Neuromuscular and nervous systems clinical application- Amyotrophic Lateral Sclerosis (ALS)

Question 1

what kind of disease is ALS?

Answer 1

a chronic, degenerative disease producing both upper and motor neuron symptoms

Question 2

ALS produces both __ and __ motor neuron symptoms

Answer 2

upper and lower

Question 3

what impairments in what areas of the brain specifically cause the symptoms of ALS?

Answer 3

demyelination, axonal swelling and atrophy within the cerebral cortex, premotor areas, sensory cortex and temporal cortex

Question 4

__, __ __ and __ within within the cerebral cortex, premotor areas, sensory cortex and temporal cortex specifically cause the symptoms of ALS

Answer 4

demyelination, axonal swelling and atrophy

Question 5

demyelination, axonal swelling and atrophy of the __ __, __ __, __ __ and __ __ cause the symptoms of ALS

Answer 5

cerebral cortex, premotor areas, sensory cortex and temporal cortex

Question 6

where does rapid degeneration and demyelination associated with ALS pathology occur within the brain?

Answer 6

the giant pyramidal cells of the cerebral cortex

Question 7

rapid degeneration and demyelination in the giant pyramidal cells of the cerebral cortex affects what other areas (4) of the brain?

Answer 7

corticospinal tracts
cell bodies of the LMN in gray matter
anterior horn cells
areas within the precentral gyrus of the cortex

Question 8

rapid degeneration and demyelination of the giant pyramidal cells of the cerebral cortex affect areas of the corticospinal tracts, cell bodies of the LMN in gray matter, anterior horn cells and area in the precentral gyrus of the cortex causes what (3)?

Answer 8

denervation of muscle fibers
muscle weakness
muscle atrophy

Question 9

is there an exact known etiology of ALS?

Answer 9

no

Question 10

what are the hypothesized causitive of ALS?

Answer 10

genetic inheritance (autosomal dominant)
slow acting virus
metabolic disturbances
lead/aluminum toxicty

Question 11

ALS is inherited as an autosomal __ trait

Answer 11

dominant

Question 12

risk for ALS is higher in men or women and usually occurs between __-__ years of age

Answer 12

men; 40-70

Question 13

early clinical presentation/signs and symptoms (8) of ALS?

Answer 13

upper and lower motor neuron symptoms:
LMN-
asymmetrical muscle weakness
fasciculations
cramping
atrophy of the hands
UMN-
incoordination of movement
spasticity
clonus
positive Babinski reflex

Question 14

bulbar involvement in ALS is characterized as what symptoms (3)?

Answer 14

dysarthria
dysphagia
emotional lability

Question 15

muscle weakness spreads throughout the body over the course of ALS disease and typically follows a __ to __ pattern

Answer 15

distal to proximal

Question 16

initially, a person with ALS may present with either __ or __ motor neuron symptoms, but overtime, both categories are affected

Answer 16

upper or lower

Question 17

as the disease course of ALS progresses, a patient will eventually present with what signs/symptoms (5)?

Answer 17

oral motor impairment
fasciculations
spasticity
motor paralysis
eventual respiratory paralysis

Question 18

what lab/imaging techniques (3) are used to assist with diagnosing ALS?

Answer 18

electromyography
muscle biopsy
spinal tap

Question 19

what does electromyography assess (2), therefore assisting with the diagnosis of ALS?

Answer 19

fibrillations and muscle fasciculations

Question 20

what does muscle biopsy assess/verify, therefore assisting with the diagnosis of ALS?

Answer 20

LMN involvement rather than muscle disease

Question 21

what does spinal tap assess/verify, therefore assisting with the diagnosis of ALS?

Answer 21

higher protein content

Question 22

a CT scan will appear normal until __ in the ALS disease process

Answer 22

late

Question 23

diagnosis of ALS relies heavily on symptoms that determine both __ and __ __ __ involvemen

Answer 23

upper and lower motor neuron

Question 24

a patient that presents with __ impairment without __ impairment is a primary indicator of ALS

Answer 24

motor w/o sensory

Question 25

definitive diagnosis of ALS first requires a physician to rule out what other conditions?

Answer 25

MS spinal cord tumor progressive muscular dystrophy Lyme disease syringomyelia

Question 26

what subjective information (8) is important to document when examining a patient with ALS?

Answer 26

PMH family history history of current symptoms current health status social history/habits occupation living environment social support system

Question 27

what objective tests and measures (14) are important to perform when assessing a patient with ALS?

