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NMSP Week 4/5 > Peripheral Nervous System and Skeletal Muscle Pathology (Martin) > Flashcards

Peripheral Nervous System and Skeletal Muscle Pathology (Martin) Flashcards

1
Q

Segmental demyelination occurs when?

What is the classic disease process of this?

A

1) There is dysfunction of Schwann cell and damage to the myelin sheath
2) Guillain-Barre

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2
Q

With segmental demyelination, what happens to the axon and myocytes?

A

Remain intact

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3
Q

A failure of the outgrowing axons to find their distal target can produce a non-neoplastic whorled proliferation of axonal processes and associated Schwann cells that results in a painful nodule termed?

A

Traumatic neuroma

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4
Q

Muscle fiber type is determined by?

A

Motor neuron

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5
Q

With axonal degeneration, muscle fibers in motor unit lose neural input and undergo?

A

Denervation atrophy

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6
Q

What are atrophic fibers that are smaller & triangular shape when denervation atrophy occurs?

What are rounded zone of disorganized myofibers in the center of the fiber?

A

1) Angulated fibers

2) Target fibers

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7
Q

A patch of contiguous myocytes having the same histochemical type is known as?

A

Type grouping

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8
Q

What causes type 2 fiber atrophy?

A

1) Inactivity or disuse

2) Glucocorticoid therapy

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9
Q

Segmental necrosis is the result of?

The loss of muscle fiber leads to?

A

1) Destruction of a portion of myocyte, followed by myophagocytosis
2) Deposition of collagen and fat

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10
Q

What happens to muscle fibers during hypertrophy?

A

They divide longitudinally (Muscle fiber splitting)

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11
Q

Patients with peripheral neuropathy (PN) generally describe the pain as?

A

Pins and needles

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12
Q

What causes several nerves to be damaged in haphazard fashion?

What is a common cause of this pattern?

A

1) Mononeuritis multiplex

2) Vasculitis

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13
Q

What does Bell’s Palsy result in?

What CN is involved?

What is it associated with?

A

1) Unilateral facial droop
2) CN VII
3) URI and DM

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14
Q

Neurogenic bladder which causes lack of bladder control can be the result of diseases/defect such as?

A

1) MS
2) Parkinson’s
3) Diabetes
4) Spina bifida

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15
Q

What is Guillain-Barre?

A

Acute inflammatory demyelinating polyneuropathy

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16
Q

How is the paralysis characterized in Guillain-Barre?

What happens to the DTRs as it progresses?

A

1) Ascending paralysis

2) Disappear

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17
Q

Guillain-Barre results in radiculoneuropathy, what does that term mean?

A

Inflammation and demyelination of spinal nerve roots and peripheral nerves

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18
Q

2/3 of cases of Guillain-Barre are preceded by?

Most commonly from?

What antibodies are present?

A

1) Acute influenza-like illness
2) Campylobacter jejuni
3) Anti-myelin Abs

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19
Q

What do we see in the CSF with Guillain-Barre?

A

1) Increased protein

2) Little or no CSF pleocytosis

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20
Q

What is the treatment for Guillain-Barre?

A

1) Plasmapheresis

2) IVIG

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21
Q

What are the most common acquired inflammatory peripheral neuropathy?

A

Chronic Inflammatory Demyelinating Poly(radiculo)neuropathy

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22
Q

The symmetrical mixed sensorimotor polyneuropathy seen in Chronic Inflammatory Demyelinating Poly(radiculo)neuropathy must persist for how long in order for it to be classified as chronic?

A

2 months

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23
Q

What distinguishes Chronic Inflammatory Demyelinating Poly(radiculo)neuropathy from Guillain-Barre?

A

Time course and response to steroids

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24
Q

The excessive Schwann cell proliferation seen with Chronic Inflammatory Demyelinating Poly(radiculo)neuropathy results in what appearance?

A

Onion bulbs

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25
Q

In regards to Lepromatous Leprosy, what pathogen invades Schwann cells, proliferates, then infects other cells?

A

Mycobacterium leprae

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26
Q

What happens to the axons with Leprosy?

What happens to the endoneurial and perineural sheaths?

A

1) Loss of myelinated and unmyelinated axons

2) Endoneurial fibrosis and thickening of perineural sheaths

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27
Q

What is the clinical presentation of leprosy?

A

1) Symmetric polyneuropathy affecting the distal (cool) extremities and face
2) Involves pain fibers leading to loss of sensation which contributes to injury

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28
Q

With Lepromatous Leprosy, smears are positive for what stain at any location?

A

AFB stain

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29
Q

Diphtheria exotoxin affects peripheral nerves and causes an early loss of what sensations?

