MYASTHENIA

GRAVIS

Presenter: Ms. Priyadharshini. B ,

Master In Medical Surgical Nursing
INTRODUCTION:
The first case of MG was documented in 1672 by Thomas Willis, an Oxford physician.
Myasthenia gravis (MG) is an autoimmune disease characterized by muscle weakness and
fatiguability of skeletal muscles. It is an antibody-mediated disease, caused by autoantibodies
targeting neuromuscular junction proteins. In the majority of patients (~85%) antibodies
against the muscle acetylcholine receptor (AChR) are detected, while in 6% antibodies against
the muscle-specific kinase (MuSK) are detected. Although MG is a rare disease, with a
prevalence of 150–300 per million population and an incidence of ~10 per million per year.
 ANATOMY OF THE
NEUROMUSCULAR JUNCTION:
IN MYASTHENIA GRAVIS:
DEFINITION:
Myasthenia gravis is a disease that is characterized by progressive
weakness and exhaustibility (fatiguability) of voluntary muscles without
atrophy and is caused by an autoimmune attack on muscle cell
receptors which normally bind to acetylcholine released at nerve
endings .
_ Brunner&Suddarth
CAUSES:
Autoimmune disorder; significant skeletal muscle weakness:
▪ Decreased acetylcholine receptor function worsens with muscle use
▪ Most common neuromuscular junction disorder
Type II hypersensitivity reaction:
▪ B cells produce antibodies against postsynaptic nicotinic
acetylcholine receptors of neuromuscular junction/ receptor-
associated proteins
▪ Autoantibodies targeted against muscle-specific receptor tyrosine
kinase
▪ (MuSK) decreased in acetylcholine receptor function
Acetylcholine cannot bind normal action potentials
cannot be generated adjacent muscle
Complement activated → inflammatory response
initiation → postsynaptic membrane damage→
acetylcholine receptor destruction
Bimodal onset age
 20-30 years old (biologically-female predominance)
 60-70 years old (biologically-male predominance)
Associated with thymic abnormality; thymus
considered antigen source promoting autoantibody
production most cases and thymoma
Neonatal myasthenia gravis:

▪ Transient myasthenia form (new-born from individual

with myasthenia gravis)

▪ Maternal antibodies → transplacental passage

neuromuscular junction function interference

Rare non-immune mediated forms:

▪ E.g. congenital myasthenia gravis

▪ Mutations affecting neuromuscular transmission

PATHOPHYSIOLOGY:
Due to etiological factors
The post-synaptic muscle membrane is distorted and simplified, having
lost its normal folded shape
The concentration of Ach receptors on the muscle end-plate membrane
is reduced, and antibodies are attached to the membrane.
Ach is released normally, but its effect on the post-synaptic membrane is
reduced.
The post-junctional membrane is less sensitive to applied Ach, and
The probability that any nerve impulse will cause a muscle action
potential is reduced.
CLINCAL MANIFESTATION:
Fluctuating muscle weakness
▪ Exacerbated by repetitive muscle use
throughout day/after
exertion/repetitive movement .
▪ Improves with rest
Progression
▪ Symptoms continuously present,
fluctuate from mild-severe
▪ Sensation, reflexes preserved
Ocular myasthenia
▪ Limited (eyelid, extraocular muscle); individuals
(50%) with ocular myasthenia will myasthenia (<
two years)
Generalized myasthenia (Ocular, bulbar, facial, limb,
respiratory muscle)
Ocular muscles
▪ Eyelid (ptosis), extraocular (binocular diplopia) .
Bulbar muscle
▪ Jaw closure (prolonged chewing → weakness),
oropharyngeal (dysarthria, dysphagia), palatal
(nasal tone, prolonged speech → hypophonia)
Facial muscle
▪ Facial weakness, facial
expression loss.
Neck muscle.
▪ Cannot keep head up ("drooped
head syndrome")
Limb muscle
▪ Proximal, asymmetric muscle
weakness
Respiratory muscle
▪ Respiratory failure (myasthenic
crisis)
DIAGNOSTIC EVALUATION:
A physical and neurological examination:

▪ Individual’s medical history and conduct a physical examination. In a

neurological examination, the Nurse will check muscle strength and
tone, coordination, sense of touch, and look for impairment of eye
movements.

