ALS

Question 1

What does ALS stand for?

Answer 1

Amyotrophic Lateral Sclerosis

Question 2

What are 2 other names for ALS?

Answer 2

1. Lou Gehrig’s

2. Charcots

Question 3

ALS is the most common what type of disorder?

Answer 3

Most common motor neuron problem

Question 4

Is ALS progressive? How long is the duration?

Answer 4

Yes, it is very progressive and a short duration

Question 5

Is ALS more common in men or women? By how much more?

What age group

Answer 5

2X more common in middle aged men

Question 6

1. How quickly would a person die after acquiring ALS?

2. What would they typically die from?

Answer 6

1. 2-5 years

2. Respiratory complications

Question 7

1. Bulbar ALS is a type of ALS, the damage in this ALS begins where?
2. What are the main 2 things affected in this ALS?

Answer 7

1. In the brainstem

2. Speech / swallowing

Question 8

What is the Et of ALS?

(3) (what is the percentage of each?

Answer 8

1. Sporadic (90-95%)
2. Familial (5-10%)
3. Other Factors

Question 9

What do the other factors include that can be an Et of ALS?

Answer 9

Autoimmune, viral

Question 10

1. The sporadic form of ALS involves a mutation in what gene and on what Chromosome?
2. This gene codes for what enzyme?
3. What does this enzyme do?
4. Therefore… if the gene is mutated, therefore enzyme mutated, what occurs?

Answer 10

1. SOD1 (superoxide dismutase gene) gene mutation, on Chrm 21 (~5%)
2. Superoxide dismutase
3. Enzyme breaks up free radicals
4. Free radicals build up and can damage the neurons

Question 11

1. The familal form ALS involves what gene on what Chrm?
2. How much of the familial form of ALS is affected by this gene?
3. There are other genes that can cause ALS, do we know them? What percentage is this?

Answer 11

1. SOD1 on Chrm 21
2. ~20% (therefore more so than the sporadic form)
3. Other genes (80%)

Question 12

The Patho of ALS:

1. Involves what type of degeneration: motor or sensory neuron?
2. Proposed Free radical and or glutamate toxicity: is this m echanism clear?

Answer 12

1. Motor neuron degeneration

2. Unclear

Question 13

The motor neuron degeneration is in what 3 places?

Answer 13

1. Anterior horn cells in spinal chord
2. motor nuclei in brain stem
3. Upper motor neurons in cerebral cortex

Question 14

The anterior horn cells in the spinal chord house what type of neurons?

Answer 14

Motor neurons

Question 15

1. The upper motor neurons in the cerebral cortex, run between ____ and _____?
2. What do the lower neurons run between?

Answer 15

1. brain and spinal chord

2. spinal chord and peripherals

Question 16

The Patho of ALS can involve the motor neuron degeneration as well as what that involves glutamate toxicity?

Answer 16

Proposed free radical and or glutamate toxicity

Question 17

What are the Mnfts of ALS? (3)

Answer 17

1. Muscle atrophy, weakness, fasciculations
2. Dysarthia
3. Acute complication: dysphagia and aspirations

Question 18

What does fasciculations mean? What is this d/t?

Answer 18

Muscle twitching d/t inadequate stimulation

Question 19

What does dysarthia mean?

Answer 19

Impaired speech

Question 20

The dysphagia and aspirations (an acute complication of ALS) occurs why?

Answer 20

Inadquate stimulation of smooth muscle in esophagus

Question 21

Are sensory fx and cognition affected in ALS?

Answer 21

No. Cognition and sensory function are still intact.

Question 22

What is ALS Dx on? (2)

Answer 22

1. Clinical Presentation

2. Electromyography

Question 23

What does electromyography measure? How does this work?

Answer 23

It measures nerve function.

Test is done through stimulating a muscle THROUGH a nerve, if muscle doesn’t work then you know it is a nerve problem.

Question 24

What is the Tx for ALS? (2)

Answer 24

1. The Tx is mostly supportive

2. Pharmacologic

Question 25

What does the supportive treatment for ALS include? (2)

Answer 25

1. Helping with swallowing (PEG tube)

2. Ensuring vaccinations to limit respiratory infections

Question 26

The pharmacologic Tx for ALS involves what drug? What does this drug do?

Answer 26

Riluzole. Protects the neurons, can extend an individuals life by a few months.