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Pathology II Exam 3 > 11 Neuropath Muscle and Nerve > Flashcards

11 Neuropath Muscle and Nerve Flashcards

1
Q

T-F– all muscle fibers in the motor unit are the same type and do not mix with other motor unit fibers?

A

False- same type per motor unit, but muscle fibers from diff. motor units can intermix with each other.

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2
Q

T-F– all muscle fibers in the motor unit are the same type and do not mix with other motor unit fibers?

A

False- same type per motor unit, but muscle fibers from diff. motor units can intermix with each other.

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3
Q

What are the 2 types of axonal degeneration?

A
  1. Classic Wallerian degeneration

2. Distal axonopathy

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4
Q

What are the 2 main causes of classic wallerian degeneration?

A

Trauma and vascular disease

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5
Q

What causes distal axonopathy? 3

A
  1. systemic metabolic disease
  2. toxins
  3. Some genetic diseases
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6
Q

What are the two types of peripheral neuropathy?

A

axonal degeneration and demyelination

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7
Q

Review the 4 main types of axonal degeneration- ischemic neuropathies?

A
  1. Vasculitis
  2. Collagen VascularDisease
  3. Paraneoplastic Microvasculitis
  4. Diabetic symmetrical sensorimotor neuropathy
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8
Q

What are we looking for in vasculitis on microscopic imaging?

A

fibrinoid necrosis of vessel walls

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9
Q

What is distinct about vasculitic neuropathy that we can see on microscopic imaging?

A
  1. fascicle to fascicle variation in degree of axon loss

[in the image, the one with the most loss has less black looking axons)

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10
Q

What is distinct about vasculitic neuropathy that we can see on microscopic imaging?

A
  1. fascicle to fascicle variation in degree of axon loss

[in the image, the one with the most loss has less black looking axons)

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11
Q

What type of neuropathy has both metabolic and toxic neuropathy?
is it predominantly axonal degeneration or demyelination?
What type of ischemic component do we see?

A
  1. diabetic neuropathy\
  2. axonal degeneration
  3. vascular hyalinization- endoneurial microvasculature thickening
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12
Q

What type of neuropathy has both metabolic and toxic neuropathy?
is it predominantly axonal degeneration or demyelination?
What type of ischemic component do we see?

A
  1. diabetic neuropathy\
  2. axonal degeneration
  3. vascular hyalinization- endoneurial microvasculature thickening
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13
Q

On microscopic view of diabetic neuropathy, what do we readily see?

A

Axonal loss (lack of black circular axons) and vascular thickening.

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14
Q

On microscopic view of diabetic neuropathy, what do we readily see?

A

Axonal loss (lack of black circular axons) and vascular thickening.

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15
Q

What neuropathy has a segmental pattern of injury? What is the etiology 3?

A
  1. demyelinating neuropathy

2. Inflammatory (guillain-barre), Toxins, Genetics (Charcot-marie-tooth)

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16
Q

The genetic disease charcot-marie-tooth disease causes what type of neuropathy?

A

demyelinating- segmental

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17
Q

What 4 things are found in Guillain-barre syndrome clinical presentation?

A
  1. Ascending Paralysis
  2. Can involve respiratory muscles
  3. slowed conduction
  4. elevated CSF protein
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18
Q

What 4 things are found in Guillain-barre syndrome clinical presentation?

A
  1. Ascending Paralysis
  2. Can involve respiratory muscles
  3. slowed conduction
  4. elevated CSF protein
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19
Q

Is guillain barre a antibody mediated disease or T-cell mediated>

A

T-cell mediated with macrophages

[endoneurial and perineurial]

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20
Q

T-F- demyelination of naked axons results in a thicker sheath?

A

Fasle- thinner and internal length changes

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21
Q

T-F- demyelination of naked axons results in a thicker sheath?

A

Fasle- thinner and internal length changes

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22
Q

Cross section microscopy of a segment of nerve undergoing demyelination will show what?

