Quiz 3 - disorders of neuromuscular system

Question 1

myelin in the peripheral nerves comes from…..

Answer 1

Schwann cells

Question 2

myelin quantity and function

Answer 2

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

Question 3

more sensory loss in peripheral or nerve root damage?

Answer 3

peripheral, in nerve root damage there is overlap

Question 4

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

Answer 4

anterior horn cell or its axons

Question 5

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

Answer 5

compensation for loss of motor neurons or axons

Question 6

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

Answer 6

Werdnig-Hoffman - infants

Kugelberg-Welander - children/young adults

Question 7

ALS damage is usually to what?

Answer 7

both upper and lower motor neurons

Question 8

who usually gets ALS

Answer 8

40-60yos

Question 9

weakness in ALS

Answer 9

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

Question 10

CN involvement in ALS

Answer 10

• tonge, face, phalanx, soft palate

- NOT eyes

Question 11

longevity and death in ALS

Answer 11

• 3-5 years

- due to respiratory muscle weakness and superimposed infection

Question 12

ALS involving only upper motor neurons is called

Answer 12

primary lateral sclerosis

Question 13

ALS involving only lower motor neurons is called

Answer 13

progressive muscular atrophy

Question 14

ALS involving only cranial musculature is called

Answer 14

progressive bulbar palsy

Question 15

what drug slows down ALS?

Answer 15

riluzole

Question 16

3 categories of peripheral nerve damage

Answer 16

1) mononeuropathy
2) mononeuropathy multiplex
3) polyneuropathy

Question 17

mononeuropathy is most often due to

Answer 17

trauma

entrapment

Question 18

PE technique for mononeuropathy

Answer 18

Tinel sign, tapping at place of damage for mechanical sensitivity

Question 19

most common mononeuropathy

Answer 19

carpal tunnel syndrome

Question 20

damage to the radial nerve is indicated by

Answer 20

wrist drop

Question 21

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

Answer 21

ulnar nerve

Question 22

what is radiculopathy?

Answer 22

damage to nerve root

Question 23

causes of radiculopathy in younger and older adults

Answer 23

younger - disc herniation

older - degenerative issues in discs, bones, joints

Question 24

polyneuropathy distribution

Answer 24

• usually symmetrical

Question 25

why are legs affected first in polyneuropathy?

Answer 25

- longer nerves with higher energy demand

Question 26

indication of larger fiber polyneuropathy

Answer 26

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

Question 27

most common symptoms of polyneuropathy

Answer 27

- numbness | - dysesthesias, paresthesias

Question 28

most common causes of polyneuropathy

Answer 28

diabetes mellitus B12 deficiency heavy metal exposure chemo and antivirals

Question 29

diseases that can cause polyneuropathy

Answer 29

Lyme tertiary syphilis HIV

Question 30

Guillain-Barre (AIDP) differences from typical polyneuropathy

Answer 30

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

Question 31

CIDP

Answer 31

- high CSF protein levels - asymmetric polyneuropathy - lost reflexes

Question 32

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

Answer 32

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

Question 33

Romberg and proprioceptive nerve loss

Answer 33

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

Question 34

most common causes of mononeuritis multiplex

Answer 34

- diabetes mellitus - systemic vasculitis -

Question 35

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

Answer 35

diabetic amyotrophy

Question 36

who most commonly gets myasthenia gravis?

Answer 36

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

Question 37

first signs of myasthenia gravis

Answer 37

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

Question 38

Lambert Eaton PE in comparison to MG

Answer 38

- exercise increases rather than decreases strength in Lambert Eaton.

Question 39

almost all myopathies include the following:

Answer 39

- symmetry | - proximal muscle weakness

Question 40

EMG of the motor unit in myopathy

Answer 40

small, brief, and polyphasic action potentials

Question 41

two major groups of muscle disease

Answer 41

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

Question 42

destructive muscle diseases fall into three categories:

Answer 42

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

Question 43

functional muscle diseases fall into two categories:

Answer 43

1) myotonic disorders | 2) periodic paralysis

Question 44

Duchenne is a type of....

Answer 44

muscular dystrophy

Question 45

inheritance of Duchenne dystrophy

Answer 45

x-linked

Question 46

Duchenne pathophysiology and disease progression

Answer 46

- 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

Question 47

contrast Becker to Duchenne

Answer 47

- same gene - less severe - somewhat later onset

Question 48

prototypical acquired metabolic myopathy

Answer 48

thyroid disease

Question 49

medications that can cause metabolic myopathy

Answer 49

- statins - colchicine - hydroxychloroquine - alcohol

Question 50

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

Answer 50

metabolic

Question 51

name 3 acquired inflammatory myopathies

Answer 51

- polymyositis - dermatomyositis - inclusion body myositis

Question 52

metabolic myopathies are either.....

Answer 52

genetic or acquired

Question 53

inflammatory myopathies are either.....

Answer 53

acquired or autoimmune

Question 54

when do people get polymyositis?

Answer 54

30-60

Question 55

contrast polymyositis and dermatomyositis

Answer 55

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

Question 56

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

Answer 56

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

Question 57

presentation of inclusion body myositis

Answer 57

- flexors of fingers and wrists as well as proximal muscles

Question 58

what are the two major myotonic disorders?

Answer 58

- congenital myotonia | - paramyotonia congenita

Question 59

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

Answer 59

congenital myotonia - chloride channels, improves with exercise paramyotonia congenita - sodium channels, worsens with exercise and with cold

Question 60

presentation of periodic paralysis

Answer 60

- weakness after large meals or following exercise

Question 61

presentation of limb-girdle dystrophy

Answer 61

- slow progressing | - proximal limb weakness

Question 62

limb-girdle dystrophy inheritance

Answer 62

- 15 different mutations | - recessive or dominant depending

Question 63

facioscapulohumeralm muscular dystrophy presentation

Answer 63

- adolescence/early adulthood | - chromosome 4 hypomethylation

Question 64

presentation of two types of myotonic dystrophy

Answer 64

type 1 (more common), distal to proximal, face involved early, numerous non-neurologic problems type 2 (less common),

Question 65

pathophysiology of type 1 myotonic dystrophy

Answer 65

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

Question 66

pathophysiology of type 2 myotonic dystrophy

Answer 66

- quadronucleotide repeats - transcription factor - 20-30yos

Question 67

oculopharyngeal dystrophy pathophysiology

Answer 67

- trinucleotide repeats | - abnormal intranuclear protein for mRNA trafficking

Question 68

oculopharyngeal dystrophy presentation

Answer 68

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