ALS

Question 1

What is the most common adult onset MND?

Answer 1

motor neuron disease is ALS

Question 2

What does ALS stand for?

Answer 2

Amyotrophic Lateral sclerosis

Question 3

How is ALS characterized?

Answer 3

progressive
fatal
paralytic muscle wasting

Question 4

What nuero system is involved in ALS?

Answer 4

UMN & LMN

Question 5

What is age onset of ALS? and average onset

Answer 5

teens to 80’s average 50’s

Question 6

What are the two types of ALS? and %?

Answer 6

SALS (sporadic) 90-95%

FALS (familial) 10-5%

Question 7

What are the two onsets of ALS? and %?

Answer 7

limb onset 70-80%

bulbar onset 20-30%

Question 8

What are limb and bulbar onset ALS?

Answer 8

limb onset: initial onset in the extremities and UMN>LMN

bulbar onset: initial involvement of bulbar muscles ie muscles of face and throat and LMN>UMN

Question 9

Which onset if more detrimental?

Answer 9

bulbar affecting breathing

Question 10

ALS male or female more common?

Answer 10

male ALS

Question 11

What are some possible reasons for ALS?

Answer 11

oxidative stress
neurotoxicity (mercury, lead)
protein aggregation
lifestyle factors

Question 12

What are common risk factors for ALS?

Answer 12

mutation: SOD1
Cluster of demographic: western Pacific male
Gender: male>female
Age
Family hx

Question 13

Disease course of ALS?

Answer 13

most die within 3-5 years due to respiratory failure

Question 14

What is Pseudobulbar Palsy? S&S?

Answer 14

Corticobulbar tract affected
Speech, mastication, swallow
Pseudobulbar effect

Question 15

What is Pseudobulbar effect?

Answer 15

sudden crying and laughing

Question 16

What is Progressive Bulbar Palsy? S&S?

Answer 16

CN affected
Swallow, mastication, facial muscles
Fasciculation of tongue PROMINENT

Question 17

What is PLS? S&S?

Answer 17

Primary lateral Sclerosis
Corticospinal tract, neuron loss in cortex
ONLY UMN affected: hyperreflexia/ spasticity
no fasciculation or atrophy

Question 18

What is PMA? S&S?

Answer 18

Progressive Muscular Atrophy
loss of motor neurons in Anterior horn cells
ONLY LMN
progressive weakness, wasting, & fasciculation of hand muscles

Question 19

MIX of what two forms results in the term ALS?

Answer 19

PLS & PMA= ALS

Question 20

What are the best indicator to survival in ALS?

Answer 20

age at time of onset,
<35 y.o has best survival rate than older pt
and
limb-onset: they have better outcome than bulbar

Question 21

What are some UMN signs?

Answer 21

spastic
hyperreflexia
clonus
Babinski
muscle weakness

Question 22

What are some LMN signs?

Answer 22

weakness
focal
asymmetrical
begins in UE, LE, or Bulbar muscles
Begins distally in extremities
hyporeflexia, hypotonia, fatigue

Question 23

What is a typical weakness in LMN ALS?

Answer 23

C/S extensors

Question 24

What are some Bulbar signs?

Answer 24

Dysarthria
Dyphagia
Sialorrhea
Pseudobulbar effect
respiratory dysfunction

Question 25

What is Dysarthria?

Answer 25

hoarseness or slurring

Question 26

What is Sialorrhea?

Answer 26

drooling

Question 27

What are common cognitive S&S in ALS?

Answer 27

frontotemporal dementia

behavior changes

Question 28

What medication is for ALS?

Answer 28

Rilotek: extends survival by 2-3 months

Question 29

What are some rehab considerations for ALS?

Answer 29

strengthening involved muscles won’t improve
do not overtrain/fatigue
rehab: flexibility and aerobic