Thoracic 3.0 Flashcards
Myasthenia Gravis
Prevalence:
5 to 12 per 100, 000 in the population.
Myasthenia Gravis
Gender prevalance and how does this effect presentation?
disease is twice as common in women
Males have more rapid progression, fewer remission, and less improvement
Age:
Women: the second to third decade of life.
Men, sixth to seventh decade of life.
Myasthenia Gravis
Grading of Symptoms
Grading of Symptoms
(I) focal disease—ocular muscle weakness
(II) generalized mild to moderate disease
(III) severe generalized weakness
(IV) life-threatening weakness—respiratory failure.
Myasthenia Gravis
Grade 1 Symptoms
Myasthenia Gravis
Grading of Symptoms
(I) focal disease—ocular muscle weakness
(II) generalized mild to moderate disease
(III) severe generalized weakness
(IV) life-threatening weakness—respiratory failure.
Myasthenia Gravis
Grade II symptoms
Myasthenia Gravis
Grading of Symptoms
(I) focal disease—ocular muscle weakness
(II) generalized mild to moderate disease
(III) severe generalized weakness
(IV) life-threatening weakness—respiratory failure.
Myasthenia Gravis
Grade III symptoms
Myasthenia Gravis
Grading of Symptoms
(I) focal disease—ocular muscle weakness
(II) generalized mild to moderate disease
(III) severe generalized weakness
(IV) life-threatening weakness—respiratory failure.
Myasthenia Gravis
Grade IV symptoms
Myasthenia Gravis
Grading of Symptoms
(I) focal disease—ocular muscle weakness
(II) generalized mild to moderate disease
(III) severe generalized weakness
(IV) life-threatening weakness—respiratory failure.
Myasthenia Gravis
Grading of Symptoms:
focal disease—ocular muscle weakness
Myasthenia Gravis
Grading of Symptoms
(I) focal disease—ocular muscle weakness
(II) generalized mild to moderate disease
(III) severe generalized weakness
(IV) life-threatening weakness—respiratory failure.
Myasthenia Gravis
How to diagnose ?
Diagnosis
- a physical examination identifying the level of weakness
- an edrophonium (shortacting anticholinesterase) test is confirmatory if the patient shows improvement.
- Assay for AChR antibody is also available.
Myasthenia Gravis
Grading of Symptoms
generalized mild to moderate disease
Myasthenia Gravis
Grading of Symptoms
(I) focal disease—ocular muscle weakness
(II) generalized mild to moderate disease
(III) severe generalized weakness
(IV) life-threatening weakness—respiratory failure.
Myasthenia Gravis
Treatment - medical therapy
- Anticholinesterase therapy (pyridostigmine)
- corticosteroids if needed for immunosuppression.
-
Plasmapheresis and IVIG are short-term
- for myasthenic crises
- preoperatively, or intermittently in patients with poor control of MG despite immunosuppression.
Myasthenia Gravis
Indications/contraindications for thymectomy
Thymectomy
indicated
- in patients with thymic hyperplasia or a thymoma.
- any MG patient with early, generalized, moderate to severe disease, especially if refractory to medical management.
Contraindications
should not be performed emergently for a myasthenic crisis/class IV disease.
Results of thymectomy for MG?
- % “some clinical improvement” ?
- % resolution of symptoms ?
- % improved symptoms ?
- % drug free remission ?
- 85% of patients will have some clinical improvement
- 25-30% will have resolution of sx
- 30-50% will have Improved symptoms
- 50-60% will have drug free remission
Age and symptomatology of mediastinal masses
Adults are more likely to be asymptomatic (60%)
Children are more likely to be symptomatic (60%)
Symptomatology of mediastinal masses
Anatomic location and symptoms
- Anterior / superior masses (75%) -most likely to be symptomatic
compared with:
- posterior (50%)
- Middle (45%)
symptomatology of mediastinal masses
which are more likely to have sx - benign or malignant?
Malignant masses are more likely to have symptoms
Mediastinal masses - by anatomic location what is the the most likely to be malignant?
- Anterior superior are most likely to be malignant (60%)
- Middle (29%)
- Posterior (16%)