Myopathies - Lubeck

Front 1

General Principles of Myopathies (5)

Back 1

- Progressive proximal muscle weakness (usually painless)
- Normal sensory fx
- Normal or reduced DTRs
- Proximal atrophy
- CPK increased

Front 2

CPK is markedly increased in _____ and ________ myopathies

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Neurotic
Inflammatory

Front 3

CPK is mild-moderately increased in ______ myopathies

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atrophic

Front 4

Classic atrophic myopathy

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Steroid myopathy

Front 5

Atrophic myopathy labs (3)

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Normal CPK
Normal of myopathic EMG
Biopsy: fiber atrophy

Front 6

Necrotic myopathies labs (3)

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-CPK increased
-EMG irritable and myopathic
-Biopsy: necrosis and inflm

Front 7

Degenerative myopathy labs

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- CPK mild increase
- EMG: irritable and myopathic
- Biopsy: vascular degeneration

Front 8

Infiltrative myopathy labs

Back 8

- CPK normal
- EMG normal
- Biopsy: interstitial inflm

Front 9

Duchenne Muscular Dystrophy is an example of a ______ myopathy

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Necrotic

Front 10

Sarcoidosis produces non-caseasting granulomas that infiltrate muscles and is called ___________ myopathy

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Infiltrative

Front 11

More common in F
Peak incidence
- 5-15
- 45-55

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Poliomyositis

Front 12

#1 sx is hip weaknes
Has various other muscle pains, dysphagia and rash
Presents within wks-months (subacutely)

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Polyomyositis

Front 13

Rash of poliomyositis most often in (2 locations)

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-Around eyes
- Extensory surface of fingers

Front 14

People with this wil have trouble getting out of chair, brushing teeth, brushing hair, etc

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Poliomyositis

Front 15

Polymyositis has many different classifications. The two main ones are:

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Polyomyositis
Dermatomyositis

Front 16

Form of poliomyositis
-More homogenous
- High incidence of cancer (breast most common)
- Little associated with CVD
- Greater severity

Back 16

Dermatomyositis

Front 17

Form of poliomyositis
- Heterogenous
- Low incidence of cancer
- Associated with CVD
- May be mild

Back 17

Polyomyositis

Front 18

-Rash of poliomyositis
-Red, purplish rash on eyelids

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Heliotrope rash

Front 19

-Poliomyositis skin lesion
- Red keratotic erythema on extensor surface of fingers

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Groton's sign

Front 20

In addition to rash on eyelids and extensor surface of fingers, people with poliomyositis can also get erythema on _____________

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Extensor surface of extremity joints

Front 21

Means "many muscle inflm"

Back 21

Poliomyositis

Front 22

Poliomyositis CPK is often ____x+ the upper limit of normal

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10x

Front 23

Treatment of poliomyositis

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steroids

Front 24

In muscle bx with dermatomyositis, get ______________ --> size of fiber at periphery is much smalelr

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Perifascicular atrophy

Front 25

Muscle biopsy of poliomyositis has (3)

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-Perivascular infiltration
-Interstitial fibrosis
-Perifascicular atrophy (dermatomyositis)

Front 26

-Age of onset > 30
-Duration> 6 mos
-Weakness of proximal AND distal arm and leg muscles

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Inclusion Body Myositis

Front 27

CPK of inclusion body myositis is ______

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12x normal

Front 28

EMG of inclusion body myositis shows _______ changes

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Inflm

Front 29

Inclusion body myositis biopsy shows (2)

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Inflm changes
Vacolated muscle fibers

Front 30

- Resistant to all therapies
- Can try steroids, immunosuppresants and IgG

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Inclusion Body Myositis

Front 31

Steroid myopathy is the myopathic feature os (2)

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Cushing syndrome
Chronic corticosteroid use

Front 32

Steroid myopathy is more common with _____ (_____) corticosteroids
So may put on alternate day regimen

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Longer acting (fluorinated) corticosteroids

Front 33

Steroid myopathy rarely begins before ______ of steroid therapy and progresses until dosage is reduced

Back 33

3 months

Front 34

Weakness beginning in pelvifemoral muscles
May progress to trunk,neck,arms

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Steroid Myopathy

Front 35

Tx of steroid myopathy (2)

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-Switch to shorter acting preparation
-Alternate day therapy

