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MSK Exam 1 Spring 2015 > Skeletal Muscle and Peripheral Nerve Pathology II > Flashcards

Skeletal Muscle and Peripheral Nerve Pathology II Flashcards

1
Q

botulism

A

clostridium botulinum neurotoxin

-blocks release of ACh from presynaptic neurons

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2
Q

canned food

A

botulism

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3
Q

double vision, blurry vision, slurred speech, difficulty swallowing, dry mouth, muscle weakness (shoulders to body)

A

botulism

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4
Q

type I muscle fibers

A

aerobic
high lipid
oxidative

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5
Q

one slow fat red ox

A 
type 1 muscle fiber
slow - aerobic exercise
high lipid
red colored - high myoglobin
oxidative
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6
Q

ATPase at differnt pH levels

A

shows type 1 or type 2 fibers - in checkerboard pattern

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7
Q

perifascicular atrophy

A

dermatomyositis

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8
Q

corticosteroid use

A

type 2 fiber atrophy and sparing of type 1 fibers

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9
Q

reinnervation

A

switch in fiber type

increase in motor unit size - more myofibers innervated by individual axon

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10
Q

type grouping

A

of muscle fibers
-with ongoing denervation and reinnervation

loss of checkerboard

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11
Q

skeletal m disorder

A

either myopathy or neurogenic issue

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12
Q

neurogenic injury

A

fiber type grouping and grouped atrophy**

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13
Q

creatine kinase

A

released with segmental myofiber degeneration

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14
Q

exercise

A

myofiber hypertrophy

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15
Q

cytoplasmic inclusions

A

seen in several primary forms of myopathy

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16
Q

three primary inflammatory myopathies

A

polymyositis
dermatomyositis
inclusion body myositis

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17
Q

lymphocytes in atrophic muscle fibers

A

inflammatory myopathy

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18
Q

dermatomyositis

A

systemic autoimmune disease
proximal muscle weakness and skin changes

swollen hands and lesions on knuckles

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19
Q

dermatomyositis autoAbs

A

anti-M2 - gottron papule and heliotrope rash (around eyes)
anti-Jo1 - lung disease, nonerosive arthritis, mechanic hand rash
anti-P155/P140 - paraneoplastic anc juvenile

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20
Q

gottron papules and heliotrope rash

A

dermatomyositis hand lesions

-with anti-Mi2

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21
Q

interstitial lung disease, nonerosive arthritis, mechanic hand rash

A

dermatomyositis

-with anti-Jo1 - worse prognosis

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22
Q

paraneoplastic and juvenile dermatomyositis

A

anti-P155/P140

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23
Q

perifascicular atrophy

A

dermatomyositis

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24
Q

proximal muscle weakness, myalgias, elevation in serum creatine kinase, heliotrope rash, gottron papules, dysphagia

A

dermatomyositis

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25
Q

heliotrope rash

A

around eyes

-with dermatomyositis

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26
Q

association with dermatomyositis

A

interstitial lung disease

rapidly progressive and can lead to death

also - cardiac involvement

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27
Q

polymyositis

A

like dermatomyositis - but no skin lesions

has myalgia and weakness

inflammation in muscles

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28
Q

endomysial mononuclear inflammatory cells in muscles

A

polymyositis

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29
Q

age of dermatomyositis and polymyositis

A

30-40yo

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30
Q

age of inclusion body myositis

A

> 60yo

31
Q

quadriceps progressive muscle weakness in patient 65yo

A

inclusion body myositis

32
Q

nuclei in middle of muscle cell

A

inclusion body myositis

33
Q

tx of polymyositis and dermatomyositis

A

corticosteroids

34
Q

tx of inclusion body myositis

A

respond poor to steroids/immunosuppressants

35
Q

common complication of statins

A

myopathy

within 6 weeks - check CK levels
if weakness - RTO for eval

36
Q

toxic myopathies

A

statins
chloroquine, hydroxychloroquine
thyrotoxic
alcohol

37
Q

acute toxic rhabdomyolysis, myoglobinuria, renal failure

A

with binge drinking alcohol

38
Q

ethanol myopathy

A

type II fibers

39
Q

inherited skeletal m disease

A

RyR - malignant hyperthermia

AD NEM and AR NEM - childhood weakness - floppy infant

XL, Xq28, AD, and AR - severe congenital hypotonia - floppy infan and poor prognosis with X-linked form

