Neurology: ALS

Question 1

What does ALS stand for?

Answer 1

(Amyotrophic Lateral Sclerosis)

Question 2

What is ALS?

Answer 2

• incurable, progressive neurodegenerative disease
• affects nerve cells in the brain and the spinal cord
• caused by loss of signalling in upper/lower motor neurons (stimulating muscle)

Question 3

Progressively, brain loses connection with muscles. ALS takes away the ability to…

Answer 3

Walk
Dress
Write
Speak
Swallow
Breath
 shortening the lifespan

Question 4

How common is ALS?

Answer 4

1 in 25.000

Question 5

In what time frame do most people die after diagnosis? Bc of what?

Answer 5

• Most die within 2-5 years of being diagnosed (respiratory failure)

Question 6

What are ALS symptoms?

Answer 6

Early symptoms: muscle weakness, stiffness

Afterwards:
progression of weakness,
wasting,
abnormal fatigue,
dropping things/tripping,
slurred speech,
muscle cramps + twitches,
paralysis of muscle of limbs, trunk, as well as those controlling vital functions (speech, swallowing, breathing)

Sometimes uncontrollable periods of laughter/crying

Question 7

List survival time with survival rate

Answer 7

Average survival time:
 3 years
- 20% 5 years
- 10% 10 years
- 5% 20 years or longer

Question 8

What is the cause of ALS?

Answer 8

• 90% occurs without family history (=sporadic) , unkown
• 10% occurs through a mutation (= familial)  50% chance each offspring inherits the gene
• Increased amounts of the neurotransmitter glutamate + autoimmune components implicated with ALS

Question 9

Risk groups ALS?

Answer 9

• Between 40-70 (av 55), however cases in 20s-30s also occur
• More men than women
• Military veterans 2x as likely

Question 10

What is often needed in final stages of ALS?

Answer 10

 Permanent ventilatory support to assist with breathing

Question 11

ALS only attacks…… all of senses are remained. Muscles of .. and .. are generally not affected

Answer 11

motor neurons
eyes
bladder

Question 12

Diagnosis ALS?

Answer 12

• Combination of tests to rule out other causes
• Tests centre on looking at your muscles + nerves, testing their capability to conduct and receive electrical signals
• Tests could be electromyogram, nerve conduction velocity test, muscle biopsy

Question 13

Treatment ALS?

Answer 13

There is no cure yet!
Two medications that slow progression of ALS and extend life of those diagnosed:

1. Riluzole (rilutek)
   Lowers the amount of glutamate
2. Edaravone (radicava)
   Antioxidant, can prevent damage to nerve cells from free radicals
   (unclear how it works to slow physical progression of patients with ALS)

Other treatments are more symptomatic (speech/physical therapy

Question 14

What is the US name for ALS?

Answer 14

• Lou gehrig’s disease

Question 15

What is the difference in origin and function of upper and lower motor neurons

Answer 15

• Upper motor neurons extend from the cerebral cortex or brain stem, carry motor information down to the spinal cord
• Lower motor neurons extend from the spinal cord or brain stem to skeletal muscle, to cause movement