ALS Flashcards
What is ALS
- rapidly progressive neurodegenerative disease of middle life that is rarely seen prior to age 40
What does ALS cause?
- 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)
Major signs - LMN
Muscle weakness, atrophy, fasciculations, hyporeflexia, muscle cramps
Major signs - UMN
- spasticity, hyper-reflexia, pathological reflexes
Major signs - Bulbar signs
- dysarthria, dysphagia
Major signs - Respiratory signs
Nocturnal respiratory difficulty, exertional dyspnea, accessory muscle use, paradoxical breathing
Major signs - Others
- Fatigue, weight loss, cachexia, tendon shortening, contractures
- Frozen shoulder (30% of persons with ALS)
Most frequent initial symptom
- 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
ALS prognosis
- 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
ALS Medications
- 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
Examination/Evaluation
- MMT/Dynamometry (be sensitive)
- Coordination
- Tone
- Balance
- Fatigue
- Psychosocial
- ALSFRS (self grading ADLS)
- ALSAG-40 and ALSAQ-5: well being, multiple domains
Consider the Multi-System Nature of the Disease during Examination
- 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
Symptoms of ALS
- fatigue
- muscle stiffness
- muscle cramps
- shortness of breath
- sleep, pain, anxiety, depression, increased saliva, constipation
- falls increasingly common with advancing disease
treating fatigue
energy conservation
f
treating muscle stiffness and cramps
stretching
treating pain
- depends on source of pain
- cramps: massage, stretch
- skin pressure: positioning
- Msk: P-AAROM and ex
Treating falls
- ADs, Orthotics, balance training
what does highly repetitive or heavy resistance exercise cause?
prolonged loss of strength in weakened or denervated muscle
what can excessive exercise cause?
- impaired recovery of degenerated muscle fiber
Pulmonary techniques
- 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
overall considerations for exercise
- 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
Safety Considerations for overuse
- 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
Stage I of ALS
- 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.
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
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
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)
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)
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
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.
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.
Goal of Stage VI
- Preserve vital functions through compensation
- Prevent pressure sores, respiratory problems