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Systems Pathology I Exam 2 > Chapter 21: Peripheral Nervous System > Flashcards

Chapter 21: Peripheral Nervous System Flashcards

1
Q

What makes the myelin in the PNS?

A

Schwann Cells

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2
Q

Pathology of any portion of a motor unit leads to what dysfunction?

A

Painless weakness (myasthenia)

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3
Q

What kind of peripheral neuropathy involves direct injury to the axon and degeneration of peripheral segments?

A

Axonal Neuropathy

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4
Q

What is the secondary loss of myelin called with axonal neuropathy?

A

Wallisian degeneration

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5
Q

After repair of the axonal neuropathy, how is the axon different from before?

A

Decreased axon density and overall amplitude is decreased

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6
Q

What kind of peripheral nerve injury involves damage to Schwann cells or myelin?

A

Demyelinating neuropathy

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7
Q

What is the pattern of destruction for demyelinating neuropathy?

A

random internode demyelination (segmental demyelination)

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8
Q

How is the myelin different after repair for demyelinating neuropathy?

A

Thinner myelin and shorter internodes leading to slowed nerve conduction velocity (NCV)

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9
Q

A shoulder dislocation could caused what type of mononeuropathy?

A

Axillary nerve palsy

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10
Q

An example of an entrapment mononeuropathy is?

A

carpal tunnel syndrome

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11
Q

What are the two primary causes of mononeuropathies?

A

entrapment or trauma

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12
Q

Diabetes and alcoholism can lead to what kind of peripheral nerve injury?

A

polyneuropathy

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13
Q

What type of damage is common among polyneuropathies?

A

Diffuse/symmetric damage

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14
Q

Polyneuropathy has what characteristic paresthesia present?

A

Stocking and glove paresthesia

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15
Q

What is the effect of Guillain-Barre syndrome on the nervous system?

A

Acute motor neuron demyelination

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16
Q

What is unique about the paralysis associated with Guillain- Barre syndrome?

A

Ascending paralysis which leads to “rubbery legs” and decreased deep tendon reflexes

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17
Q

How can Guillain-Barre syndrome be lethal?

A

Respiratory failure

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18
Q

Why is Guillain- Barre syndrome considered an auto immune disease?

A

Presence of macrophages near the nerve roots

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19
Q

What is the cause of Guillain-Barre syndrome?

A

60% the cause is unknown, but the remaining 40% are due to viruses such as C. jejuni, EBV, CMV, HIV (most of these except for HIV are minor viruses)

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20
Q

What is the age range likelihood for diagnoses of Guillain-Barre syndrome?

A

Males ages 15-35 and 50-57

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21
Q

90%of those affected by Guillain-Barre’ syndrome self-resolve

T or F

A

True

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22
Q

What is the treatment for Guillain-Barre’ Syndrome?

A

Ventilation, plasmapheresis, IV antibodies

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23
Q

What is another name for Chronic Guillain-Barre syndrome?

A

Chronic Inflammatory demyelinating polyneuropathy (CIPD)

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24
Q

What genetic peripheral nerve injury involves symmetric segmental demyelination and gives off the appearance of “onion-skin” Schwann cells?

A

Chronic inflammatory demyelinating polyneuropathy

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25
Q

What signs are associated with Chronic Inflammatory Demyelinating Polyneuropathy?

A

Paresthesia/Pain, ataxia, and decreased deep tendon reflexes

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26
Q

What age and gender are most likely to contract Chronic Inflammatory Demyelinating Polyneuropathy?

A

Males, 40-60 years old

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27
Q

In class, what two risk factors were associated with CIDP?

A

SLE and HIV

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28
Q

What is the treatment for chronic inflammatory demyelinating polyneuropathy?

A

Plasm apheresis, immunosuppression

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29
Q

Which are you more likely to recover from: Guillain-Barre Syndrome or Chronic Inflammatory Demyelinating Polyneuropathy?

A

Guillain-Barre Syndrome because CIPD is commonly associated with permanent loss of function

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30
Q

What leads to the nerve injury present in Diabetic Peripheral Neuropathy?

A

Microangiopathy

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31
Q

Which blood vessels are damaged in Diabetic Peripheral Neuropathy?

