Myopathy Flashcards

1
Q

What is the most common distal symmetric polyneuropathy?

A

DM complication

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2
Q

What is the difference between positive and negative symptoms with polyneuropathy?

A

Positive = gain of pain etc

Negative = loss of sensation, motor etc

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3
Q

What are the s/sx of large fiber neuropathy? Small fiber?

A

Large fiber = loss of myelinated fibers (proprioception)

Small = Loss of unmyelinated fibers (loss of pain, light touch)

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4
Q

True or false: symmetric proximal neuropathy excludes DM as the cause

A

False

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5
Q

Focal and multifocal neuropathies are usually caused by what insult?

A

ischemic insult

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6
Q

What is peroneal neuropathy?

A

Entrapment of nerve at fibular head

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7
Q

What is the prevalence of peripheral neuropathy in DM pts?

A

near 100%, but variable expressivity

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8
Q

Which is more common with DM neuropathy: pain and paresthesias, or sensory/motor loss?

A

Pain and paresthesias

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9
Q

What are the risk factors for developing DM neuropathy? (4)

A
  • Tall
  • Male sex
  • duration of disease
  • age
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10
Q

What is the polyol pathway of DM neuropathy pathogenesis?

A

Elevated BG levels lead to a high nerve glucose concentration, which is converted for sorbitol by aldose reductase

Resulting decrease in myoinositol interferes with Na pump

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11
Q

What is the microvascular theory of DM neuropathy?

A

Pathologic changes in diabetic nerves include capillary basement membrane thickening and resulting neuronal ischemia

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12
Q

What is the glycosylation end-product theory of DM neuropathy?

A

Chronic intracellular hyperglycemia leads to glycosylation end products which deposit around peripheral nerves

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13
Q

What is the usual presentation of DM neuropathy?

A

Insidious onset of stocking glove pattern of neuropathy

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14
Q

What are the labs that should be obtained with peripheral neuropathy?

A

CBC

ESR

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15
Q

What is the vitamin that should always be checked for with neuropathies?

A

B12 and folate

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16
Q

What is the role of EMGs with evaluating neuropathies?

A
  • May confirm symptoms are related to neuropathy

- Distinguish between axonal and demyelinating neuropathies

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17
Q

What is the treatment for Peripheral neuropathies 2/2 immune mediated diseases?

A

IVIG or plasma exchange

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18
Q

Is lowering BG levels to treat peripheral neuropathies more effective in DM I or DM II?

A

More effective in DM I

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19
Q

What are the two major antidepressants used to treat symptoms with peripheral neuropathies?

A

Tricyclics

Cymbalta

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20
Q

What are the antiepileptic drugs that are used to treat neuropathic pain?n (3)

A
  • Pregabalin
  • Gabapentin
  • Carbamazepine
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21
Q

What is the treatment for absence seizures?

A

Ethosuximide

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22
Q

What is the treatment for trigeminal neuralgia, and for partial and complex seizures?

A

Carbamazepine

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23
Q

What are the two major analgesics for neuropathy?

A
  • Tramadol

- Narcotics

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24
Q

What are the two topical agents used to treat neuropathies?

A
  • Capsaicin

- Lidocaine

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25
Q

What is the bimodal age onset for MG?

A

15-30 y/o women

60+ yo men

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26
Q

What is the most common presentation of MG?

A

Lid ptosis or diplopia with fatigue

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27
Q

What are the usual bulbar s/sx with MG? (2)

A
  • facial weakness/dysarthria

- Neck flexor weakness

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28
Q

How do you assess for ocular motor weakness with MG?

A
  • Upgaze for 30-60 seconds leads to asymmetric ptosis

- facial muscle weakness

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29
Q

What are the oropharyngeal s/sx of MG? (2)

A
  • Flaccid dysarthria with nasal regurgitation

- Jaw weakness

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30
Q

Which is usually affected more often with MG: upper or lower extremities?

A

Upper extremities–particularly the deltoid and wrist extensors

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31
Q

What is myasthenia crisis?

A

Respiratory failure 2/2 intercostal and diaphragmatic weakness

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32
Q

How do you monitor myasthenia crisis?

A

Follow VC and NIF (negative inspiratory forces)

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33
Q

What are the antibodies that mediate MG?

A

Anti-ACh antibodies

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34
Q

What is the diagnostic drug and treatment drug for MG?

A
  • Edrophonium is test

- Pyridostigmine is treatment

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35
Q

How do you diagnose MG? (3)

A
  • Edrophonium test
  • Electrodiagnostic repetitive stimulation
  • Anti-ACh antibodies test
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36
Q

True or false: there is a good correlation between the titer levels of Anti-ACh antibodies and the severity of MG

A

False

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37
Q

What are the CT chest findings of MG?

A

Thymomas

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38
Q

What is the treatment for MG?

A

Cholinesterase inhibitors–Pyridostigmine

39
Q

What are the side effects of Pyridostigmine?

A

Wet picture–GI hyperactivity

40
Q

What is the immunotherapy/surgical treatment for MG?

A

Thymectomy can reduce the immune response

41
Q

What is the role of immunosuppressants in the treatment of MG?

A

All can improve weakness, but carry their own side effects.

42
Q

What is the treatment for a MG crisis? (2)

A
  • Plasma exchange

- IVIG

43
Q

Are myopathies usually bilateral or unilateral? Proximal or distal?

A

Bilateral

Proximal

44
Q

What is the characteristics gait with myopathy?

A

Waddling gait since it involves the stronger truncal muscles

45
Q

Is there preserved sensation/reflexes with myopathies?