A

myelin debris surrounding a naked axon

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23
Q

What is the most common hereditary peripheral neuropathy?

A

hereditary motor and sensory neuropathy type 1- charcot-marie-tooth disease

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24
Q

What is the most common hereditary peripheral neuropathy? Where do we see muscular atrophy?

A
  1. hereditary motor and sensory neuropathy type 1- charcot-marie-tooth disease
  2. progressive atrophy of calves-weakness
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25
Q

What disease has repetitive demyelination and remyelination?

A

hereditary motor and sensory neuropathy type 1- charcot-marie-tooth disease

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26
Q

What disease has repetitive demyelination and remyelination?

A

hereditary motor and sensory neuropathy type 1- charcot-marie-tooth disease

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27
Q

In charcot marie tooth, what do we see on microscopic pathology?

A
  1. Thinly myelinated axons

2. Hypertrophic “onion-bulb” neuropathy

28
Q

In charcot marie tooth, what do we see on microscopic pathology?

A
  1. Thinly myelinated axons

2. Hypertrophic “onion-bulb” neuropathy

29
Q

Chronic demyelination and demyelination results in what?

A

onion-bulbs- concentric rings of schwann cell processes.

30
Q

Chronic demyelination and demyelination results in what?

A

onion-bulbs- concentric rings of schwann cell processes.

31
Q

What muscle fiber type is for sustained force and weight bearing? What is the nadh and atpase content? are lipids or glycogen abundant? slow twitch or fast twitch? color?

A
  1. Type 1
  2. NADH dark, ATPase light
  3. lipids are abundant
  4. slow-twitch
  5. red
32
Q

What muscle fiber type is for sustained force and weight bearing? What is the nadh and atpase content? are lipids or glycogen abundant? slow twitch or fast twitch? color?

A
  1. Type 1
  2. NADH dark, ATPase light
  3. lipids are abundant
  4. slow-twitch
  5. red
33
Q

What muscle fiber type is for sudden movement? What is the nadh and atpase content? are lipids or glycogen abundant? slow twitch or fast twitch? color?

A
  1. Type II
  2. NADH light, ATPase dark
  3. Glycogen are abundant
  4. fast twitch
  5. white
34
Q

What muscle fiber type is for sudden movement? What is the nadh and atpase content? are lipids or glycogen abundant? slow twitch or fast twitch? color?

A
  1. Type II
  2. NADH light, ATPase dark
  3. Glycogen are abundant
  4. fast twitch
  5. white
35
Q

When we stain muscle fibers for ATPase…what type of fibers are dark?

A

Type II– (remember that their overall color is white but they are ATPase dark and TYPE I are atpase light and appear whiter)

36
Q

When we stain muscle fibers for ATPase…what type of fibers are dark?

A

Type II– (remember that their overall color is white but they are ATPase dark and TYPE I are atpase light and appear whiter)

37
Q

What are the two main classifications of muscle disease?

A

Neurogenic and myopathic

38
Q

What are the two main classifications of muscle disease?

A
  1. Neurogenic [motor neuron disease, peripheral neuropathy, neuromuscular junction]
  2. myopathic [dystrophy, inflammatory, metabolic, congenital]
39
Q

What are the two main classifications of muscle disease?

A
  1. Neurogenic [motor neuron disease, peripheral neuropathy, neuromuscular junction]
  2. myopathic [dystrophy, inflammatory, metabolic, congenital]
40
Q

In neurogenic muscle disease where is the site of weakness? is there pain? fasciculations? serum enzyme changes? EMG signs? nerve conduction?

A
  1. distal
  2. absent
  3. present
  4. normal
  5. EMG fibrillation potentials
  6. Variable conduction
41
Q

In myopathic muscle disease where is the site of weakness? is there pain? fasciculations? serum enzyme changes? EMG signs? nerve conduction?

