MYASTHENIA GRAVIS

Disease
MYASTHENIA GRAVIS
§ Myasthenia gravis means “grave
muscle weakness” or “weakness of
the voluntary or striated muscles
of the body”.
§ It is an autoimmune and
neuromuscular disorder that fails to
transmit nerve impulses at the
myoneural junction
MYONEURAL JUNCTION / NEUROMUSCULAR JUNCTION

- a chemical synapse between

a motor neuron and a muscle
fiber.
- It allows the motor neuron to
signal to the muscle fiber,
causing muscle contraction.
 EPIDEMIOLOGY

Prevalence: most common § Affects approximately 20 in 1

neuromuscular junction disorder
§ Sex: ♀ > ♂ (3:2)
§ Peak incidence
Ø ♂ : 40–70 years
Ø ♀ : 20–40 years
million people in the United States
§ More women than men
§ Commonly in the second and
third decades of life; however,
after age 50, the gender
distribution is more equal
§ Autoimmune disease
§ Decrease acetylcholine secretion by the motor end
plate
§ Increase cholinesterase at the nerve-ending

CAUSES
PATHOPHYSIOLOGY
Due to auto- Development of Antibodies attack
Acetylcholine
immune auto- receptors at the
response antibodies motor end plate

Act against the Impair the Resulting in

nicotinic ability of voluntary
acetylcholine acetylcholine muscle
receptor receptor weakness
1. OCULAR MYASTHENIA
§ Only the extraocular
and/or eyelid muscles

2. GENERALIZED MYASTHENIA
§ All skeletal muscles may be involved.
§ Especially ocular, bulbar, limb, and
respiratory muscles

CLASSIFICATION
 3. CONGENITAL MYASTHENIA GRAVIS
§ The immune system attacks damage
communication between nerves and
muscles — the cause of the other forms of
this disease.

4. TRANSIENT NEONATAL MYASTHENIA GRAVIS

§ Symptoms can include impaired sucking
and swallowing, a weak cry, and respiratory
insufficiency.

5. JUVENILE MYASTHENIA GRAVIS

§ Clumsiness to difficulty in swallowing and
tiring easily.
 CLINICAL MANIFESTATION
§ Drooping of one or both eyelids (ptosis)
§ Blurred or double vision
§ Breathing difficulty because of the chest wall muscle
weakness.
§ Chewing swallowing difficulty, causing frequent
gagging, chocking, drooling
§ Difficulty in climbing stairs, lifting objects, or rising
from a seated position
§ Difficulty in talking(slurred speech)
§ Impaired speech (dysarthria)
§ Drooping head
§ Facial paralysis or weakness of the
facial muscle
§ Mask-like facial expression
§ Easy fatigability
§ Hoarseness or changing voice
§ Difficulty in maintaining in steady
gait
§ Weakness in arm, hands, fingers,
legs, and neck.
DIAGNOSTIC EVALUATION
 MEDICAL MANAGEMENT
§ MG is directed at improving function and reducing and
removing circulating antibodies.
§ Specific treatment to age, overall health, 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.
 Administration: Taken
Indications: Approved in
orally (syrup, conventional
U.S. for the treatment of
or extended-release
myasthenia gravis
tablets)

Contraindications: Not
Side effects: May
indicated for those with
include nausea, diarrhea,
mechanical obstructions of
vomiting, excessive
the digestive or urinary
salivation and sweating
tracts
PYRIDOSTIGMINE BROMIDE (MESTINON)
- an anticholinesterase medication, the first line of therapy. Provides
relief by inhibiting the breakdown of acetylcholine and increasing
the relative concentration of available acetylcholine.

PHARMACOLOGIC THERAPY
PREDNISONE
Suppress the immune system by
reducing the production of antibodies,
or proteins that attack or block a
specific invader or other target. This
results in less damage to receptors at
nerve-muscle junctions, easing the
symptoms of MG.
INTRAVENOUS IMMUNE
GLOBULIN (IVIG)
Used to treat exacerbations,
involves the administration of
pooled human gamma-globulin,
and improvement occurs in a few
days.

IMMUNOSUPPRESSANTS CYTOTOXIC
These therapies either inhibit or Azathioprine (Imuran) – inhibits T
prevent the activity of the immune lymphocytes and B-cell
system when it tries to fight the proliferation and reduces
receptors required to transmit nerve acetylcholine receptor antibody
signals to muscles. levels.
HERAPEUTIC PLASMA EXCHANGE (TPE)
• Formerly referred to as plasmapheresis, used to
treat exacerbations.
• Plasma components are removed through a
centrally placed large-bore double-lumen catheter.
• The blood cells and antibody-containing plasma
are separated, after which the cells and plasma
substitute are reinfused.
• Typical course:
daily or
alternate-day
treatment and
the number of
treatments is
determined by
the patient’s
response
 SURGICAL MANAGEMENT
THYMECTOMY – can produce antigen-specific
immunosuppression and result in clinical improvement, only
treatment that can result in complete remission, approx. 35% of
patients

§ Transsternal
§ Transcervical
§ Video-assisted thoracoscopic
surgery
 COMPLICATIONS
§ MYASTHENIA CRISIS – an exacerbation of the
disease process characterized by severe
generalized weakness and respiratory and bulbar
weakness that may result in respiratory failure.

§ NEUROMUSCULAR RESPIRATORY FAILURE - a

critical complication in myasthenic and cholinergic
crises.
MYASTHENIC VS CHOLINERGIC
Myasthenic Crisis
§ Under Medication
§ Increased HR/BP/RR
§ Bowel & Bladder Incontinence
§ Decreased UO
§ (-) Cough & Swallow Reflex
§ May need Mech Vent
§ Temporary improvement
symptoms with administration of § Constriction of Pupils
Tensilon
Cholinergic Crisis
§ Over Medication
§ Decreased BP
§ Abdominal Cramps
§ N/V, Diarrhea
§ Blurred Vision
§ Pallor
of § Facial Muscle Twitching
§ Tensilon has no effect
§ Symptoms improve with
administration of Anticholinergics
(Atropine)
§ Educational topics include medication management energy
conservation, strategies to help with ocular manifestations, and
prevention and management of complications.
§ Remind the patient of the importance of maintaining health-
promotion practices and following healthcare screening
recommendations
§ Factors that exacerbate symptoms and potentially cause crisis should
be noted and avoided: emotional stress, infections (particularly
respiratory infections), vigorous physical activity, some medications,
and high environmental temperature

NURSING MANAGEMENT
Yoga Exercises
§ Help to stretch the body which increases
flexibility as well as strengthens the muscles of
the body, which become weak due to MG.
§ Improves blood circulation and removes
carbon dioxide from the body & provides
oxygenated blood to every part of the body.

NUTRITION
§ Simple, nourishing, no stimulating foods, including plenty of
fresh fruits & lightly cooked vegetables, 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 place of eggs and
meats. Food should have a blend of all necessary
vitamins.