A

vasa nervorum

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32
Q

True or False: The damage to the myelin and axons in Diabetic Peripheral Neuropathy is asymmetrical?

A

False; it is symmetrical

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33
Q

What are the symptoms of diabetic peripheral neuropathy?

A

Decreased deep tendon reflexes along with decreased sensation of vibrations, soft touch, and pain

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34
Q

What is the treatment for diabetic peripheral neuropathy?

A

Blood glucose monitoring, daily inspection (analgesics and anticonvulsants)

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35
Q

What two possible signs of diabetic peripheral neuropathy are constantly checked for during daily inspections?

A

Gangrene and ulcers

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36
Q

What is it called if a patient with Diabetic Peripheral Neuropathy has microanigopathy that occurs in the retina? What are the conditions that can occur?

A

Diabetic Retinopathy

Vision problems; possible blindness

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37
Q

What is it called if a patient with Diabetic Peripheral Neuropathy has microanigopathy that occurs in the kidneys? What are the conditions that can occur?

A

Diabetic Nephorpathy

Glomerulonephritis

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38
Q

What is a possible complication of any neuropathy?

A

Neuropathic Arthropathy

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39
Q

What is the cause of Neuropathic Arthropathy?

A

Decreased sensation which means patient has unperceived trauma or injury

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40
Q

What is the most common cause of Neuropathic Arthropathy?

A

Diabetes mellitus

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41
Q

What are three other causes of Neuropathic Arthropathy besides Diabetes Mellitus?

A

Spinal cord injury, syphilis, leprosy

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42
Q

What is the generic location for Neuropathic Arthropathies?

A

progressive degeneration in weight-bearing joints such as foot and ankle; to a lesser degree hips

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43
Q

How do environmental toxins lead to peripheral nerve injury?

A

Alter axonal transport or cytoskeleton damage of the longest neurons

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44
Q

ADRs, lead, and methyl-mercury are examples of what kind of cause of peripheral nerve injury?

A

Environmental toxins

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45
Q

Vasculitis is present in how many vasculitis patients?

A

1/3

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46
Q

Vasculitis is present in what three conditions?

A

polyarteritis nodosa, SLE, and RA

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47
Q

What is an example of Inherited Peripheral Nerve Injury?

A

Charcot-Marie-Tooth Disease

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48
Q

What is a group of similar conditions that lead to axonal or demyelinating neuropathy and is caused by a PMP22 mutation?

A

Charcot-Marie-Tooth Disease

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49
Q

What type of neurons are affected by Charcot-Marie-Tooth Disease

A

motor and sensory neurons

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50
Q

When is Charcot-Marie-Tooth disease most likely to be seen?

A

Young Adulthood

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51
Q

What are the types of severity that can be noticed in Charcot-Marie-Tooth disease?

A

Variable severity such as altered gait or debilitating gate

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52
Q

What muscles are affected by Charcot-Marie-Tooth Disease?

A

Muscles innervated by the common fibular nerve

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53
Q

What is the pathway for myofiber depolarization?

A

pre-synaptic Ca++ influx -> ACh release -> post-synaptic ACh receptor -> myofiber depolarization

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54
Q

Which neuromuscular junction disorder is an autoimmune attack against the post-synaptic ACh receptors?

A

Myasthenia Gravis

55
Q

Where is the most common site of lesion in Myasthenia Gravis?

A

Thymus

56
Q

Which thymic lesions might be present in myasthenia Gravis and which is the most common?

A

Hyperplasia (60%, MC)

Thymoma (20%)

57
Q

Extreme pes cavus is seen with what cause of peripheral nerve injury?

A

Charcot-Marie-Tooth Disease

58
Q

Thymic lesions from myasthenia gravis result from

A

autoreactive B and T cells

59
Q

What is the hallmark sign of myasthenia gravis?

A

Extraocular weakness: diplopia and ptosis

60
Q

What gender is more commonly affected by myasthenia gravis?

A

Females (age 20-30)

61
Q

What is the typical age range for males acquiring myasthenia gravis

A

50-70 years

62
Q

What affect does electronic stimulation have on patients with myasthenia gravis?

A

worsens the condition

63
Q

What is the survival rate of those with myasthenia gravis?

