You are on page 1of 70

KING GEORGE’S MEDICAL UNIVERSITY

K.G.M.U. COLLEGE OF NURSING

MYASTHENIA GRAVIS

PRESENTED BY-
DIVYA PAL
M.Sc. NURSING I YEAR
OBJECTIVES

• Introduction • Sign and symptoms


• Definition
• Diagnostic Evaluation
• Incidence
• Medical Management
• Risk factors
• Surgical Management
• Causes
• Nursing Management
• Types
• Pathophysiology • Complication
INTRODUCTION

Myasthenia Gravis is an autoimmune disease of the neuromuscular junction


characterized by the fluctuating weakness of the certain skeletal muscle, group. It
results from a loss of acetylcholine receptors in the postsynaptic neurons of the
neuromuscular junction.
DEFINITION

Myasthenia gravis is a chronic autoimmune, neuromuscular disease that causes


weakness in the skeletal muscles (the muscles that connect to your bones and
contract to allow body movement in the arms and legs, and allow for breathing).
INCIDENCE

Myasthenia Gravis affects 1 or 2 people in every 10,000. It occurs more commonly


in women under 40 years and men over 60. However, it may affect individuals of
any age and we have largest experience in India for treatment of Myasthenia
Gravis.
CLASSIFICATION

Myasthenia Gravis Foundation of America Clinical Classification[10]

1. Class I: Any ocular muscle weakness, possible ptosis, no other evidence of


muscle weakness elsewhere.

2. Class II: Mild weakness affecting other than ocular muscles; may also have
ocular muscle weakness of any severity.

3. Class Ila: Predominantly affecting limb, axial muscles, or both; may also have
lesser involvement of oropharyngeal muscles
CLASSIFICATION

7. Class Ilb: Predominantly bulbar and/or respiratory mack to to top also have lesser
or equal involvement of limb, axial muscle or both.

8. Class IV: Severe weakness affecting other than ocular muscles; may also have
ocular muscle weakness of any severity.

9. Class IVa: Predominantly affecting limb, axial muscles, or both; may also have
lesser involvement of oropharyngeal muscles.
CLASSIFICATION

10. Class IVb: Predominantly bulbar and/or respiratory muscles; may also have
lesser or equal involvement of limb, axial muscles, or both (Can also include
feeding tube without intubation).

11. Class V: Intubation needed to maintain airway, with or without mechanical


ventilation.
RISK FACTORS

• Fatigue

• Stress

• Surgery

• Illness or infection
RISK FACTORS

• Some medications such as beta blockers, quinidine gluconate, quinidine sulfate,


quinine (Qualaquin), phenytoin, certain anesthetics and some antibiotics

• Pregnancy

• Menstrual periods
CAUSES

 Antibodies that destroys acetylcholine receptors.

 Genetic

• Unknown
TYPES

 Facial muscle weakness

 Bulbar muscle weakness.

 Limb muscle weakness

 Respiratory weakness

 Occular muscle weakness

 Extraoccular muscle weakness


FACIAL MUSCLE WEAKNESS

• It includes head extension and flexion weakness.

• Weakness may worse in proximal muscle.


EXTRAOCCULAR MUSCLE WEAKNESS

Ptosis is present initially in 50% of the patients and during course of disease in 90%
of patients.
BULBAR MUSCLE WEAKNESS

Group of muscles in head and neck.

• Palatal muscles

• Nasal voice ,nasal Regurgitation.

• Chewing become difficult.

• Severe jaw weakness

may cause jaw to hang open.


LIMB MUSCLE WEAKNESS

Upper limbs more Common than Lower limbs.


RESPIRATORY MUSCLE WEAKNESS

• Weakness of the intercostal space muscles and the diaphragm may result in Co2
retention due to hypoventilation.

• Weakness of pharyngeal muscles may collapse the upper airway.


OCCULAR MUSCLE WEAKNESS

Assymetric:-

• Usually affects more than one extraoccular muscles and is not limited to muscles
innervated by one cranial nerve. Weakness of lateral and medied recti may
produce & a pseudo internuclear opthalmoplegia.

• Ptosis caused by eyelid weakness.