There is selective demyelination of axons that extend into adjacent?

A

1) Proprioception and vibratory sensation

2) Anterior/posterior roots and mixed sensorimotor nerves

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30
Q

What is the most common viral infection of PNS?

A

Varicella-Zoster virus

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31
Q

Varicella-Zoster affects what areas of the spinal cord and brainstem?

Reactivation occurs in what nerves?

A

1) Sensory ganglia

2) Trigeminal nerves

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32
Q

How does the reactivation of latent infection of Varicella-Zoster present?

A

Painful, vesicular skin eruption (shingles) in a sensory dermatomal pattern

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33
Q

What occurs to the peripheral nerves with Varicella-Zoster?

There is focal destruction of large motor neurons in what areas?

A

1) Axonal degeneration of peripheral nerves after death of sensory neurons
2) Anterior horns or cranial nerve motor nuclei

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34
Q

What is the most common cause of peripheral neuropathy?

A

Diabetes

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35
Q

What is the most common polyneuropathy pattern seen with diabetes?

A

Ascending distal symmetric sensorimotor polyneuropathy

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36
Q

Diabetes mellitus is the number one cause of?

A

Non-traumatic limb amputations

37
Q

Segmental demyelination seen with DM neuropathy results in?

What happens to the endoneurial arterioles?

A

1) Loss of small myelinated fibers and unmyelinated fibers

2) Thickened vessel wall that is PAS+

38
Q

The dysfunction of autonomic nervous system seen in 20-40% of DM patients leads to what symptoms?

A

1) Postural hypotension
2) Incomplete emptying of bladder
3) Sexual dysfunction

39
Q

What is the most common Paraneoplastic neuropathy?

What is it associated with?

A

1) Sensorimotor neuronopathy

2) Small cell lung cancer

40
Q

Neuropathies associated with monoclonal gammopathies (B-cell neoplasms) results in what syndrome?

A

POEMS syndrome:

1) Polyneuropathy
2) Organomegaly
3) Endocrinopathy
4) Monoclonal gammopathy
5) Skin changes

41
Q

Carpel tunnel syndrome affects what nerve?

Numbness & paresthesia is noted at what digits?

A

1) Median nerve

2) Thumb and first 2 digits

42
Q

Saturday night palsy affects what nerve usually due to falling aspleep awkwardly?

A

Radial nerve

43
Q

What is the most common inherited peripheral neuropathies?

A

Charcot-Marie-Tooth Disease

44
Q

Myasthenia gravis has antibodies to?

A

Acetylcholine receptors

45
Q

10% of MG patients have?

30% of patients have?

A

1) Thymoma

2) Thymic hyperplasia

46
Q

Myasthenia Gravis presents with fluctuating generalized weakness that worsens with?

Diminished responses are seen after?

Weakness begins with extraocular muscles leading to?

A

1) Exertion and over the course of the day
2) Repeated stimulation
3) Diplopia, fatigue, ptosis

47
Q

Lambert-Eaton myasthenic syndrome has antibodies to?

What does this cause?

A

1) Voltage gated calcium channels

2) Blocks Acetylcholine release

48
Q

50% of Lambert-Eaton myasthenic syndrome patients have an underlying malignancy, often?

A

Neuroendocrine carcinoma of the lung

49
Q

Repetitive stimulation has what affect in Lambert-Eaton myasthenic syndrome patients?

A

Increases muscle response, opposite of MG

50
Q

With diseases of skeletal muscle in adult tissue, what can contribute to muscle regeneration following injury?

A

Satellite cells (small pool of tissue stem cells)

51
Q

What do we see in skeletal muscle with neurogenic diseases?

A

Fiber type grouping and group atrophy

52
Q

What is myofiber hypertrophy?

A

Physiologic adaptation to exercise

53
Q

Dermatomyositis leads to what specific type of atrophy?

A

Perifascicular atrophy

54
Q

What distinctive skin rash is seen with dermatomyositis?

What is this due to?

What scaling erythematous eruption or dusky red patches can be seen over the knuckles, elbows and knees?

A

1) Lilac or heliotrope discoloration of upper eyelids associated with periorbital edema
2) Telangiectasias
3) Gottron papules

55
Q

What classic symptomology is seen with dermatomyositis?

A

Proximal muscle weakness so difficulty getting up form chair and climbing stairs

56
Q

What autoantibodies associated with dermatomyositis cause the heliotrope rash and Gottron papules?

What causes “mechanic’s hands”?

What causes paraneoplastic & juvenile type?

A

1) anti-Mi2
2) anti-Jo1
3) anti-P155/P140

57
Q

What is the most common inflammatory myopathy in children?

