Peripheral Nerves and Muscles pt. I Flashcards

1
Q

What are the causes of peripheral nerve injury (5)

A

Nutritional, toxic, vascular, inflammatory, genetic

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2
Q

Charcot-Marie-Tooth nuropathies are in what etiologic category?

A

Inherited

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3
Q

What is Guillain-Barre Syndrome (BGS)?

A

Common, acute motor neuron demyelination

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4
Q

What kind of paralysis is associated with GBS?

A

ascending paralysis: “rubbery legs”, decreased DTR’s (deep tendon reflexes)

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5
Q

T/F

GBS is not lethal

A

False,

Possibly lethal from respiratory failure

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6
Q

What kind of condition is GBS?

A

Autoimmune: macrophages near nerve roots.

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7
Q

What causes GBS?

A

60% Idiopathic, viral: C.jejuni, EBV, CMV, HIV

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8
Q

Who is at most risk for GBS?

A

Males, ages 15-35 or 50-57 (biphasic distribution i.e. two specific age ranges implicated)

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9
Q

T/F

90% of GBS cases self resolve

A

True

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10
Q

What is the tx for GBS?

A

Ventilation, Plasmapheresis, I.V. antibodies

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11
Q

What is Chronic Inflammatory Demyelating Polyneuropathy (CIDP)?

A

Chronic version of GBS. Symmetricl, segmental demyelination/remyelination.

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12
Q

What cells are affected by CIDP?

A

Motor and sensory neurons, “onion-skin” Schwann cells.

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13
Q

What are some symptoms of CIDP?

A

Pain, paresthesia, ataxia, decrease DTRs

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14
Q

What kind of disorders is CIDP associated with?

A

Autoimmune disorders: lupus, HIV

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15
Q

What is the treatment for CIDP?

A

Plasmapheresis, immunosuppression,

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16
Q

What is the prognosis for CIDP patients?

A

Possible recovery, MC some residual

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17
Q

What is the #1 cause of perpheral neuropathy?

A

Diabetic peripheral neuropathy. 50% of Diabetes millitus cases.

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18
Q

What is diabetic peripheral neuropathy?

A

Axonal & demyelinating (mixed), symmetric

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19
Q

What happens with hyperglycemia relate to DPN?

A

Hyperglycemia = vascular injury -> nerve injury. Damaged vasa nervorum

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20
Q

What are some sensory abnormalities with DPN?

A

Decreased sensation: vibratory, soft touch, pain

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21
Q

What are some other signs of DPN?

A

Ulcers, gangrene, decreased (sluggish) DTRs

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22
Q

What is the tx for DPN?

A

Blood glucose, daily inspection. analgesics, anticonvulsants (because act on CNS to reduce pain).

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23
Q

How do environmental toxins cause misc. peripheral neuropathies?

A

Alter axonal transport of cytoskeletal damage (ADRs, lead, methyl-mercury). Longest neurons.

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24
Q

What does systemic vasculitis have to do with PN?

A

1/3 of vasculitis patients have peripheral neuropathy, polyarteritis nodosa (produces polyneuritis multiplex). Altered sentation (pain) and motor function.

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25
Q

What is a genetic misc PN?

A

Charcot Marie Tooth disease. PMP22 gene mutations. Axonal or demyelinating, motor and sensory

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26
Q

Who is most susceptible to Charcot Marie?

A

Young adults, slowly progressive (1:2,500)

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27
Q

What is a very notable condition associated with Charcot-Marie-Tooth disease?

A

Extreme pes cavus (foot)

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28
Q

What are neuromuscular jnx disorders?

A

Pres-synaptic Ca++ influx -> ACh -> post-synaptic ACh receptor -> myofiber depolarization (some sort of muscle contration, activity)

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29
Q

What is myasthenia gravis?

A

Autoimmune condition: acetylcholine receptor antibodies. Degradation of post synaptic ACh receptors
Rare: 3:100,000

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30
Q

What kind of lesions are noted with MG?

A

Thymic lesions: hyperplasia (60%), thymoma (20%) Autoreative T cells and B cells

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31
Q

What is the hallmark of MG?