A

Yes

46
Q

Are CKs useful for diagnosing myopathies

A

Yes–elevated in majority of myopathies, but also elevated in rhabdo and seizures

47
Q

What are the primary inflammatory myopathies?

A

Dermatomyositis
Polymyositis
Inclusion body myositis

48
Q

What is polymyositis? Progression? S/sx?

A

Polymyositis is an inflammatory myopathy mediated by cytotoxic T cells with an as yet unknown autoantigen

Subacute proximal weakness–painless

Spares eye, face
Neck weakness

49
Q

True or false: there is a high incidence of malignancy causing polymyositis

A

False

50
Q

How do you diagnose polymyositis? (3)

A
  • Elevated CK
  • EMG with myopathic and inflammatory changes
  • Muscle bx (definitive)
51
Q

What is the treatment for polymyositis?

A

Immunosuppressants

52
Q

When does dermatomyositis usually present? Cause?

A
  • Childhood or adult onset

- Unknown, but may be related to viral or bacterial infx causing autoimmune response

53
Q

What are the s/sx of dermatomyositis?

A

skin rash and proximal muscle weakness

54
Q

What is the treatment for dermatomyositis?

A

Immunosuppressants

55
Q

When does inclusion body myositis usually present?

A

Adult onset

56
Q

What are the s/sx of inclusion body myositis?

A

Finger/wrist flexor and quad weakness

57
Q

How does inclusion body myopathy respond to steroids?

A

Does not

58
Q

How does the progression of disease differ between polymyositis, dermatomyositis, and inclusion body myositis?

A

Polymyositis and dermatomyositis are subacute in onset, while inclusion body is a slow, chronic condition

59
Q

What are the muscles that are usually affected with inclusion body myositis?

A

Finger/wrist flexor weakness

60
Q

What is the major risk factor for steroid myopathy?

A

More than 30 mg of prednisone QD for more than 2 months

61
Q

What is the usual onset of steroid induced myopathy

A

Subacute to chronic presentation of proximal weakness

62
Q

What are the EMG findings of steroid myopathies?

A

Normal usually

63
Q

What are the CK findings of steroid myopathy?

A

Normal

64
Q

True or false: all cholesterol lowering agents have the potential to cause myopathic effects

A

True

65
Q

What is statin myopathy?

A

Any muscle complaint related to statin use

66
Q

What is the difference between myalgias and myositis?

A

Myositis has CK elevation

67
Q

What are the CK level with rhabdo?

A

more than 10x normal with elevated Cr

68
Q

What is the treatment for statin myositis?

A

If rhabdo, d/c drug and change

69
Q

Unless CK elevates (__)x with statin use, there is no need to stop statin therapy

A

10x

70
Q

What are the motor symptoms of motor neuron disease?

A

Asymmetric
Varibale location
Atrophy
Fasciculations

71
Q

What are the motor symptoms of neuropathies (symmetric/asymmetric? Distal or proximal?)

A

Symmetric

Distal more than proximal

72
Q

What are the motor symptoms of myopathies (symmetric/asymmetric? Distal or proximal?)

A

Symmetric

Proximal greater than distal

73
Q

What are the sensory symptoms of motor neuron diseases?

A

Absent

74
Q

What are the sensory symptoms of neuropathies?

A
  • Paresthesias
  • Dysesthesias
  • Autonomic s/sx
75
Q

What are the sensory symptoms of NMJ diseases?

A

Absent

76
Q

What are the sensory symptoms of myopathies?

A

Absent

77
Q

What are the reflex findings with Motor neuron disease?

A

Hyperactive

78
Q

What are the reflex findings with neuropathies?

A

Diminished

79
Q

What are the reflex findings with NMJ/myopathies

A

normal

80
Q

What is asymmetric proximal motor neuropathy (diabetic amyotrophy)?

A

Prominent anterior thigh pain with weakness of quadriceps/iliopsoas, with loss of patellar reflex

81
Q

True or false: the loss of the calcaneal reflex is always pathologic

A

false–very commonly lost with normal aging process

82
Q

How can you tell if MG patients will progress to a systemic form of the disease?

A

83% of patients will progress to generalized disease in less than 36 months

83
Q

What is the manifestation of MCAD deficiency?

A

Non-ketotic hypoglycemia

84
Q

What is the classic presentation of Carnitine deficiency?

A

Non-ketotic hypoglycemia

85
Q

What is type I - V GSD?

A
I = glucose-6-dehydro deficiency
II = Acid maltase (alpha glucosidase deficiency)
III = Cori disease (debranching)
V = McArdle's disease (glycogen phosphorylase)
86
Q

What is the cause and presentation of LHON?

A

Sudden onset of blindness in young adults 2/2 mutations in subunits of complex I of the ETC

87
Q

What is the cause and presentation of MERRF?

A

-myoclonic epilepsy and ragged red fibers
0point mutation for lysine tRNA leads to mitochondria containing paracrystalline arrays, and diminished cytochrome oxidase deficiency

88
Q

What is the cause and presentation of MELAS?

A
  • Mitochondrial encephalopathy, lactic acidosis, stroke-like activity
  • point mutation in leucine tRNA
89
Q

Both MELAS and MERRF have ragged red fibers. How can you differentiate the two?

A

Cytochrome oxidase activity is normal in MELAS, but diminished in MERRF

90
Q

True or false: there are usually no malignancies associated with inclusion body myositis

A

True

91
Q

What causes inclusion body myositis?

A

T cells mediated attacked against muscles, causing vacuoles to develop in muscles with abnormal protein deposits

92
Q

What are the major muscles that are affected with NMJ disorders?

A

Cranial, limb girdle, and proximal muscles

93
Q

Which usually has autonomic s/sx: MG or Eaton-Lambert?

A

Eaton-Lambert