Amyotropic Lateral Sclerosis Flashcards Preview

724: Medical Lectures > Amyotropic Lateral Sclerosis > Flashcards

Flashcards in Amyotropic Lateral Sclerosis Deck (35)
1

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?

Amyotrophic lateral sclerosis

2

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

- sporadic (90%)
- familial (10%)

3

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

1.5-2.7

4

What ethnic group has the highest rate of ALS?

Caucasians

5

Is ALS more common in males or females?

even

6

What age range has the highest chance of developing ALS?

40-75

7

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

- Guam
- West New Guinea
- Kii Peninsula Japan

8

What is the basic pathology behind ALS?

Motor neuron degeneration and death with gliosis replacing the neurons

9

Spinal cord atrophy associated with ALS causes what?

loss of peripheral innervation and muscle atrophy

10

What are the 6 signs and symptoms of ALS?

- Dysphagia
- Dysarthria
- Muscle weakness in hands, arms, and legs
- Fasciculation of muscles
- Difficulty projecting voice
- Shortness of breath

11

Symptoms begin in what muscles?

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

12

What is the average survival after diagnosis?

3-5 years

13

What is long-term survival associated with?

- Younger age at symptom onset
- Male gender
- Limb rather than bulbar symptom onset

14

What is stongly suggestive of ALS?

The presence of UMN and LMN signs

15

True or False

MRI is typically normal in patients with ALS

True

16

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

Creatine phosphokinase

17

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

myopathy

18

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

- SOD1
- TDP-43
- FUS

19

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

50

20

Pulmonary assessments must be done every _ months after diagnosis

3

21

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

60

22

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

50

23

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

25-30

24

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

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