A

95% 5 year survival rate

64
Q

What test is administered to patients with myasthenia gravis that may actually temporarily relieve symptoms?

A

The Tensilon Test (which inhibits ACh receptor antibodies)

65
Q

What treatments are available for myasthenia gravis patients?

A

tymectomy, ACHase inhibitor meds; immunosuppression, plasmapheresis

66
Q

Which third trimester teacher suffered from a unique condition known as Juvenile Ocular Myasthenia Gravis?

A

Dr. Roecker

67
Q

What neuromuscular junction disorder involves the degradation of pre-synaptic calcium channels?

A

Lambert-Eaton Syndrome (LES)

68
Q

What affect does electronic stimulation have on Lambert-Eaton Syndrome?

A

Improves conditions

69
Q

What gender is most likely to be diagnosed with Lambert-Eaton Syndrome?

A

Females (2x more likely)

70
Q

What is the usual age range for Lambert-Eaton Syndrome?

A

60 years of age

71
Q

Paraneoplastic syndrome (small cell lung cancer) is associated with which neuromuscular junction disorder?

A

Lambert-Eaton Syndrome

72
Q

Which type of infectious neuromuscular junction disorder causes an increase in ACh release?

A

Tetanus (clostridium tetani)

73
Q

What feature is commonly seen in patients diagnosed with Tetanus?

A

tetanic spasm

74
Q

What is the mode by which clostridium tetani and clostridium botulinum spread to cause infection?

A

through the soil

75
Q

What condition involves decreased ACh release resulting in descending paralysis?

A

Botulism (clostridium botulinum)

76
Q

If an infant (younger than 1 years old) is exposed to clostridium botulinum, what condition can occur and what is the name of that condition?

A

Flaccid paralysis

Floppy Baby Syndrome

77
Q

Peripheral axons innervate multiple ______ in a ______pattern.

A

myocytes; checkerboard

78
Q

Type I fibers are (slow/fast) twitch, (white/dark), (aerobic/anaerobic), and use (fat/glycogen) for energy.

A

slow, dark, aerobic, fat

79
Q

Type II fibers are (slow/fast) twitch, (white/dark), (aerobic/anaerobic), and use (fat/glycogen) for energy.

A

fast, white, anaerobic, use glycogen

80
Q

What is the name of a primary muscle disease characterized by muscular origin and painless weakness?

A

myopathy

81
Q

What is muscle tissue replaced by as it atrophies under neuropathies and myopathies?

A

it is replaced by fibrotic/fatty tissue

82
Q

Define neuropathic changes in skeletal muscle disorders?

A

altered innervation

83
Q

What effect do neuropathic changes have on motor units?

A

they become fewer in number and larger in size

84
Q

Disuse atrophy affects primarily what type of fibers?

A

type II

85
Q

Glucocorticoid atrophy affects primarily what type of fibers?

A

Type II

86
Q

Localized fractures or generalized paralysis (such as quadriplegia) could lead to which type of skeletal muscle disorder?

A

Disuse atrophy

87
Q

Name the endocrine disorder that is associated with increased levels of cortisol.

A

Cushing Syndrome

88
Q

What is the most common cause of Cushing Syndrome?

A

External corticosteroids

89
Q

Besides external corticosteroids, what is another cause of Cushing syndrome?

A

Pituitary or adrenal adenoma (causes increased levels of ACTH)

90
Q

Features such as weight gain, moon facies, purple striae, buffalo hump, insomnia, hirsutism, and infertility are all associated with what disease?

A

Cushing Syndrome

91
Q

What gender is most likely to have Cushing Syndrome

A

Young Adult Females

92
Q

True or False: Neuropathy is most likely associated with grouped atrophy

A

True

93
Q

True or False: Myopathy is most likely associated with segmental necrosis/regeneration.

A

True

94
Q

What condition is associated with a group of inherited myopathies?

A

Muscular Dystrophy

95
Q

What is the function of dystrophin?

A

protein that keeps muscles strong

96
Q

Define dystrohinopathies

A

mutated dystrophin

97
Q

What are the two categories of muscular dystrophy?

A

Duchenne and Becker Muscular Dystrophy

98
Q

Which type of Dystophinopathy is associated with absent dystrophin, and is seen by weakness by age 5, wheelchair bound by teens?

