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23 Cards in this Set
- Front
- Back
_______ is an autoimmune inflammatory disorder that results in CNS demyelination. |
Multiple Sclerosis (MS) |
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What are the 4 diff disease patterns for MS? |
Relapsing-remitting: relapse w full recovery Secondary progressive: relapsing-remitting w/ progression Primary progressive: progressive since onset, get sick & don't get better Progressing relapse: progression from onset w/ acute relapses |
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MS: symptoms (non-specific) |
optic neuritis (*MC) internuclear opthalmoplegia (Charcot's triad) sensory changes (numbness, tingling, radiculitis)(common initial sx) pain (dyesthesias, lhermitte's phenomenon) vertigo motor changes (paraperesis, paraplegia) Bowel & bladder dysfxn (urgency, UTI) Sexual Dysfxn Heat sensitivity Fatigue Depression Cognitive dysfxn Seizures |
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MS is difficult to diagnose, what are some modalities for dx? |
McDonald Criteria (MS, possible, not MS) MRI*(dx of choice, demyelination lesion in cerebral or spinal tract, Dawson's fingers, plaques) CSF (presence of oligo clonal bands) |
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*What is the Aim of tx in MS? |
Decrease relapse rates Reduce progression & disability Slow accumulation of lesions on MRI (may not be present in early disease) |
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How are acute attacks (relapses) of MS tx? What are the SEs of these drugs? |
Corticosteroids SE: weight gain, moon facies, buffalo hump, anxiety, avascular necrosis, hyperglycemia |
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ALL pts w/ MS should be put on either _________ or ___________ Tx early aggressively tx (induction phase), followed by maintenance tx to reduce progression! (may choose to delay tx bc of tons of SE's) (beneficial w/ relapsing-remitting dz) |
Interferon Beta or Glatiramer acetate |
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What are the SEs assoc w/ Interferon Beta? |
flu-like sx (tx w/ NSAIDs) asymptomatic liver transaminase elevation hepatoxicity (rare) *neutralizing Abs may limit effectiveness |
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________ is a potent inducer of TH2 suppressor cells. What SEs does it cause? |
Glatiramer acetate SE: injection site rxn transient chest pains, flushing, SOB, palpitation, & anxiety |
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In (myalgia/ myopathic) disorders weakness > pain |
Myopathic disorders (myalgia syndromes (polymyalgia rheumatic (assoc w/ temporal arteritis), fibromyalgia), pain > weakness) |
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What is the best way to differentiate true muscle weakness (fixed muscle weakness) from difficulty using muscles bc of constant pain (myalgia)? |
Ask about problems w/ daily activities (getting up out of chair, brushing teeth, going up stairs, swallowing/talking (very bad sign**) *pt may have a wide stance to help stand w/ weak muscles |
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Most common causes of true myopathies? |
Statins Cocaine alcohol endocrine disorder (inflammatory myopathies are not very common) |
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Weakness is the "cardinal symptoms" of myopathy. How does it usually present? |
symmetric proximal muscles (shoulder-girdle syndrome) Gower's sign ("climbing up self" to get up) Trendelenberg gait Difficulty getting out of chairs, lifting legs to dress, going up/down stairs, walk w/ legs separate, etc |
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_______ is a very poor prognositic sign in a pt presenting w myopathy! *admit ASAP! |
Inability to lift head or handle saliva (risk of aspiration) |
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If muscle weakness is asymmetric & distal, it is most likely d/t a _______________ *esp if assoc w/ muscle atrophy, ptosis, &/or cranial neuropathies |
Neuropathic disorder (ALS, MS, myasthenia gravis, dystrophy) |
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If a child presents w/ rapidly progressive, spotty & asymmetric muscle weakness, of distal & proximal muscles & extremely high CPK's, what should you suspect? |
Influenza virus (infectious myopathy) |
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___________ deficiency causes metabolic myopathy associated w shortness of breathe & muscle cramps |
Carnitine deficiency |
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When should you check serology for autoabs in your myopathy work up? |
when there is evidence of dermatomyositis or polymyositis (inflammatory myopathy) |
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Pt w/ dermatomyositis (inflammatory myopathy) has concurrent interstitial lung disease, raynaud's, arthritis, & mechanical hands. What do you expect to find on Ab serology? Dx? |
Anti-tRNA synthetase Abs (Jo-1) = Jo-1 syndrome |
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When do you need to hospitalize a pt w/ myopathy? |
Extremely high CK (CPK) > 10,000 Profound weakness--> inability for self care or fall risk Dysphagia w/ aspiration Loss of neck flexors |
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Tx for severe myopathy (hospitalized tx) What is the best way to dx the specific myopathy (gold standard)? |
Tx: elevate Head of bed 30 degrees (aspiration precaution) Pulse corticosteroids, taper & switch to DMARDs when stabilized Rehydration (if rhabdo), monitor electrolytes Swallowing studies Bed side PFTs IVIG Tx underlying cause Dx: muscle biopsy |
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What endocrine disorders can cause muscle weakness? *suspect if delayed DTR or exopthalmos |
Acromegaly Cushing Dz Hypo/Hyperthyroid Hyper PTH DM |
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Statins are not recomended in pts w/ myositis bc they can worsen muscle pain & weakness. However, if pt has CV disease, stay on statins (CV will kill you before myositis) & monitor CK. If pt is on statins & have myopathy, you can NEVER use __________ (additionally) |
fibrates |