Wk 4 ALS Flashcards

1
Q

What does ALS stand for?

A

Amyotrophic lateral sclerosis

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2
Q

What is the name of the baseball player who was diagnosed with ALS?

A

Lou Gehrig (Lou Gehrig’s disease)

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3
Q

What is the definition of ALS?

A

Rare, progressive, degenerative neurologic disorder characterized by the loss of motor neurons

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4
Q

ALS involves the loss of both __ and __ motor neurons

A

upper and lower

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5
Q

What is the prognosis of ALS?

A

Death usually occurs 3 years after diagnosis

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6
Q

What is the etiology of ALS?

A

Unknown

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7
Q

ALS is fatal, usually due to __ __

A

respiratory failure

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8
Q

What age does ALS begin?

A

40s

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9
Q

What age does ALS peak?

A

68-70

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10
Q

What gender is at higher risk for ALS?

A

Males

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11
Q

About 10% of ALS is __

A

genetic

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12
Q

What lifestyle is considered to be a risk factor for ALS?

A

Smoking

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13
Q

What three places do degeneration of large motor neurons occur with ALS?

A

Spinal cord
Cerebral cortex
Brain stem

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14
Q

Electrical and chemical messages in the brain do not reach the __

A

muscles

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15
Q

Degeneration of the neurons leads to… (3)

A

Exonal degeneration
Demyelination
Sclerosis

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16
Q

What is the excitotoxicity hypothesis?

A

Excessive levels of glutamate initiate a cascade of events that lead to neuron death

17
Q

What is glutamate?

A

Excitatory neurotransmitter

18
Q

Where did the excitotoxicity hypothesis come from?

A

High levels of glutamate were found in the CSF of patients with CSF

19
Q

What antiglutamineric drug improves survival rates among ALS patients?

A

Riluzole

20
Q

Early manifestations may include motor dysfunction of which parts of the body?

A

Upper, lower or both

21
Q

Over half of patients with ALS have the __ form of the disease

A

spinal

22
Q

If an ALS patient has the spinal form of the disease, where does muscle weakness start?

A

In the extremities

23
Q

Spinal ALS leads to what 4 losses?

A

Muscle atrophy
Loss of dexterity
Loss of gait
Loss of spasticity

24
Q

What are s/s of ALS?

A
Dysarthria 
Dysphagia
Drooling
Cognitive and behavioral changes
Constipation
Sleep problems
Breathing
25
Q

What drug class is riluzole?

A

Glutamate inhibitor

26
Q

What is the brand name of riluzole?

A

Rilutek

27
Q

What is the therapeutic indication of riluzole?

A

Only approved medication to treat ALS

28
Q

What is the MOA of riluzole?

A

Glutamate antagonist, reduces damage to motor neurons

29
Q

What are 3 side effects of riluzole?

A

Dizziness
GI upset
Hepatotoxicity

30
Q

How long does riluzole expand a patient’s lifespan?

A

Only 3-6 months