A

Extraocular weakness: diplopia, ptosis

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32
Q

What age/gender is affected by MG

A

All Ages

Females (MC): Age: 20-30
Males: ages 50-70 years

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33
Q

T/F

MG symptoms get better as the day progresses and show relief with e-stimulation

A

False

MG symptoms get WORSE as the day progresses. E stim makes it worse.

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34
Q

What test is used for MG?

A

Tensilon test (edrophonium) Inhibits ACh receptor antibodies.

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35
Q

What is the tx for MG

A

Immunosuppression, cholisterase inhibitors, plasmapheresis, thymectomy.

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36
Q

What is the overall survival for MG

A

95% 5-year survival

37
Q

What is Lamber-Eaton syndrome (LES)?

A

Autoantibodies inhibit pre-synaptic Ca++ channels. Decrease ACh release -> weakness (limb girdles)

38
Q

T/F

Repetitive e-stimulation improves LES? LES is rare

A

True

39
Q

What age group/gender is found having LES?

A

Females (2x), - 60 yrs old

40
Q

What is paraneoplastic syndrome? What is the prognosis?

A

Small cell lung CA. Often seen paired with LES. Poor prognosis

41
Q

What is the tx for LES?

A

Plasmapheresis, immunosuppression. Decrease autoantibodies

42
Q

What are some characteristics of congenital myasthenic syndromes

A

Mutations alter neuromuscular juntion proteins. All forms: presynaptic, synaptic, postsynaptic.

43
Q

What conditions may congential myasthenic syndromes mimic?

A

LES or MG. Few respond to achetylcholinesterase inhibitors.

44
Q

What are some infections that can lead to neuromusclular jxn disorders?

A

Soil bacteria -> neurotoxins.

45
Q

What is tetanus?

A

Clostridium tetani: increased ACh release (flooding channels with ACh). Spasticity. 10% lethal.

46
Q

What is Botulism?

A

Clostridium botulinum: Decreased ACh release. Descending paralysis (flaccidity): eyes -> throat -> respiration.

47
Q

What are skeletal muscle tissue disorders?

A

Peripheral axons innervate multiple myocytes. Checkerboard (link pink, dark pink muscle fiber types)

48
Q

What determines myofiber type?

A

Peripheral axons, not training.

49
Q

What are Type I fibers?

A

Slow twtich, dark, aerobic, fat met.

50
Q

What are Type II fibers?

A

Fast twitch, white, anaerobic, glycogen met.

51
Q

What is myopathy?

A

Disease of muscular origin, weakness. May be primary or secondary to neuropathy.

52
Q

What are some similarities of neuropathy and myopathy?

A

Both have muscle atrophy, fibrotic & fatty replacement

53
Q

What are neuropathic changes?

A

Altered innervation. Grouped atrophy. Altered muscle fiber patterns. Motor units: fewer and larger.

54
Q

What are some features of disuse atrophy?

A

Atrophy of primarily type II fibers. Localized (fx), generalized (quadriplegia)

55
Q

What are some features of glucocorticoid atrophy?

A

Atrophy of primarily type II fibers. Endogenous (Cushing disease), exogenous (MC) - taking some sort of medication.

56
Q

What are congenital myopathies?

A

Group of inherited diseases (umbrella term for specific disorders), perinatal/childhood onset. Static (doesn’t get better or worse) hyptonia, Tx is palliative

57
Q

What is Muscular dystrophy?

A

Progressive, inherited, rarely congenital.

58
Q

What are the MC cause of MD?

A

Dystrophinopathies. Dystrophin gene mutation: X chromosome. Myocyte degeneration outpaces repair

59
Q

What are the 2 types of MD?

A
Duchenne MD (DMD)
Becker MD (BMD) Less severe, shows up in adolescence. more variable.
60
Q

What is Duchenne MD?

A

Dystrophin protein is absent = more severe. Weakness by age 5, wheelchair by teens. Fatal by young adulthood.

61
Q

What is Becker MD?

A

Abnormal dystrophin = severity is variable. Adolescent onset. May have a NEARLY normal life span.

62
Q

What are some essential/shared characteristics of dystrophinopathies?

A

Vary by severity. Clumsiness, weakness, fatigue (can’t keep up). Pelvic girdle weakness (possible shoulder girdle weakness, advanced stages). Pseudohypertrophy (look cut and develped but aren’t) of calf muscles (fibro-fatty infiltrate).

