Amyotropic Lateral Sclerosis Flashcards

1
Q

What can be defined as a progressive degeneration of the motor neurons of the central nervous system, leading to wasting of the muscles and paralysis?

A

Amyotrophic lateral sclerosis

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2
Q

What are the 2 classifications of ALS? Which is most common?

A
  • sporadic (90%)

- familial (10%)

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3
Q

ALS incidence rate among Europe and North America ranges from __-__ for every 100,000 per year

A

1.5-2.7

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4
Q

What ethnic group has the highest rate of ALS?

A

Caucasians

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5
Q

Is ALS more common in males or females?

A

even

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6
Q

What age range has the highest chance of developing ALS?

A

40-75

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7
Q

There is a high prevalence in what 3 regions of the western Pacific?

A
  • Guam
  • West New Guinea
  • Kii Peninsula Japan
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8
Q

What is the basic pathology behind ALS?

A

Motor neuron degeneration and death with gliosis replacing the neurons

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9
Q

Spinal cord atrophy associated with ALS causes what?

A

loss of peripheral innervation and muscle atrophy

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10
Q

What are the 6 signs and symptoms of ALS?

A
  • Dysphagia
  • Dysarthria
  • Muscle weakness in hands, arms, and legs
  • Fasciculation of muscles
  • Difficulty projecting voice
  • Shortness of breath
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11
Q

Symptoms begin in what muscles?

A

in the muscles of speech, swallowing, or in hands, arms, and legs

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12
Q

What is the average survival after diagnosis?

A

3-5 years

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13
Q

What is long-term survival associated with?

A
  • Younger age at symptom onset
  • Male gender
  • Limb rather than bulbar symptom onset
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14
Q

What is stongly suggestive of ALS?

A

The presence of UMN and LMN signs

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15
Q

True or False

MRI is typically normal in patients with ALS

A

True

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16
Q

Levels of what are elevated in approximately 70% of patients diagnosed with ALS?

A

Creatine phosphokinase

17
Q

A muscle biopsy is performed when ____ is suspected opposed to ALS

18
Q

Genetic testing is used to identify mutations in what 3 genes?

A
  • SOD1
  • TDP-43
  • FUS
19
Q

__% of persons with familial ALS will have a mutation in once of these genes

20
Q

Pulmonary assessments must be done every _ months after diagnosis

21
Q

If vital capacity is greater than __% respiratory management can be deferred

22
Q

A vital capacity less than __% is often associated with respiratory failure and management is required

23
Q

Vital capacity less than __-__% is often associated with respiratory failure and sudden death

24
Q

What increases the risk for insufficient caloric and fluid intake/worsening of weakness and fatigue?

A

Dysphagia (difficulty swallowing)

25
What drugs can be prescribed in patients with intermittent dyspnea?
inhaled opiates (morphine) or IV midazolam
26
What drugs can be prescribed in patients with constant dyspnea?
IV morphine every 4 hours
27
What is debilitating fatigue treated with?
modafinil
28
What 3 drugs are used to treat muscle spasms?
- levetiracetam - carbamazepine - phenytoin
29
What 2 drugs are used to treat spasticity?
- Baclofen | - Tizanidine
30
What symptom is very common in ALS patients due facial muscle weakness & decreased swallowing ability?
Sialorrhea
31
What 4 drugs are used to treat sialorrhea?
- Atropine - Hyoscyamine - Amitriptyline - Glycopyrrolate
32
Describe the pseudobulbar affect associated with ALS
Patients experience sudden uncontrollable outbursts of laughter or tearfulness
33
The pseudobulbar affects __% of patients
50
34
What 3 drugs are used to treat the pseudobulbar affect?
- combination of dextromethorphan-quinidine - Amitriptyline - Fluvoxamine
35
What is the only FDA regulated drug to treat ALS
Riluzole