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Systems Path 1 > Exam 2: PNS > Flashcards

Exam 2: PNS Flashcards

1
Q

Myasthenia

A

Painless weakness

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2
Q

Pathology of any portion of motor unit may cause

A

Skeletal muscle dysfunction

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3
Q

Axon neuropathy involved direct injury to

A

Axon

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4
Q

Areas of injury for PNS damage

A

Lower motor neuron/ peripheral axon

NMJ

Innervated myoctyes

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5
Q

Myelin in PNS created by

A

Schwann cells

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6
Q

2 types of neuropathy

A

Axonal

Demyelinating

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7
Q

Wallerian degeneration

A

Distal to injury breaks down

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8
Q

Demyelinating neuropathy involves damage to

A

Myelin or Schwann cells

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9
Q

Peripheral neurons have ability to

A

Regrow

Remyelinate

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10
Q

With regrowth and demyelination of PNS…

A

Low axon density

Low amplitude on NCV

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11
Q

Demyelinating neuropathy spares

A

Axon

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12
Q

Segmental demyelination

A

Random demyelination

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13
Q

Repair of demyelinating neuropathy

A

Thin myelin

Short internodes

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14
Q

Mononeuropathy always involves either

A

Entrapment

Trauma

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15
Q

Polyneuropathy involves

A

Many nerves damaged from metabolic problems

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16
Q

Stocking and glove paresthesia observed in

A

Polyneuropathy

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17
Q

Rubbery legs, ascending paralysis, and respiratory failure observed in

A

GBS

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18
Q

Guillain-Barre Syndrome (GBS)

A

Acute motor neurons demyelination

Autoimmune

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19
Q

Age and sex MC in GBS

A

Males

15-35 or 50-60

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20
Q

Causes of PNS injury

A 
Malnutrished 
Toxic 
Vascular 
Inflammatory 
Genetic
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21
Q

Most cases of GBS ___

A

Self-resolve

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22
Q

___ near motor nerve roots in GBS

A

Macrophages

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23
Q

Treatment for GBS

A

Ventilation

IV ABs

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24
Q

Chronic inflammatory demyelinating polyneuropathy (CIDP)

A

Chronic version of GBS

Sensorimotor abnormalities at least 2 months

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25
Q

Schwann cells resembling onion bulbs observed in

A

CIDP

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26
Q

Age and sex MC for CIDP

A

Males 2X

40-60 yo

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27
Q

Microangiopathy drives

A

Diabetes problems

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28
Q

In diabetes, ROS damages

A

Vasa nervorum

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29
Q

Nerve injury in diabetes begins with

A

Decrease sensation

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30
Q

Treatment for diabetic peripheral neuropathy

A

Glucose control
Daily inspection
Analgesics
Anticonvulsants

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31
Q

Diabetic peripheral neuropathy injures both

A

Myelin and axons

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32
Q

Microangiopathy in diabetes peripheral neuropathy damages PNS neurons especially in the

A

Retina

Kidney

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33
Q

Neuropathic arthropathy involves progressive degeneration of

A

Weight bearing joints

Foot, ankle, hip
TM and MP joints

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34
Q

Neuropathic arthropathy can be a complication of any neuropathy, particularly

A

DM
Spinal cord injury
Syphilis
Leprosy

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35
Q

Neuropathic arthropathy causes decrease sensation, cause

A

Unperceived trauma

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36
Q

Environmental exposure that damages peripheral nerves affects

A

Longest neurons

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37
Q

Mononeuritis multiplex

A

Neuronal damage from vasculitis

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38
Q

Charcot-Marie-Tooth disease

A

Inherited conditions of axonal or demyelinating neuropathy

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39
Q

CMT disease due to

A

PMP22 mutation

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40
Q

Variable severity is observed in CMT disease, common symptoms are

A

Pes cavus

Altered gait

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41
Q

Myasthenia gravis

A

Autoimmune attack on post-synaptic ACH receptors

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42
Q

Extraocular weakness, including ptosis and diplopia, seen in

A

Myasthenia gravis

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43
Q

60% cases myasthenia gravis due to ____ and 20% due to ____

A

Thymus hyperplasia

Thymoma

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44
Q

Age and sex for myasthenia gravis

A

Females MC : 20-30

Males: 50-70

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45
Q

Tensilon test inhibits ___ and is used in ___

A

Antibodies

Myasthenia gravis

46
Q

Lambert-Eaton Syndrome (LES)

