m (O· Sullivan & Schmitz, 2007)

m Myasthenia gravis is an autoimmune d/o

often associated with other
immunological dse. It is characterized by
weakness and extensive fatigability.
m ërevalence: 20/100,000
m MG may begin any time in life
m Onset, two major peaks
 omen: second & third decade
 Men: sixth decade
m ïction takes place at the site of the
neuromuscular (n.m.) junction & motor
endplate.
m Risk factors
 Ôhymic d/o
 Diabetes
 Other Immune d/o
 Menstrual period
 ëregnancy
m fundamental defect = n.m. junction
m Receptors at the motor endplate
normally receive acetylcholine (ï h)
from the motor nerve terminal.
Figure 12.7 Neuromuscular junction, Human
biology, 7th edition. McGraw-Hills©2001
Figure 9-
9-1. Motor end plate, Electrodiagnosis in disease of nerves and
muscles: Principles and Practice, 3rd Edition. Oxford University Press©2001
m receptors are ÿ & those that remain are
flattened = ÿ efficiency of n.m.
transmission
m ithout ïch: nerve impulses fail to pass
across the n.m. junction to stimulate
muscle contraction
m n.m. abnormalities = autoimmune
response
 specific anti-ï h receptor antibodies
m Response
 blocks the site that normally binds ïch
 damage the postsynaptic muscle
membrane
 Endocytosis of receptor site
˜ pinching off of regions of the cell's membrane
m MG & Ôhymic d/o
 cause of the autoimmune response is not
well understood
 thymus appears to play a role in the dse
˜ 75 % of persons with MG have abnormalities of
the thymus
 ells within thymus bear ï h receptors on
their surface
 ells within the thymus bear ï h receptors
on their surface
 serve as a source of autoantigen to trigger
the autoimmune reaction within the thymus
gland when an immunologic abnormality
causes a breakdown an autoimmune attack
on acetylcholine (ï h) receptors.
CLASS I any ocular weakness; may have weakness of eye closure; all
other muscle strength is normal
CLASS II mild weakness affecting other than ocular muscles; may also
have ocular muscle weakness of any severity
IIa
predominantly affecting limb, axial muscles or both; may
also have lesser involvement of oropharyngeal weakness
IIb
predominantly affecting oropharyngeal respiratory muscles,
or both; may also have lesser involvement of oropharyngeal
weakness
CLASS III moderate weakness affecting other than ocular muscles;
may also have ocular muscle weakness of any severity
 IIIa
predominantly affecting limb, axial muscles or both; may
also have lesser involvement of oropharyngeal muscles
IIIb
predominantly affecting oropharyngeal, respiratory muscles,
or both; may also have lesser or equal involvement of limb,
axial muscles or both
CLASS IV severe weakness affecting other than ocular muscles; may
also have ocular muscle weakness of any severity
IVa
predominantly affecting limb and/or axial muscles
IVb
predominantly affecting oropharyngeal, respiratory muscles,
or both; may also have lesser or equal involvement of limb,
axial muscles or both
CLASS V defined by intubation, with or without mechanical
ventilation, except when employed during routine
postoperative management. Ôhe use of feeding tube
without intubation places the patient in class IVb.
m MG encompasses a spectrum of mild to
severe (Goodman & Fuller, 2009)
m cardinal features
 Diplopia;
 ëtosis; Unilateral frontalis muscle contraction
due to weak lid elevators on that side
m Motor symptoms
 Fatigue
 Muscle weakness
˜ Facial Muscles
˜ Eyelid closure is almost always weak
˜ eyelids are separated against forced eye closure
˜ slight involuntary opening of the eyes as the patient
tries to keep the eyes closed
˜ Snarling expression on attempted smile
˜ Sleepy or sad facial appearance
˜ Neck muscles; causes head bobbing due to
weak neck flexors
˜ Intercostal and diaphragm muscles; SOB
 ˜ ëroximal limb weakness
˜ having difficulty raising arms above the head
˜ having difficulty climbing up stairs
˜ having difficulty arising from a chair
Î Speech, voice and swallowing disorders
 Oropharyngeal muscles weakness:
Dysphagia, Dysarthria, Dysphonia, voice
may be nasal,
 Jaw weakness: prolonged chewing,
especially tough, fibrous or chewy food
m ardiopulmonary function
 weak bulbar muscles; aspiration pneumonia
 weak chest wall muscles; respiratory failure
m ëattern of Symptoms
 Fatigability of muscles with recovery to the
baseline strength after a short period of rest
 ëroximal muscles are more affected
 S/sx fluctuate throughout the day
 S/sx are provoked by exertion
 Fluctuations occur with superimposed illness,
menses, & air Ô
 Neurologic findings are normal except for
muscle weakness
 No muscular atrophy
 Reflexes and sensation normal
ë
  a mimic MG   a do
no mimic
MG
MS Onset: 20 & 40 y/o ëeak onset: 30 y/o
ërimary affected area: ërimary affected are is
nervous system NS white matter in MS
Speech impairement and neuromuscular
Fatigue, muscle weakness, junction in MG,nystagmus
Guilain- ïffects all age groups muscular weakness
Barre Respiratory involvement progress from lower
syndrome extremity-upper extremity
Lambert- Muscle weakness autoantibodies directed
Eaton ëroximal muscle involvement against the ëREsynaptic
Myasthenic a channels
syndrome
m Edrophonium hloride Ôest
m ïuto-ïntibodies in MG
 ïnti-striational muscle anti-bodies
 ïcetylcholine receptor antibodies (ï hR-
ab)
 ïnti-musK antibodies
 Other auto-antibodies
˜ ïnti-titin antibodies
˜ ïnti-RyR
m Electrodiagnostic Ôesting
 Repetitive nerve stimulation
 Single fiber EMG
m Ocular cooling/Ice ëack test
m Other studies
 omplete blood count
 Ôhyroid Function Ôest
 Ôhyroid ïntibodies
m Ôhe prognosis of MG in infancy is usually
favorable, although exacerbations may
occur in fevers. In sporadic case, a
fulminating onset with life-threatening
respiratory insufficiency may occur.
m ërogression to severe disease may be
more common in MG with onset after
the age of 50.
Î Medical, Surgical and ëharmacological
management
 Ôhymectomy
 ëlasma exchange (ëLEX)
 Intravenous Immunoglobulin (IGIv)
 holinesterase inhibitor drugs ( hI)
˜ ëyridostigmine bromide
˜ Neostigmine
 orticosteroid
˜ ërednisone
 Immunomodulatory drugs
˜ ïzathioprine
˜ yclosporine
˜ Mycophenolate mofetil
  Miscellanous
˜ Ephedrine
˜ Ôerbutaline
Î Rehabilitation management
 alking
 Stationary ergometer
 eight training
 Ôreadmill
 Swimming