• Diplopia is very common.


PATHOPHYSIOLOGY

Due to etiological Factor

Lymphocyte produce Ach Receptor antibodies that attack the postsynaptic muscle
membrane.

Depletion of acetylcholine receptors of the neuromuscular junction


PATHOPHYSIOLOGY

Defect in transmission of impulse from nerve to muscle cell

Myasthenia Gravis
CLINICAL MANIFESTATION

• Increasing muscle weakness affects movements of eyes, chewing, swallowing,


speaking and breathing

• Ptosis (drooping of one or both eyelids)


CLINICAL MANIFESTATION

• Laryngeal involvement produces dysphonia.

• Changes in facial expression

• Diplopia
CLINICAL MANIFESTATION

• Shortness of breath

• Impaired speech

• Weakness in arms, hands, fingers, legs and neck


DIAGNOSTIC EVALUATION

History collection –

• Age and gender-

The early type (before age of 50) is female predominant and the late type (after age
of 60) is male dominant.

• Race- Some studies demonstrated that the incidence, prevalence and the severity
of this disease is higher inAfrican/Americans.

• Evidence of coexisting autoimmune diseases.


DIAGNOSTIC EVALUATION

A positive history of-

 Heavy eyelids and double vision

 Eye movement problems

 Photophobia

 Facial weakness
DIAGNOSTIC EVALUATION

• Tongue weakness, chewing and swallowing problems

• Respiratory problems

• Limbs muscles weakness

• Fatigue

• Urinary incontinency
DIAGNOSTIC EVALUATION

Physical examination-

• Muscle strength and tone

• Co-ordination

• Sense of touch

• Any impairment of eye movement


DIAGNOSTIC EVALUATION

Edrophonium Tensilon test-This test is used to test eye muscle weakness and uses
injections of edrophonium chloride to briefly relieve weakness. The drug blocks the
breakdown of acetylcholine and temporarily increases the levels of acetylcholine at
the neuromuscular junction.
DIAGNOSTIC EVALUATION

Blood test-People living with myasthenia gravis may have abnormally elevated
levels of acetylcholine receptor antibodies.

A second antibody called the anti- MUSK antibody has been found in about half of
individuals with myasthenia gravis who do not have acetylcholine receptor
antibodies. A blood test can also detect this antibody.
DIAGNOSTIC EVALUATION

Electrodiagnostics-

Diagnostic tests include repetitive nerve stimulation, which repeatedly stimulates


nerves with small pulses of electricity to tire specific muscles. Muscle fibers in
myasthenia gravis, as well as other neuromuscular disorders, do not respond as well
to repeated electrical stimulation.
DIAGNOSTIC EVALUATION

Single fiber Electromyography-

It is most sensitive test for Myasthenia Gravis. It detects impaired nerve- to-
muscle transmission.
DIAGNOSTIC EVALUATION

Diagnostic imaging-

Diagnostic imaging of the chest using computed tomography (CT) or magnetic


resonance imaging (MRI) may identify the presence of a thymoma.
DIAGNOSTIC EVALUATION

• CT Scan
DIAGNOSTIC EVALUATION

M.R.I
DIAGNOSTIC EVALUATION

Pulmonary function test- Measuring breathing strength can help predict if


respiration may fail and lead to a myasthenic crisis.
DIAGNOSTIC EVALUATION

Ice pack test-When edrophonium test is contraindicated , used for patients with
ptosis.
MEDICAL MANAGEMENT

• Cholinesterase inhibitor

• Corticosteroid

• Immunosuppresants

• Plasmapheresis

• IVIg

• Monoclonal Antibodies
MEDICAL MANAGEMENT

Cholinesterase Inhibitor-

Mestinon or pyridostigmine . It slows down breakdown of Acetylcholine at the


neuromuscular junction and improve neuromuscular transmission and increase
muscle strength.
MEDICAL MANAGEMENT

Immunosuppressive drugs-Group of drugs that improve muscle strength by


suppressing the production of abnormal antibodies, such as prednisone,
azathioprine, mycophenolate mofetil, and tacrolimus.
MEDICAL MANAGEMENT

