ALS

Question 1

what is the etiology of ALS?

Answer 1

not well understood

Question 2

The pathology of ALS may be located where? (5)

Answer 2

1. motor cortex
2. corticobulbar pathway
3. cranial nerve nuclei (brainstem)
4. SC
5. anterior horn cells

Question 3

define the condition associated with ALS in the corticobulbar pathway

Answer 3

pseudobulbar palsy - UMN PATHOLOGY - spasticity and poor control over face/mouth/throat muscles as well as emotional lability

Question 4

define the condition associated with ALS in the cranial nerve nuclei (brainstem)

Answer 4

progressive bulbar palsy - LMN PATHOLOGY - flaccid, atrophied face/mouth/throat muscles without change in affect

Question 5

Re-innervation can compensate for progressive degeneration until motor unit loss is ~__%

Answer 5

50%

Question 6

Where can degeneration occur in Primary Lateral Sclerosis?

Answer 6

cortex and CST (UMN pathology)

Question 7

which is more aggressive, ALS or PLS?

Answer 7

ALS

Question 8

where can degeneration occur in progressive bulbar palsy

Answer 8

CN IX-XII (LMN pathology)

Question 9

Where do we typically see degeneration in Progressive Muscular Atrophy (aka SMA)

Answer 9

anterior horn cells (LMN) and brainstem (UMN)

Question 10

ALS or PLS: stiffness

Answer 10

PLS

Question 11

ALS or PLS: limb wasting

Answer 11

ALS

Question 12

ALS or PLS: duration

Answer 12

PLS longer duration

Question 13

ALS v PLS: mortality

Answer 13

ALS higher mortality

Question 14

how do you differentiate ALS from PLS, primarily

Answer 14

PLS = spasticity without wasting x 3 years

Question 15

Sxs of ALS depend on the pathology, but what are the five things that are typically spared?

Answer 15

• eye movement
• sensation
• bowel and bladder
• coordination
• cognition

Question 16

cardinal sign of ALS

Answer 16

weakness (beginning distally and moving proximally)

Question 17

T/F: ALS weakness is symmetrical

Answer 17

false: usually asymmetrical

Question 18

LMN signs with ALS

Answer 18

fatigue, fasciculations, hyporeflexia, decreased tone, cramping

Question 19

UMN signs with ALS

Answer 19

spasticity, hyperreflexia, pathological reflexes, and weakness

Question 20

how do you dx ALS

Answer 20

primarily EMG, location of symptoms at multiple sites

Question 21

EMG & NCV test demonstrate UMN or LMN disease?

Answer 21

EMG and NCV demonstrate LMN disease

Question 22

what is the threshold for perceived weakness in ALS patients?

Answer 22

50% motor neuron loss

Question 23

limb or bulbar onset of ALS leads to a poorer prognosis

Answer 23

bulbar onset leads to a poorer prognosis

Question 24

median survival of ALS

Answer 24

4 years

Question 25

what usually kills ALS patients

Answer 25

respiratory failure/pneumonia

Question 26

what is limb onset ALS

Answer 26

(majority of ALS) - tripping over feet or difficulty with buttoning a shirt

Question 27

what is bulbar onset ALS

Answer 27

(minority of ALS cases) - facial and hypoglossal nerve involvement causing eating/swallowing difficulty usually as a first sign

Question 28

PT is supported in the literature for ALS, how?

Answer 28

exercise protocols slow the decline of the disease (happy medium difficulty - not too easy or too hard)

Question 29

how can PT assist ALS patients other than exercise Rx

Answer 29

AD and seating system management

Question 30

ALS specific outcome measure

Answer 30

ALSFRS-R (for research)