022614 1 Neuromuscular Diseases Pathology SDL Flashcards Preview

B&B Exam 1 > 022614 1 Neuromuscular Diseases Pathology SDL > Flashcards

Flashcards in 022614 1 Neuromuscular Diseases Pathology SDL Deck (22):
1

What are the three categories of peripheral nerve disorders?

mononeuropathy
mononeuritis complex
polyneuropathy

2

What type of disease is guillain-barre syndrome?

polyneuropathy

3

What is guillain-barre syndrome?

rapid, ascending, symmetrical demyelination of motor axons only beginning distally, eventually leading to respiratory weakness.

4

What is chronic inflammatory demyelinating polyneuropathy (CIDP)?

Chronic GBS, but less severe, involves sensory axons too, and follows relapsing course.

5

What three findings are characteristic of diabetic polyneuropathy?

accumulation of glycosylation end products
hyperglycemia
increased protein C levels

6

What is a clinical finding in myasthenia gravis other than anti-Ach autoantibodies and muscle specific kinase antibody (MUSK)?

ptosis and diplopia due to weakened extraocular muscles

7

What happens during lambert eaton syndrome (LEMS)?

autoantibodies inhibit calcium channels which reduces release of Ach

8

Do clostridium tetani and clostridium botulinum release neurotoxins that interfere with NMJ’s?

yes

9

Emery dreifuss muscular dystrophy (EMD) is characterized by what?

mutations in emerin or lamin A/C

10

Hyperkalemic periodic paralysis is characterized by what?

mutation in sodium channel protein SCN4A

11

Malignant hyperthermia is characterized by what?

mutations in ryanodine receptor

12

What are the three inflammatory myopathies?

polymyositis
dermatomyositis
inclusion body myositis

13

Histologic findings in polymyositis?

intrafascicular inflammation,
increased expression of MHC-1 on myofibers leading to autoimmune attack by cytotoxic T cells,
pain

14

Histologic findings in dermatomyositis?

extrafascicular inflammation
perifascicular atrophy
pain and rash

15

Inclusion body myositis is characterized by what?

rimmed vacuoles

16

Inclusion body myositis is resistant to steroids. T or F?

T

17

What is the primary finding in mitochondrial myopathy?

ragged red fibers

18

Myofiber degeneration/regeneration, internalization of nuclei, scar formation, and inflammation
are general changes that occur during ______?

myopathy

19

What are the three congenital myopathies?

central core myopathy
rod body myopathy
centronuclear myopathy

20

What is present during early denervation?

small angular fibers due to loss of motor unit

21

What is present during chronic denervation?

large motor units due to fiber type grouping and axonal sprouting

22

What is present during late denervation?

grouped atrophy due to loss of motor unit