Plasmapheresis and intravenous immunoglobulin-Therapies that are used in severe


cases of myasthenia gravis to remove destructive antibodies that attack the
neuromuscular junction, although their effectiveness usually only lasts a few weeks
or months.
MEDICAL MANAGEMENT

https://youtu.be/oTKOxfhJ6AQ
MEDICAL MANAGEMENT

Monoclonal Antibodies-A treatment that targets the process by which


acetylcholine antibodies injure the neuromuscular junction. The U.S. Food and
Drug Administration (FDA) has approved the use of the medication eculizumab for
the treatment of generalized myasthenia gravis in adults who test positive for the
antiacetylcholine receptor (AchR) antibody.
SURGICAL MANAGEMENT

Thymectomy-An operation to remove the problematic thymus gland can reduce


symptoms, possibly by rebalancing the immune system. Myasthenia gravis with
thymoma is treated.
MANAGEMENT STRATEGIES FOR REHABLITATION

Treatment strategies may vary.

Evaluation is done on the basis of-

 Strength

 Flexibility

 Mobility

 Balance

 Gait
Benefits of Exercise in Myasthenia Gravis

 Weight reduction.

 Decrease in risk of hypertension, diabetes, cholesterol

 Decrease in risk of cardiac diseases


 Decrease in risk of osteoporosis

 Mood elevation

 Improve cognitive function

 Enhance baseline functional capacity, improved mechanical efficiency


EXERCISE GOALS

 Enhance ability to function daily

 Decrease risk of falling

 Completion of functional tasks and maintenance of independence

Smoothness and coordination of activities


EXERCISE CONSIDERATION

• Exercise at the best time of day

• Exercise at peak dose of medication (pyridostigmine)

• Exercise large, proximal muscle groups for short periods of time building up
only to moderate intensity

• Do not exceed moderate intensity exercise level


Nurses Responsibility During Exercise-

 Patient should not become short of breath at peak of exercising

 Myasthenia Gravis symptoms should not become worse during exercise


(drooping of eyes)

 Patient should not be tires after 2 hours of exercise


REHABLITATION EXERCISE

PACE is a suitable guideline.

P- Planning daily activities

A-Adapting the home ,adequate rest ,assistive equipment, asking for help.

C- Conserving energy, Check ups with doctor, cool temperature

E- Emotional stability, Excercising in moderation, eliminating unnecessary daily


tasks.
TYPES OF EXCERCISE

Types of Exercise used in Myasthenia Gravis-

 Breathing Exercises

 Postural Exercises

 Aerobic Excercises

 Strength Excercises

 Swimming
TYPES OF EXCERCISE

Breathing excercises- To strengthen the muscles that control the voice.

Vocal excercises to improve the ability to speak clearly.

Help to improve lung function.

It include respiratory muscle training-

 Pursed lip breathing

 Diaphragmatic breathing
TYPES OF EXCERCISE

Postural Exercises-

 Important in assisting with breathing, speaking and swallowing

 Keeps bones and joints in the correct alignment so that muscles are being used
properly

 Prevents fatigue because muscles are being used more efficiently, allowing the
body to use less energy
TYPES OF EXCERCISE

Strengthen Exercise -

 Should be done progressively

 Range of motion (flexibility) to light resistance to full resistance

 If significant weakness is present, active assist exercises may be necessary.

 The primary goal of therapy is to build the individual's strength to facilitate


return to work and activities of daily living.
TYPES OF EXCERCISE

Swimming-

 Patients should swim in water where they can touch the bottom.

 Deep water is dangerous and may cause patient to over exert.


NURSING MANAGEMENT

 Monitor respiratory status and ability to cough and deep breathe adequately.
Monitor for respiratory failure.

 Maintain suctioning and emergency equipment at the bedside.

 Monitor vital signs.

 Monitor speech and swallowing abilities to prevent aspiration.


NURSING MANAGEMENT

• Encourage the client to sit up when eating.

• Assess muscle status.

• Instruct the client to conserve strength.

• Plan short activities that coincide with times of maximal muscle strength.

• Monitor for myasthenic and cholinergic crises.


NURSING MANAGEMENT

 Administer anticholinesterase medications as prescribed.

