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Neuro > Skeletal Muscle & PNS pathology > Flashcards

Skeletal Muscle & PNS pathology Flashcards

1
Q

What indicates denervatoin with renervation?

A

grouping of muscle fibers

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2
Q

When does muscle hypertrophy occur?

A

secondary to increased load due to exercise or pathologic conditions

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3
Q

What does muscle degeneration/necrosis cause?

A

destruction of all or part of a myofiber, stimulates infiltration by macrophages

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4
Q

How does regeneration of muscle occur?

A

satellite cells around degenerated fiber proliferate, regenerating fiber has blueish color & enlarged nuclei

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5
Q

What does hypertrophied muscle look like on biopsy?

A

rounder/less polygonal

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6
Q

What changes occur due to denervation atrophy?

A

clusters of fibers of one type become smaller and develop angular contours (group atrophy)

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7
Q

What does lack of neural input result in on the cellular level?

A

breakdown of myosin and actin
resorption of myofibrils
decrease in cell size

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8
Q

How does fiber type grouping occur?

A

with reinnervation neighboring axons sprout and reinnervate denervated myocytes, fiber assumes the fiber type conferred by the neighboring axonW

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9
Q

What are the major differences between neurogenic and myopathis path?

A

neurogenic: bimodal size dist, angulated fibers, apparent increase in nuclei, no necrosis
myopathic: random size variation, round fibers, centralization of nuclei, necrosis

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10
Q

Is ALS an UMN or LMN problem?

A

both!!

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11
Q

Why does weakness occur in ALS?

A

loss of alpha motor neurons

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12
Q

What are signs/sx of ALS?

A 
flail arm
dysphagia/dysarthria/tongue atrophy
fasciulations
hyperreflexia
positive babinski
spasticity
pseudobulbar palsy
executive dysfunction
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13
Q

Is SMA an UMN or LMN problem?

A

LMN

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14
Q

How is SMA inherited?

A

Autosomal recessive

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15
Q

What gene is deleted in SMA?

A

SMN1 (survival motor neuron) gene

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16
Q

Where is there the most muscle weakness in SMA?

A

proximal muscles

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17
Q

What leads to death in SMA?

A

respiratory weakness/hypercapnea

diaphragm fails, intercostal weakness

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18
Q

What is seen on biopsy in SMA?

A

large groups of rounded atrophic fibers and sparse, scattered, markedly hypertrophic fibers

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19
Q

What is the difference between Duchenne & becker muscular dystrophy?

A

duchene’s–>none/nearly no dystrohpin

becker’s–>abnormal dystrophin in reduced quantity (or normal dystrophin in reduced quantity)

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20
Q

What gene is mutated in duchenne/becker MD?

A

Xp21

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21
Q

What are signs/sx of duchenne’s MD?

A 
proximal muscle weakness
slower movement than peers
large calves
gower's maneuver
face/eyes spared
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22
Q

What CK value would you expect to find in DMD?

A

> 10,000

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23
Q

What are signs/sx of becker’s MD?

A

late onset

similar to duchenne’s

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24
Q

What is the genetic problem in myotonic dystrophy?

A

CTG repeat expression in DMK gene on chr 19

DM1 dystrophia myotonica type 1

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25
Q

Who gets myotonic dystrophy?

A

adults

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26
Q

How is myotonic dystrophy inherited?

A

autosomal dominant

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27
Q

What are signs/sx of myotonic dystrophy?

A

difficulty releasing grip—>myotonia
foot drop
temporal wasting/elongated face
endocrine abnormalities (test atrophy, balding, Diabetes)
premature cataracts
cardiac arrhythmias
the higher the repeat number the lower the intelligence

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28
Q

What characteristic sound is heard on EMG of a myotonic dystrophy patient?

A

divebomber

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29
Q

What does myotonic dystrophy have in common with huntington’s disease?

A

genetic anticipation

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30
Q

What does MELAS stand for?

A 
mitochondrial myopathy
encephalopathy
lactic 
acidosis
stroke-like episodes
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31
Q

What causes melas?

A

MtDNA mutation

32
Q

What is a myopathic form of a glycogen storage disease? What causes it?

A

V-McArdle’s disease

phosphorylase deficiency

33
Q

What are the clinic sx of V-McArdle’s disease?

A

exercise intolerance with cramps and intermittent myoglobinuria

34
Q

What is a generalized form of a glycogen storage disease? What causes it?

A

Pompe’s disaese

acid maltase deficiency

35
Q

What do you see microscopically with pompe’s disease?

A

vacuoles

36
Q

What are the 3 inflamatory myopathies?

A

inclusion body myositis
dermatomyositis
polymyositis

37
Q

What are the clinical findings of polymyositis?