A

Juvenile type dermatomyositis

58
Q

Adult onset inflammatory myopathy with myalgia and symmetric, proximal weakness is characteristic of?

A

Polymyositis

59
Q

How does polymyositis differ from dermatomyosisits?

A

No cutaneous involvement

60
Q

What is the most common inflammatory myopathy in patients > 65?

A

Inclusion Body Myositis

61
Q

Inclusion Body Myositis is most severe in what areas of the body?

A

Quads and distal upper extremities

62
Q

What morphologic change is seen in Inclusion Body Myositis?

A

Rimmed vacuoles (Abnormal cytoplasmic inclusions)

63
Q

What is the first line treatment for inflammatory myopathies?

A

Corticosteroids

64
Q

Which inflammatory myopathy responds poorly to steroids or immunosuppressive treatment?

A

Inclusion body myositis

65
Q

What is a cause of toxic myopathy regardless of dose or subtype?

A

Statins

66
Q

What causes drug-induced lysosomal storage myopathy and leads to slowly progressive muscle weakness?

What fiber type is affected?

A

1) Chloroquine and hydroxychloroquine

2) Type 1

67
Q

What leads to proximal muscle weakness, exophthalmic ophthalmoplegia, and hypothyroidism?

A

Thyrotoxin myopathy

68
Q

Binge drinking may produce?

A

Rhabdomyolysis

69
Q

Congenital Muscular Dystrophy: Ullrich Congenital Muscular Dystrophy (UCMD) is due to mutation of one of the three?

A

Collagen VIα genes

70
Q

Which X-linked Muscular Dystrophy is more common and more severe?

A

Duchenne

71
Q

What gene is affected with Duchenne muscular dystrophy?

A

Dystrophin

72
Q

When does Duchenne’s present?

When does Becker’s?

A

1) Before 5 yo

2) Late childhood

73
Q

What is the difference between DMD and BMD in terms of dystrophin?

A

1) No dystrophin

2) Decreased amount of dystrophin

74
Q

What symptoms is seen with DMD?

A

Pseudohypertrophy of calves

75
Q

Immunohistochemical staining pattern for DMD shows?

A

Absence of the normal sarcolemma

76
Q

What levels are drastically increased in the first decade of life with DMD?

A

Serum CK

77
Q

Symptoms of skeletal muscle weakness, cataracts, endocrinopathy and cardiomyopathy point towards?

A

Myotonic Dystrophy

78
Q

What mutation is seen with Myotonic Dystrophy?

Due to?

A

1) DMPK mutation

2) CTG expansion

79
Q

Symptoms of slowly progressive humeroperoneal weakness, cardiomyopathy (conduction defects), early contractures of Achilles tendon, spine, and elbows points towards?

A

Emery-Dreifuss Muscular Dystrophy

80
Q

Myophosphorylase deficiency is characteristic of?

Acid maltase deficiency with generalized glycogenesis of infancy is characteristic of?

A

1) McArdle disease

2) Pompe disease

81
Q

Weakness, increased serum CK, or rhabdomyolysis along with extraocular muscle involvement and chronic progressive external ophthalmoplegia are symptoms that point to?

A

Mitochondrial myopathies

82
Q

Autosomal recessive neuropathic disorder with loss of motor neurons leading to muscle weakness and atrophy is characteristic of?

Infants with this disease present as?

A

1) Spinal Muscular Atrophy

2) Floppy baby (Wernig-Hoffman)

83
Q

Spinal Muscular Atrophy destroys what area of the spinal cord?

It is caused by what mutation?

A

1) Anterior horn cells

2) SMN1

84
Q

What is the most common Ion cannels Myopathies (channelopathies) mutation?

What is it associated with?

A

1) RYR1

2) Malignant hyperthermia

85
Q

What is a familial tumor syndrome that is generally seen in children?

What tumors is it associated with?

What are the cutaneous lesion shagreen patches characterized by?

What are the Ash-leaf patches?

A

1) Tuberous sclerosis complex
2) Hamartomas
3) Localized leathery thickenings
4) Hypopigmented areas

86
Q

Neurofibromas of peripheral nerves, optic nerve gliomas, Lisch nodules, and Café au lait spots points towards?

A

Neurofibromatosis (NF) type 1

87
Q

What is characterized by bilateral schwannomas (CN VIII) and increased meningiomas, ependymomas?

A

NF type 2

88
Q

What are Schwannoma positive for?

A

S-100

89
Q

Plexiform Neurofibroma has what characteristic appearance?

A

Bag of worms

NMSP Week 4/5 (13 decks)

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