 Instruct the client to avoid stress, infection, fatigue, and over-the counter
medications.

 Instruct the client to wear a Medic-Alert bracelet.

 Inform the client about services from the Myasthenia Gravis Foundation.
COMPLICATIONS

• Myasthenic Crisis

• Thyroid gland tumor


NURSING DIAGNOSIS

 Ineffective airway clearance related to decreased ability to cough or swallow as


evidenced by absent or ineffective cough.

 Impaired breathing pattern related to disease process as evidenced by

Presence of adventitious sounds.

 Fatigue related to muscle weakness as evidenced by lethargy.

 Activity intolerance related to low self esteem as evidenced by weakness.

 Risk for aspiration related to depressed gag reflex .


RESEARCH ARTICLE

“Global prevalence of myasthenia gravis and the effectiveness of common drugs in


its treatment: a systematic review and meta- analysis.”

Salari N et al. Transl Med. 2021.

PMID:34930325
RESEARCH ARTICLE

Background: Myasthenia gravis is a neuromuscular autoimmune disorder


characterized by weakness and disability in the voluntary muscles. There have been
several preliminary studies on the epidemiology of myasthenia gravis in different
parts of the world and the effectiveness of common drugs in its treatment using
systematic review and meta- analysis.
RESEARCH ARTICLE

Methods- Research studies were extracted from IranDoc, MagIran, Iran Medex,
SID, ScienceDirect, Web of Sciences (WoS), ProQuest, Medline (PubMed), Scopus
and Google Scholar based on Cochran's seven- step guidelines using existing
keywords extracted in MeSH browser. The 12 test was used to calculate the
heterogeneity of studies, and Begg and Mazumdar rank correlation tests were used
to assess publication bias. Data were analyzed using Comprehensive Meta-Analysis
software (Version 2).
RESEARCH ARTICLE

Results- In the search for descriptive studies based on the research question, 7374
articles were found. After deleting articles unrelated to the research question,
finally, 63 articles with a sample size of 1,206,961,907 people were included in the
meta-analysis. The prevalence of MG worldwide was estimated to be 12.4 people
(95% CI 10.6-14.5) per 100,000 population.
RESEARCH ARTICLE

. For analytical studies on the effectiveness of common myasthenia gravis drugs,


4672 articles were found initially, and after removing articles unrelated to the
research question, finally, 20 articles with a sample size of 643 people in the drug
group and 619 people in the placebo group were included in the study. As a result
of the combination of studies, the difference between the mean QMGS score index
after taking Mycophenolate and Immunoglobulin or plasma exchange drugs in the
group of patients showed a significant decrease of 1.4 ± 0.77 and 0.62 ± 0.28,
respectively (P < 0.01).
RESEARCH ARTICLE

Conclusion- The results of systematic review of drug evaluation in patients with


myasthenia gravis showed that Mycophenolate and Immunoglobulin or plasma
exchange drugs have positive effects in the treatment of MG. It also represents the
positive effect of immunoglobulin or plasma exchange on reducing SFEMG index
and QMGS index and the positive effect of Mycophenolate in reducing MG-ADL
index, SFEMG and Anti- AChR antibodies index. In addition, based on a meta-
analysis of the random-effect model, the overall prevalence of MG in the world is
12.4 people per 100,000 population, which indicates the urgent need for attention to
this disease for prevention and treatment.
SUMMARY

Myasthenia gravis is an autoimmune disease affecting the neuromuscular junction.


It causes muscle weakness. Myasthenia gravis may affect individuals of any age
and we have largest experience in India for treatment of Myasthenia gravis.
CONCLUSION

• As discussed throughout the presentation, learning about myasthenia gravis and


its management will help nurses to care for a myasthenia gravis patient.

• Nurses can do assessment of a myasthenia gravis patient, classify the level of


disease, observe the sign and symptoms, provide the necessary nursing care and
support the patient psychologically.
RECAPITULATION

 What is Myasthenia Gravis?

 What are the causes and sign and symptoms of Myasthenia Gravis?

 Write the short note on communication and rehabilitation management of


Myasthenia Gravis.

You might also like