ALS

Question 1

what is ALS

Answer 1

Amyotrophic - weakness and atrophy
Lateral - affects motor neurons in lateral spinal cord
Sclerosis - hardening or scarring in CNS

Question 2

what are 2 other names for ALS

Answer 2

• Lou Gehrig’s disease
• motor neuron disease

Question 3

is ALS an upper or lower motor neuron disease

Answer 3

both

Question 4

T or F: ALS is progressive

Answer 4

T

Question 5

mean age of onset for ALS

Answer 5

55-65

Question 6

is ALS more common in males or females

Answer 6

males

Question 7

what structures does ALS affect

Answer 7

UMN - corticospinal and corticobulbar
LMN - anterior horn cells and cranial nerves

Question 8

T or F: sensation is affected in ALS

Answer 8

F: sensation is normal

Question 9

2 onset patterns for ALD… which is more common

Answer 9

corticospinal - limb
corticobulbar - bulbar

limb onset is more common

Question 10

first symptoms in those with corticospinal onset ALS

Answer 10

• difficulty with manual dexterity
• awkwardness with ambulation

Question 11

first symptoms in those with corticobulbar onset ALS

Answer 11

• difficulty speaking, swallowing and tongue mobility
• nasality and volume loss

Question 12

T or F: regardless of which onset of ALS eventually there will be complete muscle paralysis

Answer 12

T

Question 13

does paralysis in ALS start proximal or distal

Answer 13

distal

Question 14

what happens to the hands in patients with ALS

Answer 14

they have “pancake” hand because they lose the arches from muscle loss

Question 15

T or F: the etiology of ALS is unknown

Answer 15

T

Question 16

what are some things that are associated with increased risk of ALS

Answer 16

• smoking
• diet
• military vets
• varsity/pro athletes
• repeated head trauma
• exposure to viruses, heavy metals, and toxic chemicals

Question 17

bulbar signs

Answer 17

• dysarthria
• dysphagia
• sialorrhea (difficulty with drool management)
• pseudobulbar effect (affect disorder)

Question 18

respiratory S/S of ALS

Answer 18

• nocturnal respiratory difficulty
• exertional dyspnea
• accessory muscle use
• paradoxical breathing

Question 19

people with ALS show UMN, LMN, bulbar, and respiratory s/s. what are some other general s/s?

Answer 19

• fatigue
• weight loss
• cachexia
• tendon shortening
• joint contractures

Question 20

are flexors or extensors generally more weak in ALS

Answer 20

flexors

Question 21

T or F: the progression of ALS is symmetrical

Answer 21

F: asymmetrical pattern and sparing

Question 22

T or F: pts with ALS lose 80% of motor neurons in an affected area before seeking help

Answer 22

T: it is already progressed by the time they get help

Question 23

why do pts with ALS have very expressive eyes

Answer 23

• because the oculomotor nerve is spared
• the face musculature degenerates but the eyes can still move

Question 24

what are 3 systems that remain normal in ALS

Answer 24

• cognition (mostly)
• sensation
• B/B

Question 25

T or F: 30-50% of those with ALS develop some cognitive impairment

Answer 25

T - frontotemporal dementia

Question 26

possible cognitive symptoms in ALS

Answer 26

- changes in behavior - expressive language difficulty - preserved comprehension - executive dysfunction

Question 27

what is the diagnostic criteria for ALS

Answer 27

1 - concurrent UMN & LMN signs in 3 spinal regions or 2 spinal regions and bulbar signs 2 - progression of signs and symptoms

Question 28

what are some exclusionary diagnostic criteria for ALS (5)

Answer 28

-sensory and autonomic dysfunction -sphincter control problems -oculomotor abnormalities -movement/coordination disorder -cognitive deterioration (sometimes)

Question 29

what are some diagnostic tests for ALS

Answer 29

-creatine phosphokinase levels -glutamate in CSF (spinal tap) -EMG -nerve conduction study (NCS) -genetic testing -MRI -muscle biopsy

Question 30

which would be normal in ALS: EMG or NCS?

Answer 30

NCS

Question 31

what is the life expectancy of ALS

Answer 31

2-5 years from diagnosis

Question 32

what is death often related to in ALS

Answer 32

respiratory failure

Question 33

what are some negative prognostic factors in ALS

Answer 33

- bulbar onset - female - age>60 - time of diagnosis

Question 34

T or F: there is a cure for ALS

Answer 34

F: there are FDA approved drugs that could slow progression but they have high hepatotoxicity

Question 35

less than ____ secs on SLS in either LE increases the risk of falls

Answer 35

10

Question 36

greater than ____ secs on 10 meter walk test increases risk of falling

Answer 36

10-15

Question 37

what does the braden scale measure

Answer 37

risk of developing pressure sore

Question 38

T or F: increasing strength is a good goal for pts with ALS

Answer 38

F: never write a strength goal for someone with ALS

Question 39

palliative care vs hospice care

Answer 39

- palliative = symptom management of a chronic or life-limiting illness - hospice care = comfort care at the end of life

Question 40

T or F: palliative care begins at diagnosis for those with ALS

Answer 40

T

Question 41

what kind of wheelchair should you always get for someone with ALS and why

Answer 41

power because insurance only pays for a w/c every 5-7 years

Question 42

should you have tilt or recline features on a WC for pts with ALS

Answer 42

both! - recline to decrease hip flexion contracture and tilt for pressure relief

Question 43

what are some things to think about with WC for pts with ALS

Answer 43

controls - need to be able to change them as disease progresses - ventilation support - do they want to be on a vent? - seat elevation - hard to get approved - transportation

Question 44

T or F: therex is important for pts with ASL

Answer 44

T: pts scored higher on ALS functional rating scale if they did resistance training with aerobics and stretching than stretching and gentle aerobics alone

Question 45

what kind of exercise for pts with ALS

Answer 45

- low impact - 15-20 reps - concentric or isometric - avoid next day soreness - concentrate on muscles 3/5 or greater

Question 46

3 main phases of ALS

Answer 46

1 - independent 2 - partially independent 3 - dependent

Question 47

in the independent phase of ALS what are some interventions

Answer 47

- begin ROM exercise - gentle strengthening - monitor for overwork - adaptive equipment as needed for ADLs - prescribe wheelchair - psychological support

Question 48

in the partially independent phase of ALS what are some interventions

Answer 48

- heat/massage to control pain - preventative anti-edema measures - continue to adapt WC - pt ed for transfers, positioning - home modifications

Question 49

in the dependent phase of ALs what are some interventions

Answer 49

- modify diet for dysphagia - cough assist for breathing difficulty