CT scan
▪ Chest scan to detect associated thymic abnormalities
▪ Abnormal thymus (most cases) Thymoma
LAB RESULTS
▪ Serologic test
▪ Acetylcholine receptor antibodies (AChR-Abs)/muscle-specific receptor
tyrosine kinase antibodies (MuSK-Abs)
▪ Most specific tests
▪ Seronegative for AChR-Abs, MuSK-Abs
Tensilon test:
▪ Edrophonium: acetylcholinesterase inhibitor with rapid onset, short acting
duration, this injections of edrophonium chloride to briefly relieve weakness
in people with myasthenia gravis. The drug blocks the breakdown of
acetylcholine and temporarily increases the levels of acetylcholine at the
neuromuscular junction. It is usually used to test ocular muscle weakness.
▪ Prolongs acetylcholine presence in neuromuscular junction which leads to
marked improvement
PFTs:
▪ Periodical FVC monitoring: FVC↓ reveals respiratory muscle
involvement
Ice pack test:
▪ Ice pack application (2-5 minutes) → MG-affected muscles
▪ Neuromuscular transmission improvement in low temperature
Electrodiagnostic:
▪ Diagnostic tests include repetitive nerve stimulation, which repeatedly stimulates a
person’s nerves with small pulses of electricity to tire specific muscles. Single fiber
electromyography (EMG), considered the most sensitive test for myasthenia gravis,
detects impaired nerve-to-muscle transmission. EMG can be very helpful in diagnosing
mild cases of myasthenia gravis when other tests fail to demonstrate abnormalities.
COLLABORATIVE MANAGEMENT:
▪ Avoid MG-exacerbating drugs (e.g. aminoglycosides,
tetracyclines, beta blockers, quinidine)
▪ Symptomatic therapy
▪ Immunomodulating agents decreased autoantibody
production. Individuals with poor acetylcholinesterase
inhibitor response Corticosteroids, other
immunosuppressive agents
▪ Medicine: Anticholinesterase medicines, steroids, or
medicines that suppress the immune system’s
response (immunosuppressive) medicines may be
used.
▪ Plasmapheresis. This procedure removes abnormal
antibodies from the blood and replaces the blood with
normal antibodies from donated blood.

▪ Immunoglobulin. This is a blood product that helps

decrease the immune system’s attack on the nervous
system. It's given by IV (intravenously).

▪ Infusions of monoclonal antibody. This includes

eculizumab. This treatment is effective for people with
the more common form of MG.
SURGICAL MANAGEMENT:
Thymectomy: This is surgical removal of the thymus gland. The
role of the thymus gland in MG is not fully understood.
Thymectomy, especially for thymoma; myasthenia often
improves/disappears
NURSING MANAGEMENT:
▪ Strictly administer medications at the right dose and the right time to avoid
exacerbations (under and overdoses can both produce harmful scenarios to the
client).
▪ Administer anticholinesterase drugs such as Neostigmine and corticosteroid
medication.
▪ Assist clients during plasmapheresis.
▪ Provide scheduled adequate resting periods for the client.
▪ Limit (emotional or physical) stressful activities as it could aggravate the
symptoms.
▪ Expose the client to a quiet and peaceful environment, free from extreme
temperatures.
▪ Assist the client in her basic needs and daily activities; eating, moving around, etc.
▪ Secure emergency cart or intubation set, suctioning machine, and catheters at the
bedside.
▪ Educate the client and family about the condition and compliance to treatments and
necessary procedures.
▪ Encourage the use of adaptive devices to prevent the client from injury.
▪ Assist clients in daily supportive exercises.
▪ Refer the client to physical therapists for appropriate exercises regimen.
▪ Encourage client to be involved in support groups and communities dealing with
myasthenia crisis.
Myasthenia Crisis
▪ Increase in vital signs: tachycardia, tachypnoea, high blood
pressure
▪ Cyanosis, restlessness
▪ Increased secretions
▪ Bowel and urinary incontinence
THANK YOU…