Chapter 32- Neuromuscular Diseases Flashcards

1
Q

Signs and symptoms of neuromuscular diseases

A

Exertional dyspnea
Fatigue
Orthopnea
Cor pulmonale

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2
Q

What are the major consequences of resp muscle weakness

A

Resp insufficiency and inability to clear secretions

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3
Q

What does orthopnea indicate

A

Diaphragm weakness

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4
Q

How can neuromuscular disease be suspected with normal pft

A

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

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5
Q

What is diaphragm pacing

A

Direct stimulation of an intact phrenic nerve to contract the diaphragm

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6
Q

What are suggested niv settings for neuromuscular patients

A

Lower Epap-2-3

Higher iPap-7-15

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7
Q

What is critical illness myopathy

A

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

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8
Q

What are risk factors for developing critical illness myopathy

A

Corticosteroids use
Paralytics
Hyperglycaemia
Hyperthyroidism

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9
Q

How is critical illness myopathy prevented

A

Avoiding risk factors

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10
Q

What are 3 disorders of the neuromuscular junction

A

Mg
Lambert-eaten syndrome
Poisoning (botulism, tetanus)

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11
Q

What is mg

A

Intermittent muscular weakness

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12
Q

How can mg be differentiated from other neuromuscular disorders

A

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

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13
Q

How does mg arise

A

Production of antibodies against ach

Antibodies deactivate ach and block nerve transmission

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14
Q

Mg typically starts where

A

In the eyelids

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15
Q

What is myasthenic crisis

A

Respiratory failure or inability to maintain airway

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16
Q

What treatments are considered for mg

A

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

17
Q

Which neuromuscular syndrome is associated with cancer

A

Lambert Eaton syndrome

18
Q

What is gbs

A

An acute inflammatory demyleating neuromuscular disease causing resp insufficiency

19
Q

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

A

Gbs

20
Q

Gbs typically improves within

A

2-4 weeks after progression stops

21
Q

What is critical Illness polyneuropathy

A

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

22
Q

Critical illness polyneuropathy has a strong correlation with

A

Severe sepsis

23
Q

What is another name for als

A

Lou Gehrig disease

24
Q

What is the one approved medication for als

A

Riluzole

25
Q

What is als

A

Progressive degeneration of motor neurons resulting in respiratory involvement

26
Q

Fasiculations and quivering if muscles is seen in which neuromuscular disease

A

Als

27
Q

How can respiratory complications be prevented with als

A

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

28
Q

High cervical cord injuries occur where

A

C1-2

29
Q

Middle to low cervical injury is where?

A

C3-8

30
Q

Diaphragm is innervated by which nerve roots

A

Phrenic nerves, located c3-5

31
Q

Injury above c3-5 results in

A

Diaphragm paralysis

32
Q

What is the hallmark sign of significant bilateral diaphragm weakness

A

Abdominal paradox

33
Q

Expiratory muscles are innervated where

A

T1-l1

34
Q

Injury to t1-l1 would cause

A

Diminished or absent cough

35
Q

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

A

Sitting up

36
Q

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

A

Supine

37
Q

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

A

Below

38
Q

Periodic breathing is often seen In what type of stroke

A

Subarachnoid hemorrhage

39
Q

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

A

Medula stroke