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MSK II > Peripheral nn and skeletal mm > Flashcards

Peripheral nn and skeletal mm Flashcards

1
Q

inflammatory neuropathies

A
  • Gullian-Barre
  • chronic inflammatory demyelinating polyneuropathy
  • neuropathy associated with systemic autoimmune diseases
  • neuropathy associated with vasculitis
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2
Q

gullian-barre

A

acute-onset immune mediated demyelinating neuropathay

2/3 preceded by acute flu like illness

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3
Q

what infections have been associated with gullian-barre

A

campylobater jejuni
CMV
EBV
mycoplasma pneumoniae

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4
Q

what infiltrates can be seen in nn Bx of gullian-barre

A

lymphocytes

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5
Q

gullian-barre clinical

A

ascending paralysis and areflexia
DTRs disappear early, sensory involvement not as pronounced as motor deficits
nn conduction slowed d/t demyelination
CSF has elevated protein levels

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6
Q

chronic inflammatory demyelinating poly(radiculo)neuropathy

A

most common chronic acquired inflammatory peripheral neuropathy
basically GB that persists for more then 2 months

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7
Q

neuropathy associated with systemic autoimmune diseases

A

RA
sjogrens
SLE
all can have distal sensory or sensorimotor neuropathies

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8
Q

neuropathy associated with vasculitis

A

noninfectious inflammation of vessels damages peripheral nn

polyarthritis nodosum

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9
Q

what is most common cause of peripheral neuropathy

A

DM

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10
Q

DM neuropathy

A

stocking and glove sensory neuropathy -> ulcers and fractures
dysfnx of autonomics: postural hypotension, incomplete emptying of bladder, sexual dysfnx

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11
Q

uremic neuropathy

A

most people with renal failure have peripheral neuropathy, typically distal, symmetric, that may be asymptomatic or associated with mm cramps, dysesthesias, diminished DTRs
axonal degeneration with secondary demyelination
regeneration can occur after dialysis

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12
Q

thyroid disfnx

A

hypothyroidism can lead to compression mononeuropathies such as carpal tunnel or distal symmetric sensory neuropathy
rarely hyperthyroidism can create a GB like syndrome

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13
Q

vit B12 (cyanobalamin) deficiency

A

subacute damage to long tracts of spinal cord and peripheral nn

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14
Q

B1 (thiamine) deficiency, vit B6 (pyridoxine), folate, vit E, copper, and zinc deficiencies

A

all associated with peripheral neuropathy

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15
Q

uremic frost

A

B-1 thiamine deficiency

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16
Q

toxic neuropathies

A

alcohol
heavy metals
organic solvants
chemotherapuetic agents

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17
Q

how does CA cause neuropathies

A

direct infiltration or compression of nn
paraneoplastic syndrome
monoclonal gammopathies

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18
Q

what tumor is known for causing compression of nn

A

pancoast tumor

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19
Q

monoclonal gammopathies

A

Waldonstorms macroglobulinemia in MM

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20
Q

what are some common neuropathies caused by physical forces

A

carpal tunnel

mortons neuroma- seen in dancers

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21
Q

inherited peripheral neuropathies

A

Charcot-marie-tooth disease (sensory)

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22
Q

CMT disease

A

most common inherited peripheral neuropathy

distal mm atrophy, sensory loss and foot deformities

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23
Q

what does amyloidosis look like

A

pink on histo

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24
Q

disease of NMJ

A

present with painless weakness

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25
Q

what is an associated complication of MG

A

thymic abnormalities
30%- thymic hyperplasia
10%- thymoma

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26
Q

signs of skeletal mm atrophy

A

clusters or groups of atrophic fibers seen in neurogenic disease
perifasicular atrophy in dermatomyositis
type II fiber atrophy with sparing of type I with prolonged corticosteroid therapy

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27
Q

segemental myofiber degeneration and regeneration

A

seen when only part of myofiber undergoes necrosis

degeneration is associated with release of cytoplasmic enzymes into blood such as creatine kinase

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28
Q

myofiber hypertrophy

A

physiologic adaptation to exercise or in association with certain chronic myopathic conditons

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29
Q

cytoplasmic inclusions

A

in form of vacuoles, aggregates of proteins or clustered organelles are characteristic of several primary forms of myopathy

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30
Q

inflammatory myopathies

A

polymyositis
dermatomyositis
inclusion body myositis
SLE, systemic sclerosis, and certain infections can cause myositis

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31
Q

what type of infiltrates will you see in mm

A

lymphocytes

32
Q

rodent papules

A

red spots on hands seen in dermatomyositis

33
Q

dermatomyositis

A

proximal mm weakness and skin changes

damage to small blood vessels

34
Q

anti-Mi2 Abs

A

directed against helicase implicated in nucleosome remodeling
strong assocaition with prominent Gottron papules and heliotrope rash

35
Q

Anti-Jo1 Abs

A

directed against histidyl t-RNA synthetase
associated with interstitial lung disease, nonerosive arthritis, and skin rash called mechanics hands
poor prognosis

36
Q

anti-P155/P140 Abs

A

directed against several transcription regulators

associated with pareneoplastic and juvenile dermatomyositis

37
Q

perifascicular inflammation

A

dermatomyocytis until proven otherwise

38
Q

clinical dermatomyocytis

A

mm weakness slow in onset, symmetrical, proximal mm first, accompanied by myalgias
standing up from chair or climbing steps difficult
elevation of serum creatine kinase
rashes
1/3 have dysphagias
10% interstitial lung disease- usually leads to death
cardiac involvement common, but rarely leads to cardiac failure

