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System Pathology I -- Roecker > Exam 2: PNS Pt2 > Flashcards

Exam 2: PNS Pt2 Flashcards

1
Q

What are the two primary muscle types skeletal muscle is composed of?

A 
Type I (slow twitch)
Type II (fast twitch)

are intermixed in “checkerboard” pattern

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2
Q

What is the general term for primary muscle disease? How does it manifest?

A

myopathy; most likely to cause segmental necrosis and regeneration of ind. muscle fibers

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3
Q

What is the general term for secondary muscle disease due to disrupted innervation? How does it manifest?

A

neuropathy; cause grouped atrophy and fiber type grouping

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4
Q

What does the loss of normal “checkerboard” pattern of muscle fibers and the result of larger, but fewer motor units tell us?

A

the case of myathenia is due to Neuropathic changes

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5
Q

What will both neuropathies and myopathies possibly involve?

A
  • muscle tissue atrophy
  • fibrosis
  • fatty replacement (fibrofatty infiltrate)
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6
Q

What does the presence of fibrofatty infiltrate indicate in skeletal muscle tissue disorders?

A

that the causative pathology is chronic

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7
Q

What follows prolonged disuse of skeletal muscle tissue and primarily involves atrophy of Type II muscle fibers? What are frequent causes of this?

A

Disuse atrophy

casting, bed rest, pareplegia or quadraplegia

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8
Q

What may develop in patients with elevated glucocorticoid (corticosteroid) levels

A

Glucocorticoid atrophy; most likely cause atrophy of type II muscle fibers (due to hypercortisolism)

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9
Q

What is defined by the presence of hypercorticolism that involves “wasting” (atrophy) of musculature in extremities, due to glucocorticoid atrophy?

A

Cushing Syndrome (typically due to corticoidsteroid meds or tumor of pit. gland = Cushing disease, or adrenal glands)

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10
Q

Who does Cushing syndrome characteristically affect?

A

young adult females

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11
Q

What are features of Cushing Syndrome?

A
  • moon face
  • truncal obesity and purple striae
  • insomnia
  • hirsutism
  • “buffalo hump”
  • osteoporosis
  • mood swings, depression, psychosis
  • menstrual abnorms, or infertility
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12
Q

What represents a group of related genetic disorders that interfere with productions of proteins needed to produce and maintain healthy muscle mass?

A

Muscular Dystrophy

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13
Q

What do conditions that fall under the category of Muscular Dystrophy manifest like?

A

progressive weakness and muscle atrophy, primarily w/in skeletal muscle tissue, but cardiac muscle tissue may also be involved

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14
Q

What is a form of muscular dystrophy that results from X-linked mutations in dystrophin gene (DMD gene)?

A

Dystrophinopathies

codes for protein Dystrophin

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15
Q

What does the protein dystrophin function to do?

What occurs if it is absent?

A

functions to stabilize muscle fibers during contraction

muscles have reduced stiffness, and are more prone to tearing, and overall stability is reduced

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16
Q

T/F. Dystrophinopathies cause segmental necrosis of myocytes.

A

true

causing creatine kinase levels in blood to rise

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17
Q

What may the severe atrophy ass. with Dystrophinopathies be masked by?

A

due to ingrowth of fibrofatty infiltrate

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18
Q

What are two kinds of Dystrophinopathies?

A
  1. Duchenne Muscular Dystrophy (DMD)

2. Becker Muscular Dystrophy (BMD)

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19
Q

What is the most common form of muscular dystrophy? How many ppl does it affect?

A

Duchenne Muscular Dystophy (DMD)

~1 in 3,500 males

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20
Q

What is Gower sign characteristic of?

A

Duchenne Muscular Dystrophy (DMD)

–must use upper extremity to assist in standing from a seated or squatting position (due to lower extremity and pelvic girdle weakness)

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21
Q

What develops from an X-linked recessive DMD mutation that causes absence of dystrophin, and is the most severe?

A

Duchenne Muscular Dysrophy (DMD)

absence of dystrophin causes muscle tissues to breakdown at a faster rate than can be repaired

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22
Q

What develops from an X-linked recessive DMD mutation that causes absence of dystrophin, and is less severe, progressing at a slower rate?

A

Becker Muscular Dystrophy (BMD)

-manifests in late childhood or young adulthood

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23
Q

T/F. Duchenne Muscular Dystrophy is a mutation that is congenital and therefore manifests at birth.