63
Q

What lab findings indicate dystrophinopathies?

A

Increase creatine kinase (muscle breakdown)

64
Q

What causes death with dystrophinopathies?

A

Cardiorespiratory insufficiency: cardiomyopathy, arrythmia, pneumonia.

65
Q

What is Gower sign? What condition does it indicate?

A

Children using hands to push on legs in order to stand up (bc of weak pelvic girdle muscles). Duchenne muscular dystrophy.

66
Q

What is polymyositis:

A

Aquired Inflammatory myopathy. Autoimmune, T cells attack the endomysium; prox. muscle weakness (shoulder/pelvic girdle muscles), myalgia, dysphagia (difficulty swallowing), males age 45-60.

67
Q

What is dermatomyositis?

A

Inflammatory myopathy: Autoimmune, attack on muscle and skin, pruritus (itching), skin lesions, myalgia, prox. muscle weakness, MC inflamm. myopathy in CHILDREN, paraneoplastic syndrome in adults.

68
Q

What is inclusion body myositis?

A

Inflammatory myopathy: tau proteins in the cytoplasm, progressive weakness and wasting, dysphagia - may be degenerative. MC inflammatory. Myopathy in elderly (>60 years).

69
Q

What is thyrotoxic myopathy?

A

Overproduction of thyroxine (thyrotoxicosis), goiter or Graves disease, acute or chronic, prox. muscle weakness and necrosis.

70
Q

What is ethanol myopathy, and what are some of it’s causes?

A

Binge drinking, rhabdomyolysis progresses to acute renal failure (myoglobinuria - dark urine), acute myalgia, myocyte swelling/necrosis.

71
Q

What is drug myopathy?

A

ADR (adverse drug reaction), various meds, myopathic signs/symptoms, MC without inflammation.

72
Q

What kind of drugs are most implicated in drug myopathies?

A

Statins (decreased cholesterol, myalgia, cramps).

73
Q

What are some general characteristics of peripheral nerve sheath tumros?

A

Tumors arise in PNS. MC in adults, benign or malignant. Schwann cell differentiation is common. Soft tissue mass: impingement -> pain, LOF

74
Q

What is a Schawannoma?

A

Benign, peripheral nerve sheath tumor, 90% sporadic.

75
Q

What cranial nerve is MC implicated in scwannomas?

A

MC involves CN VIII (vestibular schwannoma)

76
Q

What is a schwannomatosis?

A

Familial, multiple CNS and cutaneous schwannomas.

77
Q

T/F

Schwannomatosis involves vestibular scwannomas

A

False,

No vestibular scwannomas (avoids CN VIII)

78
Q

What is a neurofibroma?

A

Benign nerve sheath tumor. Cutaneous, subQ (diffuse), plexiform (in nerve plexuses).

79
Q

What is a neurofibromatosis type II?

A

BILATERAL vestibular schwannomas, autsomal dominant, Merlin gene. Vision/hearing problems

80
Q

T/F

NF2s are known for cutaneous lesions

A

False

No cutanenous lesions

81
Q

What is Neurofibromatosis Type 1

A

Pronounced neurofibromas, vascular stenosis. Decreased cognition, seizures.

82
Q

NF1 is AKA?

A

vonRecklinghausen disease

83
Q

What mutation is found with NF1

A

Autosomal dominant, mutation on chromosome 17

84
Q

Besides multiple cutaneous lesions, what other kind of skin lesions/marks can arise with NF1?

A

Cafe au lait spots, axillary freckling, Lisch nodules (pigmented nodules of the iris)

85
Q

What is NF1 at risk for?

A

Risk of malignant peripheral nerve sheath tumors (transformed neurofibromas, MC plexiform).

86
Q

What is a key way to keep NF1 and NF2 straight?

A

NF1 was named first, because they involve leasions that are seen clearly on the body. NF2 is seen on MRI therefore found second.

87
Q

What are some characteristics of malignant peripheral nerve sheath tumors?

A

Large mass, anaplastic, infiltrative.

88
Q

What is the % of patients have malignant peripheral nerve sheath tumors that come from NF1?

A

50%

89
Q

What is a traumatic neuroma?

A

Post neuronal injury (surgery). Reactive (non neoplastic) lesion. Painful mass: Scwann cells, axons, & CT.