Quiz 3 - disorders of neuromuscular system Flashcards Preview

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Flashcards in Quiz 3 - disorders of neuromuscular system Deck (68):
1

myelin in the peripheral nerves comes from.....

Schwann cells

2

myelin quantity and function

heavy - somatic motor, sensory to reflexes
intermediate - touch, proprioception, joint position
light - sharp pain, autonomic motor preganglionic
none - burning, aching, temp, postganglionic autonomic

3

more sensory loss in peripheral or nerve root damage?

peripheral, in nerve root damage there is overlap

4

fasciculations, along with weakness and/or atrophy indicates damage to what?

anterior horn cell or its axons

5

what does weakness with a high firing rate of motor units indicate?

compensation for loss of motor neurons or axons

6

what are the two pediatric inherited anterior horn cell degenerative diseases and who do they affect?

Werdnig-Hoffman - infants
Kugelberg-Welander - children/young adults

7

ALS damage is usually to what?

both upper and lower motor neurons

8

who usually gets ALS

40-60yos

9

weakness in ALS

can be varied between upper and lower motor neurons in single patient

10

CN involvement in ALS

- tonge, face, phalanx, soft palate
- NOT eyes

11

longevity and death in ALS

- 3-5 years
- due to respiratory muscle weakness and superimposed infection

12

ALS involving only upper motor neurons is called

primary lateral sclerosis

13

ALS involving only lower motor neurons is called

progressive muscular atrophy

14

ALS involving only cranial musculature is called

progressive bulbar palsy

15

what drug slows down ALS?

riluzole

16

3 categories of peripheral nerve damage

1) mononeuropathy
2) mononeuropathy multiplex
3) polyneuropathy

17

mononeuropathy is most often due to

trauma
entrapment

18

PE technique for mononeuropathy

Tinel sign, tapping at place of damage for mechanical sensitivity

19

most common mononeuropathy

carpal tunnel syndrome

20

damage to the radial nerve is indicated by

wrist drop

21

nerve damage in thoracic outlet syndrome is in the distribution of the....

ulnar nerve

22

what is radiculopathy?

damage to nerve root

23

causes of radiculopathy in younger and older adults

younger - disc herniation
older - degenerative issues in discs, bones, joints

24

polyneuropathy distribution

- usually symmetrical

25

why are legs affected first in polyneuropathy?

- longer nerves with higher energy demand

26

indication of larger fiber polyneuropathy

- loss of vibration and joint position sense
- can result in poor balance

27

most common symptoms of polyneuropathy

- numbness
- dysesthesias, paresthesias

28

most common causes of polyneuropathy

diabetes mellitus
B12 deficiency
heavy metal exposure
chemo and antivirals

29

diseases that can cause polyneuropathy

Lyme
tertiary syphilis
HIV

30

Guillain-Barre (AIDP) differences from typical polyneuropathy

- rapid course - ether ascending or descending
- severe weakness
- follows diarrheal or viral disease often
- lost reflexes
- high CSF protein levels

31

CIDP

- high CSF protein levels
- asymmetric polyneuropathy
- lost reflexes

32

name a relatively common inherited polyneuropathy and what are the two forms?

Charcot-Marie-Tooth disease (HMSN)
type 1 - demyelinating - nerve conduction testing
type 2 - axonal

33

Romberg and proprioceptive nerve loss

- will often improve dramatically when touching stationary object with only one finger

34

most common causes of mononeuritis multiplex

- diabetes mellitus
- systemic vasculitis
-

35

diabetic inflammatory disorder of the lumbar or rarely the brachial plexus resulting in weakness of quads and loss of patellar reflex

diabetic amyotrophy

36

who most commonly gets myasthenia gravis?

- young adult women
- small spike in late middle aged men with thymic tumors

37

first signs of myasthenia gravis

- ptosis/diplopia (ocular myasthenia)
- pharynx and soft palate (bulbar myasthenia)

38

Lambert Eaton PE in comparison to MG

- exercise increases rather than decreases strength in Lambert Eaton.

39

almost all myopathies include the following:

- symmetry
- proximal muscle weakness

40

EMG of the motor unit in myopathy

small, brief, and polyphasic action potentials

41

two major groups of muscle disease

- destruction of muscle fibers - progressive weakness
- functional defect - with little muscle wasting

42

destructive muscle diseases fall into three categories:

1) muscular dystrophies
2) metabolic myopathies
3) inflammatory myopathies

43

functional muscle diseases fall into two categories:

1) myotonic disorders
2) periodic paralysis

44

Duchenne is a type of....

muscular dystrophy

45

inheritance of Duchenne dystrophy

x-linked

46

Duchenne pathophysiology and disease progression

- disrupts a protein in muscle structure
- presents after child is walking because another protein in early childhood takes its place
- progressive decline in muscle strength until death in early 20s

47

contrast Becker to Duchenne

- same gene
- less severe
- somewhat later onset

48

prototypical acquired metabolic myopathy

thyroid disease

49

medications that can cause metabolic myopathy

- statins
- colchicine
- hydroxychloroquine
- alcohol

50

mitochondrial (Kearn-Sayer) diseases are what type of myopathy?

metabolic

51

name 3 acquired inflammatory myopathies

- polymyositis
- dermatomyositis
- inclusion body myositis

52

metabolic myopathies are either.....

genetic or acquired

53

inflammatory myopathies are either.....

acquired or autoimmune

54

when do people get polymyositis?

30-60

55

contrast polymyositis and dermatomyositis

in dermatomyositis
- purple rash
- more muscle soreness
- in children and older adults
- more commonly associated with autoimmune disorders

56

in polymyositis and dermatomyositis you often see elevations in....

- ESR and C-reactive protein (inflammatory markers)
- CPK

57

presentation of inclusion body myositis

- flexors of fingers and wrists as well as proximal muscles

58

what are the two major myotonic disorders?

- congenital myotonia
- paramyotonia congenita

59

contrast congenital myotonia and paramyotonia congenita in terms of channel affected and presentation

congenital myotonia - chloride channels, improves with exercise

paramyotonia congenita - sodium channels, worsens with exercise and with cold

60

presentation of periodic paralysis

- weakness after large meals or following exercise

61

presentation of limb-girdle dystrophy

- slow progressing
- proximal limb weakness

62

limb-girdle dystrophy inheritance

- 15 different mutations
- recessive or dominant depending

63

facioscapulohumeralm muscular dystrophy presentation

- adolescence/early adulthood
- chromosome 4 hypomethylation

64

presentation of two types of myotonic dystrophy

type 1 (more common), distal to proximal, face involved early, numerous non-neurologic problems

type 2 (less common),

65

pathophysiology of type 1 myotonic dystrophy

- autosomal dominant
- trinucleotide repeat disease
- myotonic protein kinase
- starts in childhood or young adult life, sometimes even infancy

66

pathophysiology of type 2 myotonic dystrophy

- quadronucleotide repeats
- transcription factor
- 20-30yos

67

oculopharyngeal dystrophy pathophysiology

- trinucleotide repeats
- abnormal intranuclear protein for mRNA trafficking

68

oculopharyngeal dystrophy presentation

- ptosis in fifth decade of life
- progresses to paralysis of eye muscles and throat muscles