A

Duchenne Muscular Dystrophy

99
Q

Which dystrophinopathy is fatal by young adulthood?

A

Duchenne Muscular Dystrophy

100
Q

Which dystrophinophathy is associated with abnormal dystrophin and has an adolescent onset?

A

Becker Muscular Dystrophy

101
Q

Which dystrophinopathy may have a normal life span?

A

Becker Muscular Dystrophy

102
Q

List the shared features between dystrophinopathies:

A

clumsiness, weakness, fatigue, pelvic girdle weakness, pseudohypertrophy of calf muscles, lethal cardio-respiratory insufficiencies,, labs that show increased creatine kinase.

103
Q

Gower’s sign is associated with what condition?

A

Duchenne Muscular Dystrophy

104
Q

Which toxic myopathy involves the overproduction of thyroxine?

A

Thyrotoxic myopathy

105
Q

Goiter and Graves’ Disease are associated with which toxic myopathy?

A

Thyrotoxic myopathy

106
Q

Which toxic myopathy can be acute or chronic, and is characterized by proximal muscle weakness and necrosis?

A

Thyrotoxic myopathy

He also mentioned this is a type II hypersensitivity

107
Q

Binge drinking or rhabdomyolysis is associated with which toxic myopathy?

A

ethanol myopathy

108
Q

Why can ethanol myopathy be lethal?

A

progresses to acute renal failure

109
Q

Acute myalgia, myocyte swelling and necrosis are all associated with which toxic myopathy?

A

Ethanol myopathy

110
Q

What is the most common cause of drug myopathy?

A

statins

111
Q

Peripheral Nerve Sheath tumors are most common in what age groups?

A

adults

112
Q

How do peripheral nerve sheath tumors usually manifest?

A

manifest as pain or loss of function

113
Q

Are schwannomas usually benign or malignant?

A

benign

114
Q

What cranial nerve is most commonly involved with a schwannoma?

A

C.N. VIII

115
Q

Which type of peripheral nerve sheath tumor is 90% sporadic?

A

Schwannoma

116
Q

Which type of peripheral nerve sheath tumor involves multiple CNS and cutaneous schwannomas and is familial?

A

Schwannomatosis

117
Q

True or False: Vestibular Schwannomas is associated with Schwannomatosis.

A

False, only with schwannomas

118
Q

Possible hearing loss or tinnitus is associated with which kind of peripheral nerve tumor?

A

Schwannoma

119
Q

What is a neurofibroma?

A

benign nerve sheath tumor

120
Q

Which condition involves bilateral vestibular Schwannomas?

A

Neurofibromatosis type 2 (NF2)

121
Q

What is the genetic pattern of Neurofibromatosis Type 2?

A

Austomsomal dominant, Merlin gene invovled

122
Q

What are the problems often seen with Neurofibromatosis Type 2?

A

vision and hearing problems, but no cutaneous lesions

123
Q

Which condition involves pronounced neurofibromas and vascular stenosis?

A

Neurofibromatosis Type 1

124
Q

What is the genetic pattern of Neurofibromatosis Type 1?

A

Autosomal dominant, mutation on chromosome 17

125
Q

What are three visible conditions associated with Neurofibromatosis Type 1?

A

Lisch nodules, axillary freckling, and cafe’ au lait spots

126
Q

What are some effects of neurofibromatosis type 1?

A

decreased cognition and seizures

127
Q

What is the most common location for malignant peripheral nerve sheath tumors?

A

plexiform

128
Q

Cancer of Schwann cells and/or neuronal connective tissue, as well as transformed neurofibromas all may be a sign of what condition?

A

Malignant Peripheral Nerve Sheath Tumor (MPNST)

129
Q

Malignant Peripheral Nerve Sheath Tumor can be seen on what parts of the body?

A

Arms and legs; painless and edematous

130
Q

50% of all MPNST cases arise from patients with:

A

NF1 (Neurofibromatosis 1)

131
Q

MPNST affects what percentage of all NF1 patients?

A

10%

132
Q

What is the most common cause of a traumatic neuroma?

A

surgery

133
Q

What is a common location for traumatic neuromas?

A

feet

Systems Pathology I Exam 2 (1 decks)

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