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724: Medical Lectures > Amyotropic Lateral Sclerosis > Flashcards

Amyotropic Lateral Sclerosis Flashcards

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USMLE® Step 1 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

A

myopathy

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

A

50

20
Q

Pulmonary assessments must be done every _ months after diagnosis

A

3

21
Q

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

A

60

22
Q

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

A

50

23
Q

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

A

25-30

24
Q

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

A

Dysphagia (difficulty swallowing)

25
Q

What drugs can be prescribed in patients with intermittent dyspnea?

A

inhaled opiates (morphine) or IV midazolam

26
Q

What drugs can be prescribed in patients with constant dyspnea?

A

IV morphine every 4 hours

27
Q

What is debilitating fatigue treated with?

A

modafinil

28
Q

What 3 drugs are used to treat muscle spasms?

A
  • levetiracetam
  • carbamazepine
  • phenytoin
29
Q

What 2 drugs are used to treat spasticity?

A
  • Baclofen

- Tizanidine

30
Q

What symptom is very common in ALS patients due facial muscle weakness & decreased swallowing ability?

A

Sialorrhea

31
Q

What 4 drugs are used to treat sialorrhea?

A
  • Atropine
  • Hyoscyamine
  • Amitriptyline
  • Glycopyrrolate
32
Q

Describe the pseudobulbar affect associated with ALS

A

Patients experience sudden uncontrollable outbursts of laughter or tearfulness

33
Q

The pseudobulbar affects __% of patients

A

50

34
Q

What 3 drugs are used to treat the pseudobulbar affect?

A
  • combination of dextromethorphan-quinidine
  • Amitriptyline
  • Fluvoxamine
35
Q

What is the only FDA regulated drug to treat ALS

A

Riluzole

724: Medical Lectures (42 decks)

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