A
  1. proximal
  2. variable
  3. absent
  4. elevated
  5. EMG polyphasic potentials
  6. normal
42
Q

In myopathic muscle disease where is the site of weakness? is there pain? fasciculations? serum enzyme changes? EMG signs? nerve conduction?

A
  1. proximal
  2. variable
  3. absent
  4. elevated
  5. EMG polyphasic potentials
  6. normal
43
Q

What is the difference in histological atrophy between neuropathy and myopathy?

A

in neuropathy the fibers are angular and in groups, while in myopathy the fibers are rounded and scattered.

44
Q

What is the difference in histological atrophy between neuropathy and myopathy?

A

in neuropathy the fibers are angular and in groups, while in myopathy the fibers are rounded and scattered.

45
Q

What type of muscle disease results in fiber type grouping

A

neuropathy- nerve loss is followed by regeneration leads to larger motor units and fiber type grouping

46
Q

Review the myopathic diseases-

A

Dystrophy= duchenne, Becker, limb-girdle, myotonic.

Metabolic=mitoch. myopathy, inborn errors of metabolism, hormonal abnormalities, alcohol rhabdo

Inflammatory= polymyosits, dermatomyositis, inclusion body myositis

Congenital= centronuclear myopathy, nemaline myopathy, central core disease

47
Q

Review the myopathic diseases-

A

Dystrophy= duchenne, Becker, limb-girdle, myotonic.

Metabolic=mitoch. myopathy, inborn errors of metabolism, hormonal abnormalities, alcohol rhabdo

Inflammatory= polymyosits, dermatomyositis, inclusion body myositis

Congenital= centronuclear myopathy, nemaline myopathy, central core disease

48
Q

Is duchenne’s x linked?

A

YEs

49
Q

What does dystrophin do?

A

links cytoplasmic actin to the cytoskeleton

50
Q

What do we see histologically in muscular dystrophy early in the disease?

A

myocyte necrosis

with atrophy and hypertrophy

51
Q

What do we see histologically in muscular dystrophy early in the disease?

A

myocyte necrosis

with atrophy and hypertrophy

52
Q

What do we see histologically in muscular dystrophy late in the disease?

A

endomysial fibrosis and fatty replacement

53
Q

What do we see histologically in muscular dystrophy late in the disease?

A

endomysial fibrosis and fatty replacement

54
Q

What disease is autosomal dominant, trinucleotide, and has multiple internal nuclei and occasional ring fibers?

A

myotonic dystrophy

55
Q

What do we see histologically in polymyositis early in the disease?

A

inflammation and muscle fiber necrosis

56
Q

What do we see histologically in polymyosits later in the disease?

A

regenerating muscle, inflammation, and scarring fibrosis

57
Q

What do we see histologically in polymyosits later in the disease?

A

regenerating muscle, inflammation, and scarring fibrosis

58
Q

What disease is described as gradual onset, male predominance after age 50. proximal and distal weakness. Lymphocystic inflammation, angular and round atrophy, autophagic “rimmed” vacuoles?

A

inflammatory myopathy- inclusion body myositis

59
Q

What do the rimmed vacuoles look like on stain?

A

stain is blue overall, clear vacuoles with purple surrounding

60
Q

What do the rimmed vacuoles look like on stain?

A

stain is blue overall, clear vacuoles with purple surrounding

61
Q

pompe’s deficiency is in what enzyme? McArdles disease?

A
  1. lysosomal a1,4-glucosidase

2. myophosphorylase

62
Q

What is highly indicative of mitochondrial myopathy?

A

ragged red fibers

63
Q

How many mitochondrial genes are there?

A

37
22 tRNA
2 ribosomal RNA
13 polypeptide subunits of respiratory chain

64
Q

How many mitochondrial genes are there?

A

37
22 tRNA
2 ribosomal RNA
13 polypeptide subunits of respiratory chain

65
Q

What are the ragged red fibers?

A

areas of mitochondrial proliferation with paracrystalline inclusions

Pathology II Exam 3 (13 decks)

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