ALS Flashcards
1
Q
What is ALS
A
- rapidly progressive neurodegenerative disease of middle life that is rarely seen prior to age 40
2
Q
What does ALS cause?
A
- Disease of UMN and LMNs.
- Loss of anterior horn cells in SC and the motor cranial nuclei in brainstem (LMN)
- Demyelination and gliosis of corticospinal and corticobulbar tracts in motor cortex (UMN)
3
Q
Major signs - LMN
A
Muscle weakness, atrophy, fasciculations, hyporeflexia, muscle cramps
4
Q
Major signs - UMN
A
- spasticity, hyper-reflexia, pathological reflexes
5
Q
Major signs - Bulbar signs
A
- dysarthria, dysphagia
6
Q
Major signs - Respiratory signs
A
Nocturnal respiratory difficulty, exertional dyspnea, accessory muscle use, paradoxical breathing
7
Q
Major signs - Others
A
- Fatigue, weight loss, cachexia, tendon shortening, contractures
- Frozen shoulder (30% of persons with ALS)
8
Q
Most frequent initial symptom
A
- focal weakness beginning in the leg, arm, or bulbar muscles * No sensory symptoms initially
- Cognition, extraocular eye movements and autonomic, bowel, bladder, and sexual functions usually remain intact initially
9
Q
ALS prognosis
A
- Death usually results from respiratory failure with 50% of
patients surviving 3-4 years after the onset of symptoms unless mechanical ventilation is used - Most die within 5 years
10
Q
ALS Medications
A
- Riluzole, is expensive and adds 2-3 months to a person’s life. Affects breathing function.
- Radicava: unknown mechanism. Possibly anti-oxidant. Slowed decline in function in RCT in Japan
11
Q
Examination/Evaluation
A
- MMT/Dynamometry (be sensitive)
- Coordination
- Tone
- Balance
- Fatigue
- Psychosocial
- ALSFRS (self grading ADLS)
- ALSAG-40 and ALSAQ-5: well being, multiple domains
12
Q
Consider the Multi-System Nature of the Disease during Examination
A
- Autonomic Function
- Sensory Function
- Screen for cognitive impairments
- Up to 13% can have fronto-temporal dementia; up to 50% with cognitive decline
- Represents poorer prognosis
- Screen for extrapyramidal signs
13
Q
Symptoms of ALS
A
- fatigue
- muscle stiffness
- muscle cramps
- shortness of breath
- sleep, pain, anxiety, depression, increased saliva, constipation
- falls increasingly common with advancing disease
14
Q
treating fatigue
A
energy conservation
15
Q
f
A
16
Q
treating muscle stiffness and cramps
A
stretching
17
Q
treating pain
A
- depends on source of pain
- cramps: massage, stretch
- skin pressure: positioning
- Msk: P-AAROM and ex
18
Q
Treating falls
A
- ADs, Orthotics, balance training
19
Q
what does highly repetitive or heavy resistance exercise cause?
A
prolonged loss of strength in weakened or denervated muscle
20
Q
what can excessive exercise cause?
A
- impaired recovery of degenerated muscle fiber
21
Q
Pulmonary techniques
A
- Inspiratory muscle training prolongs respiratory strength for FVC
- mean length of survival increases
- Lung volume recruitment training strongly enhances immediate cough efficacy with improved FVC
22
Q
overall considerations for exercise
A
- ROM and stretching are accepted forms of exercise
- Resistance of unaffected muscles and possibly muscles ≥ 3/5 using low to moderate load and intensity and aerobic ex at sub- maximal levels (50-65% of HRR) may be safe and effective
- Aerobic and resistance ex more appropriate for early-mid stage and in those with slowly progressing disease.
- Monitoring exercise load and tolerance is critical
- Avoiding excessive fatigue and overwork damage
- Instruct pts to not carry out activities to extreme fatigue
23
Q
Safety Considerations for overuse
A
- Inability to perform daily activities following exercise due to exhaustion or pain
- Reduction in maximal muscle force that gradually recovers
- Increased or excessive cramping, soreness, fatigue or
fasciculations - Keep an exercise log
- Stop if overuse signs occur
24
Q
Stage I of ALS
A
- Independent in ADLs. Ambulatory but clumsy. Specific group of muscles are mildly weak. Mostly doing education, energy conservation, home/workplace modification and psych support
- Mild focal weakness, asymmetrical distribution, hand cramping possibly
- Continue normal activities
- Resistive strengthening for unaffected muscles;
- Submaximal levels of activity otherwise.
25
Stage II of ALS
* Moderate weakness, still selective; slightly decreased independence
now – climbing stairs, raising arms, buttoning etc.
* May start to see some muscle wasting * Time to assess need for adaptive equipment, assistive devices, watch
for falling.
* Stretching and AROM exercise, strengthening of unaffected muscles,
* Aerobic activities as he or she is able.
* AAROM and PROM by caregivers/family of affected joints to minimize contracture formation
26
Stage II ALS exercise considerations
* Prevention of overuse fatigue and disuse atrophy.
* Brief exercise several times throughout day with good time for rest.
* 30-45 minutes of total exercise divided into 2-3 sessions.
* Resistance, aerobic, active ex.
* Active/resistive exercise may --> repetitive performance of functional activities
27
Stage III (middle)
* Ambulatory but with severe weakness in certain muscle groups: distal ankle and
hand; mild to moderate limitation in function (should still be ambulatory)
* Goal: keep physically independent.
* Adaptive equipment
* Begin respiratory therapy (deep breathing exercise)
28
Adaptive Equipment for Stage III
- AFO, powered chairs, splints for hands more typical
wheelchair use for longer distances.
- Possible head control difficulty with soft collar use indicated.
* If disease is progressing rapidly, go with generic rather than custom equipment (save
the patient $$)
* Lightweight canes, rolling walker only
* WC prescription (insurance probably only pays for 1 motorized device/wc)
29
Stage IV (middle)
* Severe weakness overall but with less weakness in UE. Wheelchair use. May be able to perform some ADLs. Likely see muscle wasting in LEs
* PROM and AAROM exercises to prevent contractures.
* Strengthening of any uninvolved muscles.
* Education for skin inspection as a result of loss of functional mobility.
* Sleep systems for pressure relief are indicated
30
Stage V (Late)
* Increasing and progressive weakness throughout.
* Deteriorating mobility and endurance.
* Constant wheelchair use.
* Transfers require significant physical assist.
* Decreased bed mobility requires additional assistance and repositioning to prevent pressure ulcers.
* Pain may be an issue in immobilized joints. But pain may be due to spasticity, cramps too.
* Likely significant head control issues exist. Head support is needed in order to eat, see, and breathe.
31
Stage VI (late)
* Confined to bed and requires maximal assistance with ADLs.
* Proper postural support is needed in bed.
* Frequent repositioning.
* Pain management.
* Progressive respiratory distress.
32
Goal of Stage VI
* Preserve vital functions through compensation
* Prevent pressure sores, respiratory problems