A

False. Yes, it is congenital mutation, but it manifests until childhood

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24
Q

What disorder appears in childhood and the child has an “inability to keep up with peers”, pelvic girdle weakness, and lower extremity weakness, and has calf muscle pseudohypertrophy?

A

Duchenne Muscular Dystrophy (DMD)

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25
Q

What disorder in advanced stages has shoulder girdle weakness and cardiorespiratory failure and is wheelchair-bound by adolescence?

A

Duchenne Muscular Dystrophy (DMD)

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26
Q

Is Duchenne Muscular Dystrophy (DMD) fatal? If so how?

A

yes, uniformily fatal by young adulthood

due to complications from cardiorespiratory failure

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27
Q

What are reasons for cardiorespiratory failure?

A
  • lethal cardiomyopathy
  • cardiac decompensation (mimic CHF)
  • prolonged arrhymia
  • pneumonia
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28
Q

T/F. Becker Muscular Dystrphy (BMD) is more common than Duchenne Muscular Dystrophy (DMD).

A

False. BMD is less common at 1 in 30,000 males

DMD is 1 in 3,500 males

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29
Q

Describe Toxic Myopathies.

A
  • cause weakness, cramping, and pain
  • occurs in older adults
  • acute onset
  • originate from intrinsic or extrinsic exposures
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30
Q

What is a common first manifestation of thyrotoxicosis and commonly manifest with muscle weakness?

A

Thyroxic myopathy

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31
Q

What causes most cases of thyrotoxicosis?

A

Graves Disease – an autoimmune reaction directed against TSH receptors

32
Q

Who will most likely get Grave’s Disease?

A

adult females 40-60 years

33
Q

What disease are these characteristics representing: goiter, fatigue, exopthalmosis, tachycardia, insomnia, unintended weight loss, pretibial myxedma, and is a Type II hypersensitivity?

A

Graves Disease

34
Q

What type of necrosis of myocytes does Graves Disease typically produce?

A

segmental

35
Q

What may develop following episodes of binge drinking resulting is a range of variability of mild muscle swelling to acute pain?

A

Ethanol myopathy (alcohol myopathy)

36
Q

What is Ethanol myopathy likely to be described as a cause of, rather than its own disease?

A

rhabdomyolysis

37
Q

What is the most severe consequence of Ethanol myopathy?

A

acute renal failure

38
Q

What is Drug-induced Myopathy?

A

may develop following exposure to a variety of meds

poorly understood)–> describe as Adverse Drug Reaction (ADR

39
Q

What type of features are caused by Drug-Induced myopathy?

A
  • mild-to-severe myalgia
  • mild-to-severe weakness
  • maybe even rhabdomyolysis
40
Q

What is a very common Drug-induced myopathy that involves:

  • muscle pain, fatigue
  • nocturnal cramping
  • flu-like symptomes
  • elevated creatine kinase levels ???
A

Statin myopathy

41
Q

How many people in US use statin meds to reduce blood cholesterol levels? How those, how many experience ADR?

A

25%; ~2-5% ADR

42
Q

T/F. Peripheral Nerve sheath tumors can be benign or malignant and manifest with Schwann cell differentiation, MC in adulthood.

A

Trrruuueee

43
Q

What occurs when a growing neoplasm causes secondary injury by taking up space and displacing or impinging the surrounding tissues?

A

Mass effect

44
Q

What are Schwannomas?

A

benign peripheral nerve sheath tumors, develop from transformed Schwann cells

45
Q

Do Schwannomas have a familia ass?

A

90% are sporadic

but some occur in families with history of Neurofibromatosis Type 2

46
Q

What is the most common type of Schwannoma?

A

vestibular schwannoma (AKA “acoustic neuroma”)

47
Q

What tumor develops w/in cranium and commonly develops off vestibular portion of CN VIII?

A

Vestibular schwannoma (aka “acoustic neuroma)

still a PNS tumor

48
Q

What will a vestibular schwannoma (acoustic neuroma) cause?

A

compression of CN VIII:

  • hearing loss or tinnitius
  • vertigo
  • headache
  • facial numbness or paralysis if compress CN VII
49
Q

What is characterized by multiple schwannomas throughout the body and presents with numerous subcutaneous masses and multiple tumor w/in CNS?

A

Schwannomatosis

50
Q

Schwannomatosis may cause multiple intracranial schwannomas, so what makes them different than a Schwannoma?

A

the lesions DO NOT develop in area of CN VIII (no vestibular involvement!!)

51
Q

What is the primary symptom of Schwannomatosis?

What other symptoms can occur?

