L6 ALS

Question 1

ALS definition

Answer 1

progressive neurodegenerative disease that affects upper and lower motor neurons

eventually leads to paralysis and death

no cure for ALS, can be medically managed to reduce S/S and prolong life

Question 2

Mean survival time

Answer 2

2-5 years on average

Question 3

Epidemiology ALS

Answer 3

90-95% of cases are sporadic

prevalence of disease is 5.2 per 100,000

most people are between 40 to 70, average of 55

more common in men than women, caucasian

Question 4

Pathophysiology of ALS

Answer 4

onset of this disease is sporadic and cause is unknown

motor neurons are uniquely vulnerable to the disease process

Question 5

Pattern of loss for motor neurons

Answer 5

loss of UMNs in CST and motor cortex

loss of LMNs in brainstem and anterior horn of SC

Question 6

Pathophysiology of ALS

Answer 6

factors that contribute to motor neuron cell degeneration and/or death

-oxidative stress
-defective glutamate metabolism
-genetic variations
-cytoskeletal protein defects preventing normal cell
-autoimmune dysfunction
-inflammatory responses

Question 7

Limb-onset ALS

Answer 7

extremity and bulbar tract signs

most common, 2/3rds of patients

often starts in the limbs and then progresses to proximal tracts controlling swallowing and respiration

Question 8

Bulbar ALS

Answer 8

1/3 of patients

starts with difficulty with speech and trouble swallowing that can be followed by limb involvement in later stages

progresses more quickly than limb onset variant, shortening survival time

Question 9

ALS-plus syndrome

Answer 9

extremity and bulbar symptoms and dementia

Question 10

Medical Diagnosis of ALS

Answer 10

diagnosis of exclusion, delayed diagnosis and treatment

average time to diagnosis to 10-15 months

Question 11

Arlie House Criteria

Answer 11

presence of LMN and UMN symptoms in the bulbar region and at least 2 spinal regions

presence of LMN and UMN symptoms in 3 spinal regions

Question 12

Other characteristics suggestive of ALS

Answer 12

absence of sensory symptoms

progressive spread of symptoms or signs

no evidence of other disease processes

Question 13

Diagnoses that are against ALS

Answer 13

presence of isolated radicular pain

symmetric proximal OR distal limb weaknesses

cog wheel rigidity

prominent sensory loss

isolated fasciculations or cramps without weakness

rapid onset with no progression

Question 14

UMN Lesion Characteristics

Answer 14

Weakness
Increased Reflexes
Increased Muscle Tone

Question 15

LMN Lesion characteristics

Answer 15

Weakness
atrophy
fasciculations
decreased reflexes
decreased muscle tone

Question 16

Features of ALS associated with poor prognosis

Answer 16

-elderly onset
-elderly females presenting with bulbar-onset
-pts whose ALS diagnosis has been given less than 8 mo after symptom onset
-patients losing more than 1.4 points/mo

Question 17

Medical Treatment ALS

Answer 17

predominately symptom management

slow down the progression of disease

Question 18

Predominately symptom management

Answer 18

-respiratory
-dysphagia
-sialorrhea = excesive salivation
-pseudobulbar affect = episodes of uncontrollable laughter or crying
-communication
-muscle spasms and pain
-weakness and loss of functional mobility
-depression

Question 19

Slowing the progression of the disease

Answer 19

riluzole

thought to reduce glutamate-induced excitotoxicity

improved survival

Question 20

Patient and family education

Answer 20

-disease progression, will lead to death
-no cure
-medications can lessen symptoms
-therapy and AT can help improve QOL
-discussion of palliative progressing

Question 21

Speech Language Pathology

Answer 21

early in the disease process

see SLP to establish a baseline of speech rate

begin message and voice banking so that your own voice can be used in a speech generating device

Question 22

Alternative tech

Answer 22

thick barreled pen to improve grip strength

increasing cursor speed of a mouse so less movement is required

switch to single click on tech rather than double click

head, foot, eye movement controls

Question 23

ALS Functional Rating Scale Revised

Answer 23

measure of physical function for patients with ALS created in 1991

12 question scale with likert scale responses

total score range 0-48, with lower score is more impaired

stronger predictor of survival than muscle strength or respiratory function

Question 24

Areas that are measured in ALS functional rating scale

Answer 24

Bulbar = speech, salivation, swallowing

fine motor = handwriting, cutting food, dressing and hygiene

gross motor = turning in bed, walking, climbing stairs

respiratory = dyspnea, orthopnea, respiratory insufficiency

Question 25

Palliative Care

Answer 25

meant to improve the quality of life of patients who have a serious or life-threatening disease. approach to care that addresses the person as a whole, not just their disease. Goal is to prevent or treat as early as possible, the symptoms and side effects of the disease and its treatment in addition to any related psychosocial and spiritual problems. Anyone can receive palliative care regardless of their age or stage of disease

Question 26

Overall goal of care for ALS

Answer 26

slow the decline of loss of function and provide more support as they decline

Question 27

Short term effects of exercise in ALS

Answer 27

Improved ALSFRS-R = possibly hypertrophy, more likely due to increased recruitment by collateral innervation

No improvement in ALSFRS-R = early in the disease it is difficult to maintain high score levels of b/c of natural history, possible that these patients were under-dosed

Question 28

Clinical Bottom Line of short term effects of exercise

Answer 28

you need to use outcome measures to monitor your patient’s response to a new exercise program and adjust plan of care accordingly

Question 29

Medium Term Effects of Exercise in ALS

Answer 29

4/5 studies showed less loss of function for those that did aerobic exercise

one study showed an exception; they did both strength and aerobic exercise

Question 30

Clinical Bottom Line of Medium Term Effects of Exercise in ALS

Answer 30

Training must be balanced between muscle overwork and underwork. overwork may increase fatigue and weakness

five times/week would not be recommended for ALS patients

not enough research for intensity/frequency

Question 31

Long-Term Effects of Exercise in ALS

Answer 31

5/6 studies showed less loss of function in exercise group

there is a consistent documented benefit of exercise on function in patients with ALS

even small changes can improve QOL

Question 32

Overall Clinic Bottom Line for exercise

Answer 32

combining strength training and aerobic exercise at moderate intensity, 2/week

optimize functional benefit without increasing fatigue

must monitor for overwork fatigue

Question 33

Rehab in Reverse

Answer 33

as the disease progresses so will your interventions and goals

go from least restrictive to more supportive equipment

education from how to be independent to caregiver support

exercise to increase strength to then maintain strength for as long as possible

Question 34

Late stage ALS

Answer 34

most individuals wished they had begun sooner with wheelchair