A

Autoantibodies against pre-synaptic Ca2+ channels

47
Q

Symptoms worsening throughout the day is characteristic of

A

Myasthenia gravis

48
Q

LES inhibits ___, causing girdle weakness

A

ACH release

49
Q

LES improves with

A

E-stimulation

50
Q

Elderly autoimmune attack

51
Q

Paraneoplastic syndrome can cause

52
Q

Tetanus

A

Clostridium tetani increases ACH release

Spasm

53
Q

Botulism

A

Clostridium botulinum inhibits ACH release

Descending paralysis

54
Q

Floppy infant syndrome due to botulism can be due to

A

Soil or honey

55
Q

Infant botulism effects ages

A

6 weeks - 6 mo

56
Q

Myopathy

A

Primary disease of muscle

57
Q

Type 1 myofiber type

A

Slow twitch
Aerobic
Fat

58
Q

Type 2 myofiber type

A

Fast twitch
Anaerobic
Glycogen

59
Q

Disuse atrophy affects

60
Q

Glucocorticoid atrophy affects

61
Q

Cushing syndrome

A

Elevated cortisol

62
Q

Myasthenia

A

Painless muscle weakness

63
Q

Neuropathic changes cause

A

Grouped atrophy

64
Q

Cushing syndrome can be due to

A

External corticosteroids (MC)

Adenoma (pituitary or adrenal)

65
Q

Features of Cushing syndrome

A 
Weight gain 
Moon facies
Purple striae 
Buffalo hump
Insomnia
Infertility 
Hirsutism
66
Q

Cushing syndrome MC in

A

Young adult females

67
Q

Inherited skeletal muscle disorders include

A

Muscular dystrophy

68
Q

2 types of muscle dystrophy

A
  1. Duchesse muscular dystrophy

2. Becker muscular dystrophy

69
Q

Inheritance of dystrophinopathies

A

X- linked (xp21)

70
Q

In muscular dystrophy, ____ outpaces repair due to mutated dystrophin

A

Myocyte degeneration

71
Q

Duchenne MD

A

Absent dystrophin
1 in 3500 males
Childhood weakness
Fatal

72
Q

Becker MD

A

Abnormal dystrophin (variable)
1 in 30000 males
May have normal life

73
Q

___ always lethal

A

Duchenne MD

74
Q

Features of dystrophinopathies

A 
Clumsiness
Weakness 
Fatigue 
Pelvic girdle weakness -> gower sign 
Pseudohypertrophy of calves 
Cardiorespiratory insufficiency
75
Q

In labs, high ___ observed in dystrophinopathies

A

Creatine kinase

76
Q

Gower sign MC associated with

A

Duchenne MD

77
Q

Graves’ disease

A

Autoimmunity against TSH receptor

78
Q

Graves’ disease MC in

79
Q

Graves’ disease causes hyperthyroidism, resulting in

A

Elevated T3 and T4

80
Q

Signs of Graves’ disease

A 
Goiter 
Exophthalmos 
Pretibial myxedema 
Insomnia
Weight loss
Fatigue 
Irregular HB
81
Q

Pseudohypertrophy of calf muscles in DMD due to

A

Fibrofatty inflitrate

82
Q

Muscular dystrophies are lethal due to

A

Cardiorespiratory insufficiency

83
Q

Thyrotoxic myopathy

A

Overproduction of thyroxine

84
Q

Ethanol myopathy

A

Binge drinking

85
Q

Graves can cause

A

Thyrotoxine myopathy

86
Q

MC drug myopathy

A

Statins myopathy

87
Q

Drug myopathy is usually w/o

A

Inflammation

88
Q

Types of toxic myopathies

A

Thyrotoxic

Ethanol

Drug

89
Q

Ethanol myopathy can progress to

A

Acute renal failure

90
Q

Schwannoma

A

Benign peripheral nerve sheath tumor

91
Q

Most schwannomas are

A

Sporadic and involve CN 8

92
Q

Schwannomatosis

A

Multiple CNS and cutaneous schwannomas

93
Q

Schwannoma aka

A

Acoustic neuroma

94
Q

Schwannoma can cause

A

Tinnitus
Vertigo
Headache
Hearing loss

95
Q

Neurofibromas

A

Benign nerve sheath tumors

96
Q

Neurofibromatosis type 1 (NF1)

A

Pronounced neurofibromas

Vascular stenosis

97
Q

NF1 due to

A

Mutation in chromosome 17 (autosomal dominant)

98
Q

Freq of NF1

A

1 in 3,000

99
Q

NF1 can have 3 distinct characteristics

A

Lisch nodules
Axillary freckling
Cafe au lait spots

100
Q

NF2 due to

A

Mutated Merlin gene

101
Q

NF2 results in

A

Bilateral vestibular schwannomas

102
Q

NF2 results in risk for CNS tumors, including

A

Ependymomas

Meningiomas

103
Q

Freq of NF2

A

1 in 50,000

104
Q

Malignant peripheral Nerve sheath tumor (MPNST)

A

Malignant Schwann tumor mixed with neuronal connective tissue

105
Q

MPNST associated with

106
Q

MPNST is large in infiltrative, affecting

107
Q

MPNST is

A

Painless and edematous

108
Q

Traumatic neuroma

A

Non-neoplastic growth of nerve tissue, following trauma to nerve

109
Q

Traumatic neuroma MC due to

110
Q

NF2 has no

A

Cutaneous lesions

111
Q

Traumatic neuroma is painful and

A

Non-invasive

Systems Path 1 (12 decks)

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