Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

SBM neurology > Quiz 3 - disorders of neuromuscular system > Flashcards

Quiz 3 - disorders of neuromuscular system Flashcards

1
Q

myelin in the peripheral nerves comes from…..

A

Schwann cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

myelin quantity and function

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

more sensory loss in peripheral or nerve root damage?

A

peripheral, in nerve root damage there is overlap

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

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

A

anterior horn cell or its axons

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

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

A

compensation for loss of motor neurons or axons

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

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

A

Werdnig-Hoffman - infants

Kugelberg-Welander - children/young adults

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

ALS damage is usually to what?

A

both upper and lower motor neurons

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

who usually gets ALS

A

40-60yos

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

weakness in ALS

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

CN involvement in ALS

A
  • tonge, face, phalanx, soft palate

- NOT eyes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

longevity and death in ALS

A
  • 3-5 years

- due to respiratory muscle weakness and superimposed infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

ALS involving only upper motor neurons is called

A

primary lateral sclerosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

ALS involving only lower motor neurons is called

A

progressive muscular atrophy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

ALS involving only cranial musculature is called

A

progressive bulbar palsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what drug slows down ALS?

A

riluzole

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

3 categories of peripheral nerve damage

A

1) mononeuropathy
2) mononeuropathy multiplex
3) polyneuropathy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

mononeuropathy is most often due to

A

trauma

entrapment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

PE technique for mononeuropathy

A

Tinel sign, tapping at place of damage for mechanical sensitivity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

most common mononeuropathy

A

carpal tunnel syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

damage to the radial nerve is indicated by

A

wrist drop

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

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

A

ulnar nerve

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

what is radiculopathy?

A

damage to nerve root

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

causes of radiculopathy in younger and older adults

A

younger - disc herniation

older - degenerative issues in discs, bones, joints

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

polyneuropathy distribution

A
  • usually symmetrical
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

why are legs affected first in polyneuropathy?

A
  • longer nerves with higher energy demand
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

indication of larger fiber polyneuropathy

A
  • loss of vibration and joint position sense

- can result in poor balance

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

most common symptoms of polyneuropathy

A
  • numbness

- dysesthesias, paresthesias

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

most common causes of polyneuropathy

A

diabetes mellitus
B12 deficiency
heavy metal exposure
chemo and antivirals

29
Q

diseases that can cause polyneuropathy

A

Lyme
tertiary syphilis
HIV

30
Q

Guillain-Barre (AIDP) differences from typical polyneuropathy

A
  • rapid course - ether ascending or descending
  • severe weakness
  • follows diarrheal or viral disease often
  • lost reflexes
  • high CSF protein levels
31
Q

CIDP

A
  • high CSF protein levels
  • asymmetric polyneuropathy
  • lost reflexes
32
Q

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

A

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

33
Q

Romberg and proprioceptive nerve loss

A
  • will often improve dramatically when touching stationary object with only one finger
34
Q

most common causes of mononeuritis multiplex

A
  • diabetes mellitus
  • ## systemic vasculitis
35
Q

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

A

diabetic amyotrophy

36
Q

who most commonly gets myasthenia gravis?

A
  • young adult women

- small spike in late middle aged men with thymic tumors

37
Q

first signs of myasthenia gravis

A
  • ptosis/diplopia (ocular myasthenia)

- pharynx and soft palate (bulbar myasthenia)

38
Q

Lambert Eaton PE in comparison to MG

A
  • exercise increases rather than decreases strength in Lambert Eaton.
39
Q

almost all myopathies include the following:

A
  • symmetry

- proximal muscle weakness

40
Q

EMG of the motor unit in myopathy

A

small, brief, and polyphasic action potentials

41
Q

two major groups of muscle disease

A
  • destruction of muscle fibers - progressive weakness

- functional defect - with little muscle wasting

42
Q

destructive muscle diseases fall into three categories:

A

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

43
Q

functional muscle diseases fall into two categories:

A

1) myotonic disorders

2) periodic paralysis

44
Q

Duchenne is a type of….

A

muscular dystrophy

45
Q

inheritance of Duchenne dystrophy

A

x-linked

46
Q

Duchenne pathophysiology and disease progression

A
  • 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
Q

contrast Becker to Duchenne

A
  • same gene
  • less severe
  • somewhat later onset
48
Q

prototypical acquired metabolic myopathy

A

thyroid disease

49
Q

medications that can cause metabolic myopathy

A
  • statins
  • colchicine
  • hydroxychloroquine
  • alcohol
50
Q

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

A

metabolic

51
Q

name 3 acquired inflammatory myopathies

A
  • polymyositis
  • dermatomyositis
  • inclusion body myositis
52
Q

metabolic myopathies are either…..

A

genetic or acquired

53
Q

inflammatory myopathies are either…..

A

acquired or autoimmune

54
Q

when do people get polymyositis?

A

30-60

55
Q

contrast polymyositis and dermatomyositis

A

in dermatomyositis

  • purple rash
  • more muscle soreness
  • in children and older adults
  • more commonly associated with autoimmune disorders
56
Q

in polymyositis and dermatomyositis you often see elevations in….

A
  • ESR and C-reactive protein (inflammatory markers)

- CPK

57
Q

presentation of inclusion body myositis

A
  • flexors of fingers and wrists as well as proximal muscles
58
Q

what are the two major myotonic disorders?

A
  • congenital myotonia

- paramyotonia congenita

59
Q

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

A

congenital myotonia - chloride channels, improves with exercise

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

60
Q

presentation of periodic paralysis

A
  • weakness after large meals or following exercise
61
Q

presentation of limb-girdle dystrophy

A
  • slow progressing

- proximal limb weakness

62
Q

limb-girdle dystrophy inheritance

A
  • 15 different mutations

- recessive or dominant depending

63
Q

facioscapulohumeralm muscular dystrophy presentation

A
  • adolescence/early adulthood

- chromosome 4 hypomethylation

64
Q

presentation of two types of myotonic dystrophy

A

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

type 2 (less common),

65
Q

pathophysiology of type 1 myotonic dystrophy

A
  • autosomal dominant
  • trinucleotide repeat disease
  • myotonic protein kinase
  • starts in childhood or young adult life, sometimes even infancy
66
Q

pathophysiology of type 2 myotonic dystrophy

A
  • quadronucleotide repeats
  • transcription factor
  • 20-30yos
67
Q

oculopharyngeal dystrophy pathophysiology

A
  • trinucleotide repeats

- abnormal intranuclear protein for mRNA trafficking

68
Q

oculopharyngeal dystrophy presentation

A
  • ptosis in fifth decade of life

- progresses to paralysis of eye muscles and throat muscles

SBM neurology (22 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions