Escolar Documentos
Profissional Documentos
Cultura Documentos
Age onset is
between 2nd-3rd decades, and 6th-8th decade
2. 10-15% people with myasthenia gravis have underlying thymoma
3. epidemiology:
a. 10-20 new cases per million a year
b. prevalence: 150-200 per million
c. neonates can have transient form of MG called neonatal MG- due
to transplacental passage of maternal ab that interfere with
function of neuromuscular junction
4. pathogenesis
a. autoimmune disorder characterized by weakness and
fatiguability of skeletal muscles due to dysfunction of
neuromuscular junction
b. MG is a condition that fulfills major criteria for a disorder
mediated by autoantibodies against acetylcholine receptor.
Linkage between AchR-ab and myasthenia gravis are not
absolute. Autoantibodies directed against muscle-specific
receptor tyrosine kinase (MuSK) and other postsynaptic
neuromuscular junction are also pathogenic.
c. Some patients who are seronegative for AchR-ab have Ab
directed against another target on the surface of the muscle
membrane, MuSK.
d. Seronegative myasthenia gravis, also called Ab negative
myasthenia gravis is in 6-12% pts with MG
e. Majority of patients with AchR antibody positive MG have thymic
abnormalities: hyperplasia in 60-70% patients, thymoma in 1012% patients. The disease often improve or diappear after
thymectomy. The thymus has been evaluate as possible source
of antigen to drive this autoimmune disease
5. symptoms:
a. fluctuating muscle weakness, often with true muscle fatigue.
Fatiue is manifest by worsening contractile force of muscle
b. 2 clinical forms of MG: ocular and generalized. In ocular MG:
weakness is limited to eyelids and extraocular muscles. In
generalized MG: weakness may affect ocular muscle, but also
combination of bulvar, limb, and respiratory muscles
c. more than 50%pts present with ptosis and or diplopia
d. about 15% pts present with bulbar symptoms- fatigable
chewing, dysphagia, and dysarthria
e. less than 5% present with proximal limb weakness alone
f. eyelid muscle weakness = ptosis throughout the day, extraocular
muscle weakness = binocular diplopia that disappears when pt
closes or occludes eyes
g. prolonged chewing can cause muscle of jaw fatigue.
Oropharyngeal weakness produces dysarthria and dysphagia
h. facial muscle involvement causes expressionless