ALS

Question 1

What is ALS

Answer 1

A fatal, progressive neurodegenerative disorder

Question 2

What does ALS stand for and broken up meaning

Answer 2

Muscle atrophy

Degeneration and eventual death of motor neurons in the cortex, brainstem, anterior horn cells and pyramidal tracts

Question 3

Two forms

Answer 3

Familial

sporadic (90%)

Question 4

Risk factors for sporadic ALS

Answer 4

Smoking
Exposure to certain metals
Electrical work
Military 
Environment*
Head trauma*
Excessive physical activity*
Genetics*

Question 5

Pathophys of ALS

Answer 5

Progressive deterioration of motor nerve cells in brain and spinal cord

Question 6

What’s the protein found misfolded in the cytoplasm

Answer 6

TDP 43

Question 7

Links ALS to FTD

Answer 7

TDP 43

Question 8

General clinical manifestations of ALS

Answer 8

UMN
LMN
Limb and spine
Bulbar
Pseudobulbar
Respiratory

Question 9

UMN

Answer 9

Spasticity
HyPER reflexia and clonus
Pseudobulbar effect

Question 10

LMN

Answer 10

Muscle weak and atrophy
Fasciculations
Cramps
HYPOreflexia

Question 11

Limb and spine

Answer 11

Asymmetric limb weak most common

Question 12

What’s affected first in limb issues

Answer 12

Finger extensors and ankle DF affected first

Question 13

Cervical

Answer 13

Weak extensors- head flop

Question 14

Bulbar from brainstem

Answer 14

Dysphagia
Dysarthria
Sialorrhea

Question 15

Pseudobulbar

Answer 15

Inappropriate crying and laughter

Question 16

Respiratory issues (6)

Answer 16

DOE or with lying flat
Weak cough
Use aux muscles more
Daytime sleepy
Less concentration
Headaches

Question 17

Signs and Sx of FTD (5)

Answer 17

Emotional blunting
Loss of insight
Decreased social conduct
Personality changes 
Decreased word generation

Question 18

ALS does NOT affect? (5)

Answer 18

Bowel and bladder
Internal organs
Sexual function
Eye muscles 
Sensation

Question 19

One of the most common Sx with ALS

Answer 19

Fatigue

Question 20

Weakness with specific patterns

Answer 20

Doesn’t improve or get worse with exercise
Distal limbs affected first
Spine extensors most affected

Question 21

Presence of both

Answer 21

UMN and LMN Sx

Question 22

3 other common themes in ppl with ALS

Answer 22

Fatigue
Difficulty with lying flat
Personality changes

Question 23

Cranial nerves involved

Answer 23

PBP = progressive bulbar palsy

Question 24

UMN

Answer 24

Primary lateral sclerosis (PLS)

Question 25

LMN

Answer 25

Progressive muscle atrophy (PMA)

Question 26

Which one?

Pt complains of fatigue, muscle weak and cramping

Answer 26

PMA b/c theyre LMN

Question 27

Which one?

Pt complains of fatigue, stiffness and crying inappropriately

Answer 27

PLS b/c UMN

Question 28

Which one?

Pt complains of fatigue, weak, stiff and fasciculations

Answer 28

ALS b/c both UMN and LMN

Question 29

3 meds as treatment

Answer 29

No cure but
Riluzole
Nuedexta
Radicava

Question 30

Can increase survival by 2-3 months

Answer 30

Riluzole

Question 31

May help bulbar Sx med

Answer 31

Nuedexta

Question 32

New treatment
May decrease slope of decline
Very expensive
No long term results

Answer 32

Radicava

Question 33

Exercise guidelines

Answer 33

Avoid eccentric contraction

Avoid high reps and intensity

Question 34

Important exercise for ALS

Answer 34

STRETCH

30 sec, 2-4 x/day

Question 35

Managing respiratory issues

Answer 35

NIPPV (biPAP)

NOT OXYGEN

Question 36

Psychosocial factors of ALS

Answer 36

Depression
Insomnia
Difficult decisions