Chapter 32- Neuromuscular Diseases

Question 1

Signs and symptoms of neuromuscular diseases

Answer 1

Exertional dyspnea
Fatigue
Orthopnea
Cor pulmonale

Question 2

What are the major consequences of resp muscle weakness

Answer 2

Resp insufficiency and inability to clear secretions

Question 3

What does orthopnea indicate

Answer 3

Diaphragm weakness

Question 4

How can neuromuscular disease be suspected with normal pft

Answer 4

Repeat while patient is supine, if fev1 or fvc is 20 percent less, diaphragm weakness is present

Question 5

What is diaphragm pacing

Answer 5

Direct stimulation of an intact phrenic nerve to contract the diaphragm

Question 6

What are suggested niv settings for neuromuscular patients

Answer 6

Lower Epap-2-3

Higher iPap-7-15

Question 7

What is critical illness myopathy

Answer 7

Flaccid weakness of proximal muscles making patients difficult to wean from mv

Question 8

What are risk factors for developing critical illness myopathy

Answer 8

Corticosteroids use
Paralytics
Hyperglycaemia
Hyperthyroidism

Question 9

How is critical illness myopathy prevented

Answer 9

Avoiding risk factors

Question 10

What are 3 disorders of the neuromuscular junction

Answer 10

Mg
Lambert-eaten syndrome
Poisoning (botulism, tetanus)

Question 11

What is mg

Answer 11

Intermittent muscular weakness

Question 12

How can mg be differentiated from other neuromuscular disorders

Answer 12

Tensilion test- muscle weakness will improve when given eprophonium or neostigmine

Question 13

How does mg arise

Answer 13

Production of antibodies against ach

Antibodies deactivate ach and block nerve transmission

Question 14

Mg typically starts where

Answer 14

In the eyelids

Question 15

What is myasthenic crisis

Answer 15

Respiratory failure or inability to maintain airway

Question 16

What treatments are considered for mg

Answer 16

Anti cholinesterase meds- neostigmine, edrophonium
Corticosteroids
Immunosuppressants
IgG in myasthenic crisis

Question 17

Which neuromuscular syndrome is associated with cancer

Answer 17

Lambert Eaton syndrome

Question 18

What is gbs

Answer 18

An acute inflammatory demyleating neuromuscular disease causing resp insufficiency

Question 19

Autonomic nervous system problems such as hypotension, flushing, bradycardia are common with

Answer 19

Gbs

Question 20

Gbs typically improves within

Answer 20

2-4 weeks after progression stops

Question 21

What is critical Illness polyneuropathy

Answer 21

Muscle weakness, atrophy, loss of deep tendon reflexes and loss of peripheral sensation.

Question 22

Critical illness polyneuropathy has a strong correlation with

Answer 22

Severe sepsis

Question 23

What is another name for als

Answer 23

Lou Gehrig disease

Question 24

What is the one approved medication for als

Answer 24

Riluzole

Question 25

What is als

Answer 25

Progressive degeneration of motor neurons resulting in respiratory involvement

Question 26

Fasiculations and quivering if muscles is seen in which neuromuscular disease

Answer 26

Als

Question 27

How can respiratory complications be prevented with als

Answer 27

Feeding tubes or modifying food texture Clearing of secretions Niv respiratory support

Question 28

High cervical cord injuries occur where

Answer 28

C1-2

Question 29

Middle to low cervical injury is where?

Answer 29

C3-8

Question 30

Diaphragm is innervated by which nerve roots

Answer 30

Phrenic nerves, located c3-5

Question 31

Injury above c3-5 results in

Answer 31

Diaphragm paralysis

Question 32

What is the hallmark sign of significant bilateral diaphragm weakness

Answer 32

Abdominal paradox

Question 33

Expiratory muscles are innervated where

Answer 33

T1-l1

Question 34

Injury to t1-l1 would cause

Answer 34

Diminished or absent cough

Question 35

Patients with inspiratory muscle weakness would prefer to be in what position

Answer 35

Sitting up

Question 36

Patients with expiratory muscle weakness would prefer to be in what position

Answer 36

Supine

Question 37

Spinal cord injury causes muscle paralysis above or below the level of injury

Answer 37

Below

Question 38

Periodic breathing is often seen In what type of stroke

Answer 38

Subarachnoid hemorrhage

Question 39

Ataxic breathing (irregular resp rate and vt) is associated with

Answer 39

Medula stroke