39
Q

rashes dermatomyocytis

A

helical rash- lilac colored discoloration of upper eyelids with assocaited periorbital edema
gottron papules- scaling erythematous eruption of dusky red patches over knuckles, elbows, and knees

40
Q

polymyositis

A

adult-onset inflammatory myopathy which shares myalgais and weakness with dermatomyositis, but lacks cutaneous features

41
Q

CD68

A

marks presence of macros which should not be seen in mm and indicative of necrosis
seen in polymyositis

42
Q

inclusion body myositis

A

disease of late adulthood >50
most common inflammatory myopathy >65
slowly progressing mm weakness most severe in quads and distal UL

43
Q

inclusion body myositis histo

A

will see inclusions or nuclei in center of mm fiber which is not normal, usually all cell component of mm fiber around periphery

44
Q

Tx of inflammatory myopathies

A

Corticosteroids 1st line
immunosupressives
inclusion body myositis responds poorly to steroids and immunosupression (maybe not an inflammatory disease)

45
Q

toxic myopathies

A

statins
chloroquine and hydroxychloroquine
thrytoxic myopathy
alcohol

46
Q

statins

A

1.5% of users
unrelated to dose or type
must check people after 2 weeks of initiation of drug

47
Q

cholorquine and hydroxychloroquine

A

used as antimalarials and can be used in systemic autoimmune diseases

48
Q

thyrotoxic myopathy

A

presents as acute or chronic proximal mm weakness w/associated hyperthyroid symptoms

49
Q

alcohol

A

binge drinking can produce acute toxic rhabdomyolysis, myoglobuinuria, and renal failure

50
Q

central core disease

A

AD
RYR1 mutation
floppy baby
scoliosis, hip dislocation, foot deformities
normal sacromeres disrupted with increases mito

51
Q

nemaline myopathy (NEM)

A

floppy baby
childhood weakness
aggreagates of spindal shapred partcles (nemoline rods) in type 1 fibers
gomori stain

52
Q

centronuclear myopathy

A

severe congenital hypotonia-floppy baby - poor prognosis

childhood onset or adult onset can also occur

53
Q

x-linked muscular dystrophies

A

Duchenne and Becker
both mutations of dystophin gene
duchenne worse

54
Q

duchenne and becker

A

both marked by chronic mm damage that outpaces capacity for repair

55
Q

duchenne and becker mm Bx

A

segemental myofiber degenreation and regeneration w/admixture of atrophic myofibers
usually no inflammation
with progression mm replaced with fat

56
Q

duchenne and becker mm histo

A

duchenne stain for dystophin will show thats its completely absent, beckers, just deficient

57
Q

duchenne and becker clincial

A

normal at birth
very early motor milestones mets, but walking dealyed
weakness in pelvic girdle mm -> shoulder girdle -> pseudohypertrophy
mean onset of wheel chair dependance 9.5
eventually have respiratory failure

58
Q

Dx duchenne or becker

A

serum creatine kinase markedly elevated in 1st decade of life then falls as mm mass is lost
can be confirmed with genetics studies

59
Q

myotonic dystrophy

A

AD multisystem disorder assocaited with skeletal mm weakness, cataracts, endocrinopathies, and cardiomyopathies
can be congenital with manifestations in infancy

60
Q

cause of myotonic dystophy

A

expansions of CTG triplet repeats on DMPK gene

61
Q

diseases of lipid or glycogen metabolism

A

produce one of two patterns of mm dysfnx:

  • symptomatic only with exercise or fasting producing mm cramping, pain, or rhabdomyolysis
  • slowly progressive mm damage w/o episodic manifestations
62
Q

mito myopathies

A

skeletal mm involvement can manifest as weakness, elevations in serum creatine kinase or rhabdomyolysis

63
Q

ragged red fibers

A

markers of mito disease

64
Q

lipid myopathies

A

mm Bx normal w/o special staining

65
Q

buzz words for mito disease

A

ragged red fibers
phonograph records
rhomboid paracrystalline inclusions

66
Q

peripheral nn sheath tumors

A

schwannoma
neurofibroma
malignatn peripheral nerve sheath tumor (MPNST)

67
Q

familial tumor syndromes of peripheral nn sheath tumors

A

NF1
NF2
schwan-nomatosis

68
Q

schwannomas

A

benign tumors that exhibit Schwann cell differentiation often arise directly from peripheral nn
loss of NF2 gene whose product is merlin in ALL schwannomas

69
Q

buzz words for schwannomas

A

antoni A areas and antoni B areas
verocay bodies
carrot shavings

70
Q

schwannomas clinical

A

symptoms dt local compression

commonly occur at cerebellopotine angle -> compress CNVIII and commonly referred to as acoustic neuroma

71
Q

neurofibromas

A 
benign n sheath tumors more  heterogeneous in composition than schwannomas admixed with:
perineurial like cells
fibroblasts
mast cells
CD34+ spindle cells
72
Q

superficial cutaneous neurofibromas

A

often present as peduculated nodules that can be seen isolated if sporadic or multiple when associated with NF1

73
Q

diffuse neurofibromas

A

often present as large plaque like elevation of skin and typically assocaited with NF1

74
Q

plexiform neurofibromas

A

can be found in deep or superficial locations in association with nn roots or large nn and are uniformly NF1 associated

75
Q

MPNST

A

85% high grade
about 50% in NF1 pts
malignant transformation of plexiform neurofibroma
divergent differentiation

76
Q

triton

A

MPNST tumor with divergent differentiation

can have glandular, cartilaginous, osseous, or rhadomyoblastic tissue

MSK II (19 decks)

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