscle contraction
 The amount of Ach released per impulse
normally declines on repeated activity
(termed presynaptic rundown).
 PRESYNAPTIC RUNDOWN
DECREASED
EFFICIENCY
OF NMT
MUSCLE
MYASTHENIA WEAKNESS
GRAVIS

NORMAL
PRESYNAPTIC
RUNDOWN
 An immune response to muscle-specific kinase (musk),
a protein involved in achR clustering at
neuromuscular junctions, can also result in myasthenia
gravis, with reduction of achRs demonstrated
experimentally.

 The pathogenic antibodies are igG, and are t cell

dependent
 Immunotherapeutic strategies directed against either the
antibody-producing B CELLS or helper T CELLS
are effective in this antibody-mediated disease.
 The autoimmune response is initiated and maintained in
mg is not completely understood, but the thymus
appears to play a role in this process.
 The thymus is abnormal in ∼75% of patients with MG;
in ∼65% the THYMUS is “hyperplastic,” with the
presence of active germinal centers detected
histologically, though the hyperplastic thymus is not
necessarily enlarged.

 An additional 10% of patients have thymic tumors

(THYMOMAS)
 Muscle-like cells within the thymus (MYOID
CELLS),
*Bear AChRs on their surface
*May serve as a source of autoantigen and
trigger the autoimmune reaction within the
thymus gland.
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.
 Myasthenia gravis may be inherited as a rare, genetic
disease, acquired by babies born to mothers with MG
 Nerves communicate with the muscles by releasing
chemicals, called NEUROTRANSMITTERS.
 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.
 Themain symptom of myasthenia gravis is WEAKNESS
IN THE VOLUNTARY SKELETAL MUSCLES,
which are muscles under our control.

 The failure of muscles to contract normally occurs

because they can’t respond to nerve impulses. Without
proper transmission of the impulse, a blocked
communication occurs between nerve and muscle and
weakness results.
Weakness associated with myasthenia
gravis typically gets worse with more
activity and improves with rest. Some
of the main symptoms to be noticed are:
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)
 Blurred vision, which may come and go
In about percent of people
15 gravis,
with the first
myasthenia
involve face and
symptoms
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.
› CHEWING. The muscles used for chewing may
wear out halfway through a meal, particularly if
eating.
› FACIAL EXPRESSIONS. Family members may
note "lost smile" if the muscles that control facial
expressions are affected
 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

If having trouble with:
 BREATHING
 SEEING
 SWALLOWING
 CHEWING
 WALKING
 HISTORY
 Diplopia
 Ptosis
 Weakness in characteristic distribution
 Fluctuation and fatigue: worse with repeated
activity,
 Improved by rest effects of previous
treatments
PHYSICAL EXAMINATION
 Ptosis
 Diplopia
 Motor power survey: quantitative testing of
muscle strength
 Forward arm abduction time(5 min)
 Vital capacity, Absence of other neurologic signs
 EDROPHONIUM TEST:

Injection of the chemical edrophonium (Tensilon) may result

in a sudden, although temporary, improvement in muscle
strength — an indication that 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.

 IMAGING SCANS: CT scan or an MRI to confirm

a tumor or other abnormality in thymus.
 • SINGLE FIBER-EMG

 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.

• CT CHEST
A. THERAPEUTIC MANAGEMENT:

 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)
 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, but
it can take a week or two to start working and the benefits
usually last less than a month or two.
 THYMECTOMY
 surgical removal of the
thymus gland. The role of
the thymus gland in MG is
fully not understood, and the
thymectomy may or may not
improve child’s symptoms
Because the cause of Myasthenia Gravis is unknown,
there is no way to prevent it. However, once the disease has
developed, there may be ways to prevent episodes of
worsening symptoms or flare-ups:

 Give yourself plenty of rest.

 Avoid strenuous, exhausting activities.
 Avoid excessive heat and cold.
 Avoid emotional stress.
 Whenever possible, avoid exposure to any kind of
infection, including colds and influenza (flu). You
should be vaccinated against common infections, such
as influenza.

 Work with your doctor to monitor your reactions to

prescription medications. Some drugs commonly
prescribed for other problems, such as infections, heart
disease or hypertension, may make myasthenia gravis
worse. You may need to choose alternative therapies or
avoid some medications entirely.