40
Q

malignant hyperthermia

A

mutation in RyR

41
Q

muscular dystrophies

A

most X-linked

mutations in dystrophin**

42
Q

duchenne and becker muscular dystrophy

A

differ in amount of loss of dystrophin function

both LOF mutations of dystrophin - on X chromosome

over time - disease progression - fatty replacement

43
Q

dystrophin

A

mutated in muscular dystrophy

44
Q

dystrophin stain

A

absent in duchenne

reduced in becker

45
Q

walking delayed and can’t keep up with peers

A

duchenne dystrophy

see marked elevation of creatine kinase during first decade of life - then falls as muscle mass loss

46
Q

pseudohypertrophy

A

enlargement of lower leg muscles with weakness

-fat replacement

47
Q

crawl around, hands to stand up, hands on hip to stand

A

muscular dystrophy - duchennes or becker

48
Q

myotonic dystrophy

A

auto dominant

-skeletal m weakness, cataracts, endocrinopathy, cardiomyopathy

49
Q

myotonia

A

sustained involuntary contraction of muscles

50
Q

mutation in myotonic dystrophy

A

expansion of CTG triple repeats in 3’ non-coding region of DMPK gene

51
Q

association with myotonic dystrophy

A

hirsutism

52
Q

symptoms with exercise or fasting

A

lipid or glycogen metabolism diseases

53
Q

bad with exercise

A

mitochondrial myopathies

54
Q

mcardles disease

A

can’t break down sugars

55
Q

ragged red fiber and phonograph record

A

myopathies due to inborn errors of metabolism

56
Q

DDx for infantile hypotonia

A

primary disease of skeletal muscle
abnormality of brain
neuronopathy - spinal muscular atrophy

57
Q

spinal muscular atrophy

A

rare hypertrophy of muscle fibers with surrounding atrophic fibers

58
Q

periodic paralysis

A

ion channel myopathies

can be hyperK, hypoK, or normoK

59
Q

RYR1 mutation

A

malignant hyperthermia
-anesthetic trigger hypermetabolic state

tetany and excessive heat production

60
Q

rimmed vacuoles

A

inclusion body myositis

61
Q

MPNST

A

malignant peripheral nerve sheath tumor

62
Q

peripheral nerve sheath tumor

A

schwannoma
neurofibroma
malignant peripheral nerve sheath tumor

63
Q

loss of merlin

A

schwannoma

64
Q

schwannoma

A

benign

cell differentiation and often arise from peripheral nerves

65
Q

schwannoma histo

A

dense eosinophilic antoni A areas and loose pale antoni B areas

also hyalinized blood vessels

66
Q

veracay bodies

A

antoni A area where tumor cell nuclei align with areas of anuclear zone

in schwannoma

67
Q

tinnitus and hearing loss

A

schwannoma at CPA - cerebellopontine angle

CN VIII

aka acoustic neuroma

68
Q

neurofibroma

A

benign nerve sheath tumor

heterogenous composition

neoplastic schwann cells admixed with perineural like cells, fibroblasts, mast cells, CD34 spindle cells

69
Q

NF1

A

associated with neurofibromas

multiple** superficial cutaneous neurofibromas
diffuse neurofibroma - large plaquelike elevation of skin
plexiform - deep - nerve roots or large nerves

70
Q

MPNST

A

high grade tumors

divergent differentiation - focal areas that exhibit other lines of differentiation

triton tumor

71
Q

triton tumor

A

MPNST

malignant peripheral nerve sheath tumor

72
Q

cafe au lait spots

A

with neurofibromatosis type I

73
Q

b/l CN VIII schwannoma and multiple meningiomas

A

neurofibromatosis type II

74
Q

NF2

A

associated with schwannomas

MSK Exam 1 Spring 2015 (21 decks)

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