Amyotrophic Lateral Sclerosis ALS

Question 1

What is Amyotrophic Lateral Sclerosis?

Answer 1

Lou Gehrig disease

Progressive / fatal

Motor neuron disease; scars form on upper motor neurons in corticospinal pathways

Upper and Lower motor neurons also affected

Inflammatory component in the process

Question 2

What are the types of ALS?

Answer 2

Familial: 10% of cases, genetic mutation, family history

Sporadic: most cases, speculation viral, retroviral, environmental causes, genetics

Question 3

What are some environmental causes of ALS?

Answer 3

Exposure to chemicals / lead

electromagnetic fields

Question 4

What is the population most affected by ALS?

Answer 4

white males older than 60 yrs

Question 5

Which sex is more affected by sporadic ALS? Familial ALS?

Answer 5

Sporadic ALS affects more males vs females

Familial ALS affects male / female relatively same

Question 6

Signs and symptoms of ALS

Answer 6

Weakness of FM muscles of the hand / asymmetrical foot drop (most common)

Night cramps (calf muscles)

Spasticity

Loss of emotional control

Difficulty speaking / swallowing

Reduced body temp regulation

Reduced executive functions

Respiratory failure

Distal to proximal progression

Hyperresponsive reflexes

Distorted speech

Question 7

Upper motor neuron (UMN) signs of ALS

Answer 7

spasticity

hyperreflexia

pathological reflexes

Question 8

Lower motor neuron (LMN) signs of ALS

Answer 8

muscle weakness

muscle atrophy

fasciculations

hypotonicity

muscle cramps

Question 9

Bulbar signs of ALS

Answer 9

dysarthria

dysphagia

sialorrhea (hypersalivation)

pseudobulbar palsy

Question 10

Respiratory symptoms of ALS

Answer 10

nocturnal respiratory difficulty

exertional dyspnea

accessory muscle use

paradoxical breathing

Question 11

King’s ALS clinical staging, stage 0

Answer 11

presymptomatic

Question 12

King’s ALS clinical staging, stage 1

Answer 12

involvement of 1 clinical region (disease onset)

Question 13

King’s ALS clinical staging, stage 2

Answer 13

involvement of 2 clinical regions

Question 14

King’s ALS clinical staging, stage 3

Answer 14

involvement of 3 clinical regions

Question 15

King’s ALS clinical staging, stage 4

Answer 15

substantial respiratory or nutritional failure

Question 16

King’s ALS clinical staging, stage 5

Answer 16

death

Question 17

MITOS ALS functional staging, stage 0

Answer 17

functional involvement (disease onste)

Question 18

MITOS ALS functional staging, stage 1

Answer 18

loss of independence in one functional domain

Question 19

MITOS ALS functional staging, stage 2

Answer 19

loss of independence in 2 functional domains

Question 20

MITOS ALS functional staging, stage 3

Answer 20

loss of independence in 3 functional domains

Question 21

MITOS ALS functional staging, stage 4

Answer 21

loss of independence in 4 functional domains

Question 22

MITOS ALS functional staging, stage 5

Answer 22

death

Question 23

Typical age of onset of ALS

Answer 23

between 16 and 77 yrs

Question 24

Average diagnosis age

Answer 24

55 – 77 yrs

Question 25

What is the sporadic peak age of onset?

Answer 25

58-63 yrs

Question 26

What is the familial peak age of onset?

Answer 26

47-52 yrs

Question 27

What is the survival rate after diagnosis?

Answer 27

1 to 5 yrs survival

Question 28

What is death in those with ALS often caused by?

Answer 28

respiratory failure / pneumonia

Question 29

What can ALS be medically/surgically managed?

Answer 29

Riluzole: only medication approved to treat; Extends life ~3 months; Can ease respiratory symptoms

Antispasmodic medications

Nonsteroidal anti-inflammatory medications

Gastrostomy

Noninvasive positive-pressure ventilation

Nebulizers