Front 36

Acute hypokalemic myopathy
K+ < ____ =
Weakness, fatigue, myalgia

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3.5

Front 37

Acute hypokalemic myopathy
K+ &lt; ____ =
Moderate proximal muscle weakness, CPK may increase

Back 37

2.5

Front 38

Acute hypokalemic myopathy
K+<____ =
Proximal limb weakness, muscle pain

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2.0

Front 39

Acute hypokalemic myopathy prognosis

Back 39

Improves within days after K+ replacement

Front 40

Myopathy that could be from a diuretic, n/v, diarrhea

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Acute Hypokalemic Myopathy

Front 41

Delayed motor milestones (don't walk by certain age)
Clumsiness
Progressive weakness

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Duchenne's Muscular Dystrophy

Front 42

Weakness begins in pelvifemoral muscles --> shoulder girdle --> distal and intercostal muscles

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Duchenne's Muscular Dystrophy

Front 43

Usually wheelchair bound by age 7-10
Death by age 20

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Duchenne's Muscular Dystrophy

Front 44

Dz characterized by pseudohypertrophy of calves
--> lg calves compared to rest of atrophic muscle --> this is really fat infiltration

Back 44

Duchenne's Muscular Dystrophy

Front 45

Mild MR
Cardiac involvement --> mortality commonly from cardiomyopathy

Back 45

Duchenne's Muscular Dystrophy

Front 46

Sign in Duchenne's MD (but not specific to it)
- Put child in cross legged position and ask to rise
- Use calves to come up
- Don't straighten b/c too weak, rather crawl hands up to help them straighten

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Gower's sign

Front 47

Sex linked recessive
Gene located on short arm of chromosome X

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Duchenne's Muscular Dystrophy

Front 48

Protein that is absent in Duchenne's
(normally present in all muscle)

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dystrophin

Front 49

In Duchenne's MD, CPK is ______

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Massively elevated
Often 1,000x normal

Front 50

As muscle mass decreases in Duchenne's MD, CPK

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decreases (as oppposed to beginning of dz when it is massively elevated - 1,000x normal)

Front 51

Biopsy of Duchenne's MD (3)

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-Interstitial inflm
- Adipose
- Fibrosis

Front 52

Tx of Duchenne's MD (2)

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- Bio counseling - identify F carriers
- Maintain ambulation as late as possible

Front 53

Muscle stiffness
- aka especially slow relaxation after a voluntary contraction

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Myotonia

Front 54

Repetiive discharge after voluntary ctx which results in delayed or difficult relaxation

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Myotonia

Front 55

EMG of myotonia

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"dive bomber" discharge

Front 56

Autosomal Dominant
Onset usually 20-25
May be congenital or present in middle age

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Myotonic Dystrophy

Front 57

Weakness of this dz
-Primarily hands and feet
- Bulbar: dysarthria, dysphagia, ptosis

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Myotonic Dystrophy

Front 58

Myotonic dystrophy:
- ___________- faces due to temporalis atrophy

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Hatchet facies
(sharp, narrowed facial appearance)

Front 59

Systemic involvement of this dz:
- Smooth muscle: pharynx, esophagus, uterus
-Cardiac - conduction defects, arrhythmias
-Brain: dec IQ
- Endocrine: Testicular atrophy, DM, gynecomastia
-Eye: cataracts
- Skin: frontal balding

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Myotonic Dystrophy

Front 60

Pathology of Myotonic Dystrophy

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Increased internal nuclei, ring fibers

Front 61

Myotonia congenita is different from myotonia dystrophy b/c congenita has

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No muscle weakness

Front 62

-Autosomal recessive or dominant
-Marked muscle hypertrophy
-No weakness or systemic involvement

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Myotonia congenita

Front 63

Autosomal dominant
Onset 5-20yrs
-Facial weakness (difficult eye closure)
-Scapular winging
-Weakness of biceps and triceps with deltoid spared
-Anterior tibial weakness

Back 63

Facioscapulohumeral Dystrophy

Front 64

_________ = weakness/atrophy of biceps and triceps with deltoid spared
- Characteristic of Facioscapulohumeral Dystrophy

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Popeye arm

Front 65

Fascioscapulohumeral Dystrophy CPK = ___-

Back 65

Usually 2-3x normal
(minimal increase)