A

symmetric proximal muscle weakness
no rash
F>M
assoc w/other autoimmune diseses

38
Q

What cell type is dominent in the inflammation of polyomyositis?

A

CD8 T cells (endomysial infiltrattes)

39
Q

What is the pathology of dermatomyositis?

A

Microvasculature is attacked by antibodies and complement
B cells predominate
Ischemic atrophy and necrosis (perifascicular atrophy)

40
Q

What are signs/sx of dermatomyositis?

A 
shawl sign
heliotrope (violaceous) rash around eyes
nail bed with thrombi and hemorrhages
gottron's papules 
subcutaneous calcifications
proximal weakness
41
Q

What is sign microscopically with dermatomyositis?

A

chronic inflammation
perifascicular atrophy
perivascular, perimysial and endomysial infiltrates

42
Q

Which inflammatory myopathies respond to immunosuppression?

A

Dermatomyositis and polymyositis

43
Q

What conditions are associated with polymyositis?

A

SLE
PAN
scleroderma

44
Q

What conditions are associated with dermatomyositis?

A

myocarditis
interstitial lung disease
malignancy
vasculitis

45
Q

What are group is affected most my dermatomyositis?

A

children & adults

46
Q

Is there a rash associated with IBM?

A

no

47
Q

What is seen microscopically with IBM?

A 
endomysial infiltrates (CD8)
rimmed vacuoles
amyloid depositis
invasion of non-necrotic fibers 
tubulofilamentous inclusions
48
Q

What is the pattern of weakness in IBM?

A

proximal=distal
deep finger flexors
quads
dysphagia

49
Q

Describe steroid induced myopathy?

A

proximal weakness
high dose, prolonged steroid use
type 2b atrophy

50
Q

What type of myopathy is induced by statins?

A

necrotizing immune mediated

51
Q

What is neuronopathy/axonopathy?

A

generalized abnormality affecting neuronal cell body and or axon

52
Q

What is wallerian degeneration?

A

focal lesion causing axonal injury

portion of fiber (axon and myelin) distal to focal injury degenerates (ie transection)

53
Q

What is another name for acute inflammatoroy demyelinating polyradiculoneuropathy?

A

guillain barre syndrome

54
Q

What are the symptoms of AIDP?

A 
rapidly ascending weakness 
respiratory weakness
bulbar weakness
autonomic-cardiac rhythm
preceeding viral illness
areflexia
55
Q

What is seen in the CSF of AIDP & CIDP patients?

A

high protein, normal cells, cytoalbuminologic dissociation

56
Q

What do both AIDP and CIDP show on EMG/NCS?

A

demyelinating features

57
Q

What are the symptoms of CIDP?

A

slowly progressive weakness, moving from proximal to distal

no preceeding illness

58
Q

Is AIDP or CIDP monophasic?

A

AIDP

59
Q

How is AIDP treated?

A

IVIG

PLEX

60
Q

How is CIDP treated?

A

Prednisone
IVIG
PLEX

61
Q

Describe mononeuritis multiplex/vasculitis?

A

peripheral nerves are infarcted leading to foot drop and hand weakness

62
Q

What is seen on biopsy for mononeuritis multiplex?

A

inflammation of nerve

vascular necrosis

63
Q

What causes leprosy?

A

mycobacterium leprae

64
Q

What form of leprosy develops if patient mounts a T cell response?

A

tuberculoid

65
Q

What happens when mycobacterium leprae invade the nerves and skin?

A

they cause inflammation and caseating granulomas

66
Q

What is herpes zoster?

A

a VZV reactivation

67
Q

Where does VZV remain dormant?

A

satellite cells, DRG

68
Q

How is pain distributed in herpes zoster?

A

dermatomal distribution

69
Q

What is an example of a hereditary neuropathy?

A

charcot marie tooth type 1A

70
Q

What mutation causes CMT1A?

A

PMP22 mutation, Autosomal dominant

71
Q

What are the signs/sympomts of CMT1A?

A 
onset early teens
demyelinating disease
foot drop
"inverted champagne bottle/stork legs"
tripping, falling, clumsy walk
claw hands
ataxia, loss of proprioception
pes cavus, hammer toes
72
Q

What is the key histologic finding for CMT1A?

A

onion bulbs

73
Q

How does diabetic neuropathy present?

A

stocking/glove distribution
OR
diabetic amyotrophy (lumbosacral plexus lesion -> quad atrophy)

Both: painful, tingling, numbness, foot drop, proprioceptive loss

74
Q

What nerve is impinged in carpal tunnel syndrome?

A

median

75
Q

What is a Morton’s neuroma/traumatic neuroma?

A

intraneural scarring

caused by fracture, compression or surgical lesion of nerve

76
Q

what are the symptoms of morton’s neuroma?

A

persisten, incapacitating pain & tingling at site of injury

Neuro (14 decks)

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