Answer 27

aerobic capacity/endurance: vital signs at rest/ with activity; RPE anthropometrics: weight and height A&O posture (resting/dynamic) A/PROM muscle strength/endurance muscle tone, atrophy reflex integrity: DTR, pathological (Babinski ATNR) motor function: motor assessment scales, coordination, equilibrium, righting reactions reflex movement patterns, involuntary mvmt assessment, sensory integration tests, gross and fine motor skills gait, locomotion, balance: static/dynamic balance in sit/stand, safety during gait w/ w/o AD AD/adaptive devices Barthel index environmental/home/work current/anticipated barriers/hazards ventillation, respiration, circulation: respiratory muscle strength, accessory muscle utilization, assessment of cough

Question 29

progression of ALS allows for significant deterioration within the brain and spinal cord and a patient may exhibit what s/s (5)?

Answer 29

paralysis of vocal cords swallowing impairment contractures decubiti (pressure injury?) breathing difficulty requiring ventilatory support

Question 30

what bodily processes (3) are preserved throughout the course of ALS disease?

Answer 30

sensation eye movement bowel and bladder function

Question 31

effective management of ALS is based on what two factors?

Answer 31

supportive care symptomatic therapy

Question 32

pharmacological management of ALS may include what?

Answer 32

riluzole (Rilutek)

Question 33

what are the effects of riluzole (Rilutek)?

Answer 33

appears to have an effect of the progression of the ALS disease process, however its longterm effects are unknown

Question 34

what might symptomatic therapy for ALS include (3)?

Answer 34

anticholinergics antispasticity meds antidepressant meds

Question 35

what therapies (5) may be warranted for a patient with ALS and what should these therapies be focused on (2)?

Answer 35

PT, SLP, OT, nutrition, respiratory therapies quality of life and caregiver training

Question 36

what should physical therapy for a patient with ALS generally include?

Answer 36

low-level exercise program ROM mobility training assistive/adaptive devices wheelchair prescription bronchial hygiene energy conservation techniques pt/family/caregiver assistance training

Question 37

home exercise programs for patients with ALS must consider what two important factors?

Answer 37

rate of disease progression level of respiratory involvement

Question 38

the general goal of home exercise programs for patients with ALS is maximizing what?

Answer 38

functional capacity

Question 39

a low-level exercise program is indicated as part of an HEP for a patient with ALS as long as the patient does exercise to __ and promote further __

Answer 39

fatigue, weakness

Question 40

family involvement is encouraged to assist patient with ALS with HEP activities like (4)?

Answer 40

mobility pacing skills energy conservation techniques safety

Question 41

during the later stages of the ALS disease course, family/caregivers must be compentent in what skills (4)?

Answer 41

positioning ROM bronchial hygiene assistance with mobility

Question 42

what is the likely outcome of physical therapy in terms of the disease course of ALS?

Answer 42

may assist with current issues, but it does not alter the progression of ALS

Question 43

the average course of ALS disease is __-__ years

Answer 43

2-5

Question 44

the average course of ALS disease is 2-5 years, with roughly __% of patients surviving longer than 5 years

Answer 44

25

Question 45

Research indicates that although there is no structured course of the ALS disease process, if a patient is diagnosed before the age of __, the disease course is usually longer

Answer 45

50

Question 46

Research indicates that although there is no structured course of the ALS disease process, if a patient is diagnosed before the age of 50, the disease course is usually ___

Answer 46

longer

Question 47

death in a patient with ALS typically occurs due to what?

Answer 47

respiratory failure

Question 48

what condition is a common differential diagnosis of ALS?

Answer 48

muscular dystrophy

Question 49

what is muscular dystrophy?

Answer 49

a group of inherited disorders that are progressive and exhibit degeneration of the muscles without sensory or neural impairment

Question 50

muscular dystrophy is a group of __ disorders that are progressive and exhibit degeneration of the __ without __ or __ impairment

Answer 50

inherited; muscles; sensory; neural

Question 51

what is the pathology of muscular dystrophy?

Answer 51

progressive weakness occurs to the muscle fibers secondary to absence of dystrophin within skeletal muscles

Question 52

the pathology of muscular dystrophy includes progressive weakness that occurs to the muscle fibers in the absence of __ within __ muscles

Answer 52

dystrophin, skeletal

Question 53

muscular dystrophy presents __ in life and usually __ life expectancy

Answer 53

early; shortens

Question 54

what are the common characteristics (5) of the muscular dystrophy disease process?

Answer 54

disuse atrophy muscle deterioration contractures cardiac weakness respiratory weakness

Question 55

what does a patient with muscular dystrophy typically die from?

Answer 55

respiratory or cardiac complications secondary to the primary disease process