2.2 Cs Exam 5: Ms & Myopathic Disorders

Front 1

_______ is an autoimmune inflammatory disorder that results in CNS demyelination.

Back 1

Multiple Sclerosis (MS)

Front 2

What are the 4 diff disease patterns for MS?

Back 2

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

Front 3

MS: symptoms

(non-specific)

Back 3

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

Front 4

MS is difficult to diagnose, what are some modalities for dx?

Back 4

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)

Front 5

*What is the Aim of tx in MS?

Back 5

Decrease relapse rates

Reduce progression & disability

Slow accumulation of lesions on MRI (may not be present in early disease)

Front 6

How are acute attacks (relapses) of MS tx?

What are the SEs of these drugs?

Back 6

Corticosteroids

SE: weight gain, moon facies, buffalo hump, anxiety, avascular necrosis, hyperglycemia

Front 7

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)

Back 7

Interferon Beta or Glatiramer acetate

Front 8

What are the SEs assoc w/ Interferon Beta?

Back 8

flu-like sx (tx w/ NSAIDs)

asymptomatic liver transaminase elevation

hepatoxicity (rare)

*neutralizing Abs may limit effectiveness

Front 9

________ is a potent inducer of TH2 suppressor cells.

What SEs does it cause?

Back 9

Glatiramer acetate

SE:

injection site rxn

transient chest pains, flushing, SOB, palpitation, & anxiety

Front 10

In (myalgia/ myopathic) disorders weakness > pain

Back 10

Myopathic disorders

(myalgia syndromes (polymyalgia rheumatic (assoc w/ temporal arteritis), fibromyalgia), pain > weakness)

Front 11

What is the best way to differentiate true muscle weakness (fixed muscle weakness) from difficulty using muscles bc of constant pain (myalgia)?

Back 11

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

Front 12

Most common causes of true myopathies?

Back 12

Statins

Cocaine

alcohol

endocrine disorder

(inflammatory myopathies are not very common)

Front 13

Weakness is the "cardinal symptoms" of myopathy. How does it usually present?

Back 13

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

Front 14

_______ is a very poor prognositic sign in a pt presenting w myopathy!

*admit ASAP!

Back 14

Inability to lift head or handle saliva

(risk of aspiration)

Front 15

If muscle weakness is asymmetric & distal, it is most likely d/t a _______________

*esp if assoc w/ muscle atrophy, ptosis, &/or cranial neuropathies

Back 15

Neuropathic disorder

(ALS, MS, myasthenia gravis, dystrophy)

Front 16

If a child presents w/ rapidly progressive, spotty & asymmetric muscle weakness, of distal & proximal muscles & extremely high CPK's, what should you suspect?

Back 16

Influenza virus

(infectious myopathy)

Front 17

___________ deficiency causes metabolic myopathy associated w shortness of breathe & muscle cramps

Back 17

Carnitine deficiency

Front 18

When should you check serology for autoabs in your myopathy work up?

Back 18

when there is evidence of dermatomyositis or polymyositis (inflammatory myopathy)

Front 19

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?

Back 19

Anti-tRNA synthetase Abs (Jo-1)

= Jo-1 syndrome

Front 20

When do you need to hospitalize a pt w/ myopathy?

Back 20

Extremely high CK (CPK) > 10,000

Profound weakness--> inability for self care or fall risk

Dysphagia w/ aspiration

Loss of neck flexors

Front 21

Tx for severe myopathy (hospitalized tx)

What is the best way to dx the specific myopathy (gold standard)?

Back 21

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

Front 22

What endocrine disorders can cause muscle weakness?

*suspect if delayed DTR or exopthalmos

Back 22

Acromegaly

Cushing Dz

Hypo/Hyperthyroid

Hyper PTH

DM

Front 23

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)

Back 23

fibrates