ALS

Question 1

What is ALS

Answer 1

• rapidly progressive neurodegenerative disease of middle life that is rarely seen prior to age 40

Question 2

What does ALS cause?

Answer 2

• 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)

Question 3

Major signs - LMN

Answer 3

Muscle weakness, atrophy, fasciculations, hyporeflexia, muscle cramps

Question 4

Major signs - UMN

Answer 4

• spasticity, hyper-reflexia, pathological reflexes

Question 5

Major signs - Bulbar signs

Answer 5

• dysarthria, dysphagia

Question 6

Major signs - Respiratory signs

Answer 6

Nocturnal respiratory difficulty, exertional dyspnea, accessory muscle use, paradoxical breathing

Question 7

Major signs - Others

Answer 7

• Fatigue, weight loss, cachexia, tendon shortening, contractures
• Frozen shoulder (30% of persons with ALS)

Question 8

Most frequent initial symptom

Answer 8

• 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

Question 9

ALS prognosis

Answer 9

• 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

Question 10

ALS Medications

Answer 10

• 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

Question 11

Examination/Evaluation

Answer 11

• MMT/Dynamometry (be sensitive)
• Coordination
• Tone
• Balance
• Fatigue
• Psychosocial
• ALSFRS (self grading ADLS)
• ALSAG-40 and ALSAQ-5: well being, multiple domains

Question 12

Consider the Multi-System Nature of the Disease during Examination

Answer 12

• 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

Question 13

Symptoms of ALS

Answer 13

• fatigue
• muscle stiffness
• muscle cramps
• shortness of breath
• sleep, pain, anxiety, depression, increased saliva, constipation
• falls increasingly common with advancing disease

Question 14

treating fatigue

Answer 14

energy conservation

Question 15

f

Question 16

treating muscle stiffness and cramps

Answer 16

stretching

Question 17

treating pain

Answer 17

• depends on source of pain
• cramps: massage, stretch
• skin pressure: positioning
• Msk: P-AAROM and ex

Question 18

Treating falls

Answer 18

• ADs, Orthotics, balance training

Question 19

what does highly repetitive or heavy resistance exercise cause?

Answer 19

prolonged loss of strength in weakened or denervated muscle

Question 20

what can excessive exercise cause?

Answer 20

• impaired recovery of degenerated muscle fiber

Question 21

Pulmonary techniques

Answer 21

• 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

Question 22

overall considerations for exercise

Answer 22

• 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

Question 23

Safety Considerations for overuse

Answer 23

• 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

Question 24

Stage I of ALS

Answer 24

• 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.

Question 25

Stage II of ALS

Answer 25

• 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

Question 26

Stage II ALS exercise considerations

Answer 26

• 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

Question 27

Stage III (middle)

Answer 27

• 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)

Question 28

Adaptive Equipment for Stage III

Answer 28

• 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)

Question 29

Stage IV (middle)

Answer 29

• 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

Question 30

Stage V (Late)

Answer 30

• 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.

Question 31

Stage VI (late)

Answer 31

• Confined to bed and requires maximal assistance with ADLs.
• Proper postural support is needed in bed.
• Frequent repositioning.
• Pain management.
• Progressive respiratory distress.

Question 32

Goal of Stage VI

Answer 32

• Preserve vital functions through compensation
• Prevent pressure sores, respiratory problems