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24 Cards in this Set
- Front
- Back
What are the three steps to approaching a patient with generalized muscle weakness?
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1. Distinguish as TRUE muscle weakness
2. Localize site of lesion 3. Determine cause |
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How do you determine TRUE muscle weakness?
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Formal muscle testing
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How do you localize muscle lesion site?
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EMG/NCV
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List three Idiopathic Inflammatory Myopathies (IIM).
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1. Polymyositis
2. Dermatomyositis 3. Juevenile DM/PM |
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What population group is most likely at risk for PM/DM?
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Black women
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In caucasian patients, what are the genetic components associated with IIM? x3
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HLA-DR3
HLA-DR6 HLA-138 |
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What genetic component is associated with an increased risk of development of PM and Juvenile DM
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HLA-DR3
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What is the diagnostic criteria for PM/DM? x6
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Symmetric weakness
Proximal muscle weakness EMG myopathic changes Characteristic abnormalities in biopsy Serum muscle enzymes elevated Typical rash for DM |
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In PM/DM, what is the definite test for diagnosis?
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Muscle Biopsy
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What are the three rules to follow when doing a biopsy?
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1. Must be obtained from weak/inflamed muscle
2. Avoid muscles with atrophy 3. Prefer to use Open Needle Biopsy |
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What are the common pathological features of PM/DM? x4
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1. Muscle fiber Degeneration
2. Muscle fiber Regeneration 3. Muscle fiber Necrosis 4. Inflammatory cell infiltrate (lymphocytic) |
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Which IIM is a humorally mediated disorder?
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DM
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Which IIM is a cell mediated disorder?
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PM
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Pathological features specific for DM. x4
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Perivascular Inflammation
Perifascicular atrophy/necrosis B lymphocytes Increased ratio of CD4/CD8 T-cells |
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Pathological features specific for PM. x3
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Infiltrate within fascicle
CD8+ T cells NO VASCULOPATHY |
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PM/DM:
- Symptoms x3 |
1. Symmetric and Proximal weakness
2. Reflux 3. Aspiration 4. Dysphagia 5. Dysphonia |
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PM/DM:
- What muscles are spared? - Pain |
Facial and ocular muscles
20-25% |
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PM/DM:
- Non-muscle related symptoms x4 |
Fever
Raynaud's Arthritis Weight loss |
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Skin manifestations of DM x2
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Gottron's papules
Heliotrope rash |
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What is pathognomonic for DM?
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Gottron's papules
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What labs would you expect in PM/DM? x4
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Increased CK
Increased Aldolase Positive ANA Positive anti-JO-1 |
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Prognosis for PM/DM?
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80% to 90% have 15 yr survival rate
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In what population group is morbidity/mortality worse?
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Black females
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What type of patients should be screened for underlying malignancy in association with IIM?
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Over 50 y.o. with new onset IIM.
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