A

widespread and intense pain due to compression of sensory nerves

  • weakness, paresthesia, and bowel or bladder problems
52
Q

What are Neurofibromas?

A

benign nerve sheath tumors of PNS

90% are isolated tumors; but if genetic condition (Neurofibromatosis Type I) will have mult.

53
Q

What type of tumor develops from a transformed nonmyelinating Schwann cell and may develop w/in dermis or in a “plexiform” location?

A

Neurofibromas

54
Q

Where do dermal neurofibromas develop? Where do plexiform neurofibromas develop?

A

Dermal–> w/in an subcutaneous area and are small and round or large and disfiguring

Plexiform–> in body’s plexuses; brachial or lumbosacral plexus (more likely to cause permanent neurological damage)

55
Q

Do Neurofibromas have a risk of malignant transformation?

A

Yes, small risk; and resulting tumor = malignant peripheral nerve sheath tumor

56
Q

What is an autosomal dominant genetic disorder that develops from a mutation in NF1 gene and results in multiple cutaneous and plexiform neurofibromas?

A

Neurofibromatosis Type 1

NF1 = TSG

57
Q

Besides the cutaneous and plexiform neurofibromas ass. with NF1, what other characteristics manifest?

A
  • cafe-au-lait-spots
  • freckles in axilla and groin
  • Lisch nodules on iris of eye
58
Q

When do most growths of Neurofibromatosis Type 1 appear?

A

childhood or adolescence and may enlarge into adulthood

59
Q

T/F. Neurofibromatosis Type 1 increase risk of learning disabilities, attention deficit disordors, and seizures.

A

True (even though have normal intelligence)

60
Q

How common is Neurofibromatosis Type 1? How does it arise?

A

~1 in 3,000

from inherited or sporadic NF1 mutation

61
Q

About __ percent of all Neurofibromatosis Type 1 patients have a neurofibroma that transitions into cancerous mass, known as ______________

A

10%; malignant peripheral nerve sheath tumor

62
Q

What is defined by the presence of a “bilateral vestibular schwannomas”?

A

Neurofibromatosis Type 2 (NF2)

63
Q

T/F. Neurofibromatosis Type 2 obviously have neurofibromas due to its name.

A

FALSE!!!!

64
Q

What develops from an autosomal dominant mutation in the Merlin gene?

A

Neurofibromatosis Type 2

Merlin gene = TSG

65
Q

Individuals with Neurofibromatosis Type 2 have an increased risk of developing what three things?

A
  • mulitple schwannomas
  • meningiomas (CNS)
  • ependymomas (CNS)
66
Q

About how many people get Neurofibromatosis Type 2? How does it arise?

A

~1 in 50,000

1/2 inherited and 1/2 sporadic mutations to Merlin gene

67
Q

What develop from transformed neurofibromas and about 1/2 of all cases occur in patients with Neurofibromatosis Type 1 (NF1)?

A

Malignant peripheral nerve sheath tumors

68
Q

What are cancerous tumors that manifest with some degree of Schwann cell differentiation?

A

Malignant peripheral nerve sheath tumors

69
Q

When do Malignant peripheral nerve sheath tumors develop?

A

adulthood

70
Q

How do Malignant peripheral nerve sheath tumors manifest?

A
  • highly anaplastic cellular appearance
  • high rate of growth and infiltrate quickly
  • common on extremities
  • painless or painful surrounded by edema
71
Q

What is a non-neoplastic reactive lesion following transsection of a peripheral nerve?

A

Traumatic Neuromas (aka amputation neuromas)

72
Q

How do Traumatic Neuromas (aka amputation neuromas) manifest?

A
  • peripheral neuron will try to produce axonal sprout after injuried
  • sprouts “miss there target” at distal transected nerve and develop a haphazard mixture of axons, Schwann cells, and CT
73
Q

After injury to a peripheral nerve, what develops following an axonal sprout “missing its target” and developing haphazard mixture of axons, Schwann cells, and CT?

A

Traumatic Neuromas (aka amputation neuromas)

74
Q

T/F. Traumatic Neuromas are painful, but are non-invasive and have NO malignant potential

A

True

75
Q

What is the name given to traumatic neuroma that develops b/w the 3rd and 4th metatarsal heads (of the foot)?

A

Morton’s Neuroma

76
Q

Describe how Morton’s Neuromas manifest?

Who are they MC in?

A

due to location, are painful when weight-bearing– “standing on pebble, rock, or marble”

10x MC in women

System Pathology I -- Roecker (14 decks)

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