PRESENTATION ON

MYASTHENIA
GRAVIS
PRESENTED BY:
SANDHYA HARBOLA

PCNMS
FUNCTIONING OF NEUROMUSCULAR JUNCTION

In neuromuscular junction acetylcholine is stored in

vesicles. Released on action potential

Combines with AchRs at post synaptic membrane folds.

Rapid influx of cations sodium.

Depolarization at the end plate region of muscle fiber-If

sufficiently large-muscle contraction

ACH is rapidly hydrolysed by AchE present in synaptic

folds
 BACKGROUND

 Myasthenia gravis was first described by Thomas Willis in

1672.
 An uncommon chronic autoimmune disorder, literally meaning
‘muscle weakness.
 Prevalence of MG is about 20 per 100,000 population in U.S.A.
 A neuromuscular disease, defect in the transmission of nerve
impulse.
 Women are affected more than men.
 DEFINITION
 Myasthenia gravis is an autoimmune disorder affecting the
neuromuscular junction, is characterized by varying degrees of
weakness of the voluntary muscle.
CAUSES

 Autoantibodies block the receptors of acetylcholine in

neuromuscular junction.
 Autoantibodies against Musk protein- tyrosine kinase
receptor which helps in neuromuscular junction formation.
 Thymoma- enlargement of thymus.
 Rare hereditary form of myasthenia gravis.
FACTORS THAT CAN WORSEN
MYASTHENIA GRAVIS
 Fatigue
 Illness
 Stress
 Extreme heat
 Some medications — such as beta blockers, calcium
channel blockers, quinine and some antibiotics
PATHOPHYSIOLOGY
Due to autoimmune response

Development of auto antibodies

Antibody attacks acetylcholine receptor

Act against nicotinic acetylcholine receptor

Impair the ability of acetylcholine to bind with

acetylcholine receptor

Resulting in voluntary muscle weakness that escalated

with continue activity
CLINICAL MANIFESTATIONS

 Eye muscles:
Drooping of one or both eyelids (ptosis).
Double vision (diplopia)

 Neck and limb muscles:

Weakness in arms, legs, neck, fingers etc.
 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.
 Chewing: The muscles used
for chewing may wear out halfw
-ay through a meal, particularly if
eating.
 Facial expressions: Family mem
-bers may note "lost smile" if the
muscles that control facial expressions
are affected.
 Respiratory muscle weakness
 Diagnosis
 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
Forward arm abduction time(5 min), Vital capacity,
Absence of other neurologic signs.
 LABORATORY INVESTIGATIONS
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.
 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.
 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.
 MEDICAL

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.
 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.
 NURSING DIAGNOSIS
ion-

1) Ineffective breathing pattern related to neuromuscular

weakness of the respiratory muscle as evidence by spo2
monitoring.
Goal- the patient will maintain normal oxygen saturation.
Intervention-
 Assess respiratory pattern of the patient.
 Provide fowler position.
 Provide oxygen therapy.
2)self care deficit related to muscle weakness as evidence by
observation.
Goal- the client will able to perform his ADL activity.
Intervention-
 Observe the patient’s ability to perform activities of daily living.
 Encourage patient to perform his ADL activity.
 Allow enough time or tsk performance.
 Assess the patient in performing self care task.
3) Imbalancednutrition less than body requirement related to
dysphagia as evidence by weight monitoring.
Goal- to improve patient’s nutrition level.
Intervention-
 Assess the condition of the patient.
 Provide liquid diet to patient.
 Encourage patient to tale small but frequent diet.
 COMPLICATION
 Myasthenia Crisis
This sudden onset of muscle weakness is usually the result of
under medication or no cholinergic medication at all. Myasthenia
crisis may result from progression of the disease, emotional
upset, systemic infections, medications, surgery, or trauma. The
crisis is manifested by sudden onset of acute respiratory distress
and inability to swallow or speak.
 Contd….

 Cholinergic Crisis
Caused by overmedication with cholinergic or anticholinesterase
drugs, cholinergic crisis produces muscle weakness and the
respiratory depression of myasthenia crisis as well as
gastrointestinal symptoms (nausea, vomiting, diarrhoea),
sweating, increased salivation, and bradycardia.
PREVENTION
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.
 RESEARCH ARTICLE

 Title: Physical exercise in myasthenia gravis is safe and improves

neuromuscular parameters and physical performance-based measures: A
pilot study.
 Author : Elisabet Westerberg, Carl Johan Molin, Ida Lindblad,
Margareta Emtner, Anna Rostedt Punga.
 Methods: In this prospective pilot study, 10 MG patients with mild
disease performed supervised aerobic and resistance training twice
weekly for 12 weeks. The Myasthenia Gravis Composite (MGC) score,
compound motor action potential (CMAP), repetitive nerve stimulation,
muscle force, physical performance-based measures, serum levels of
interleukin-6, muscle enzymes, and immuno-microRNAs miR-150-5p
and miR-21-5p were assessed before and after the training period.
 Results: Physical exercise was well tolerated, and the MGC score
was unchanged. Muscle resistance weights and CMAP amplitudes
increased for biceps brachii and rectus femoris muscles, and
physical performance-based measures improved. Muscle enzymes
remained normal, whereas disease-specific microRNAs miR-150-5p
and miR-21-5p were reduced after the training period.
 Conclusions: We propose that general recommendations regarding
physical exercise can be applied safely to well-regulated MG
patients.
 CONCLUSION

 An autoimmune neuromuscular disorder caused by auto antibodies

against AchR .
 Myasthenia gravis is an immune-mediated post-synaptic disorder of
neuromuscular transmission, most commonly presenting as
oculobulbar and proximal muscle weakness associated with easy
fatigability.
 The typical main symptom is weakness of muscles that gets worse
with activity and improves with rest.
 Once diagnosed, proper treatment is necessary for living a normal
life.
 REFERENCES

 Suddarth's and Brunner. Textbook of medical surgical nursing: 13th ed. Vol 2.
Wolters Kluwer India pvt ltd. PP 2026-28.
 www.slideshare.com
 Westerberg E, Molin CJ, Lindblad I, Emtner M, Punga AR. Physical exercise
in myasthenia gravis is safe and improves neuromuscular parameters and
physical performance-based measures: A pilot study. Muscle Nerve.2017
Aug;56(2):207-214.doi:10.1002/mus.25493.Epub2017Apr 2.Availabe at
https://pubmed.ncbi.nlm.nih.gov/27935072/