ALS - Leu Gehrig’s Disease

Question 1

ALS Overview / Introduction

Answer 1

• Progressive neurodegenerative disease that attacks corticospinal tracts (UMN) along with the motor cortex (UMN) and brainstem (UMN) and anterior horn cells (LMN
• We do not know cause of this
• Male
• Occurs in 50s mostly, but can be early onset in 20-30s

Question 2

ALS Early Symptoms

Answer 2

• Distal, asymmetrical, focal (specific muscle) weakness
• Bulbar weakness (dysarthria, dysphagia, dysphonia - tongue, swallowing, speaking)
• Spasticity in extremities
• UMN symptoms

Question 3

ALS Late Symptoms

Answer 3

• Flaccidity (wide spread weakness)
• Respiratory depression
• Pseudobulbar affect (poor emotional control - ex: laughing when should be crying)
• LMN symptoms

Question 4

ALS Prognosis

Answer 4

• Rapid progressive
• No cure
• Patient’s normally die
• Death occurs within 3-5 years after diagnosis and usually results from respiratory failure

Question 5

ALS Impairments

Answer 5

• Lung secretion clearance (without this, lung infection can happen)
• Widespread weakness (cervical extensor weakness is common)
• Respiratory weakness
• Energy conservation
• Fatigue
• Spasticity in earlier phases with decreased ROM, flaccidity in later phases

Question 6

ALS Pulmonary Intervention Formula

Answer 6

1. Are secretions stuck? If so, use vibration, percussion, vest, or flutter device
2. Does patient need mobilization and clearance after secretions are unstuck? Then use active cycle breathing technique (relaxed breathing, deep breathing, huff, cough, repeat) or autogenic drainage
3. Does patient just need clearance? If so, how much assistance can they provide? We can do huffing, coughing, manually assisted cough, MI/E, and suctioning

Question 7

ALS Respiratory Interventions

Answer 7

• High frequency chest wall oscillation vest
• MI/E
• IMT (inspiratory muscle training for restrictive conditions)
• Diaphragmatic breathing (used for restrictive conditions)

Question 8

ALS Overuse Weakness

Answer 8

• 3/5 is the magic number
• Overuse weakness does not occur in muscles that have a MMT of 3/5 or greater (anything under this can have overuse weakness where muscles can degrade)
• Strength gains can occur in muscles that are 3/5 MMT or greater
• Heavy eccentrics should be avoided (they can create microtears and harm)
• Do NOT exercise patients to point of fatigue

Question 9

ALS Exercise Prescription

Answer 9

• Frequency = 2-3 days/week on alternate days to give rest time
• Intensity = moderate (70-80% 1RM)
• Time = 30 minutes per session or a minimum of 10 mins x 3
• Type = strength, aerobic
• Morning is better
• Do NOT exercise to point of fatigue

Question 10

ALS Wheelchair Prescription

Answer 10

• Early to Middle Stage = rent short term lightweight wheelchair
• Late Stage = purchase power wheelchair with required modifications (such as sup and puff, head control, or potentially joystick)

Question 11

ALS Orthotics

Answer 11

• HKAFO and KAFO are too heavy - they would need a wheelchair if this is required
• SAFO chosen if patient is homebound, not moving a lot, spasticity, and lacking ankle stability
• Hinged AFO (articulated) chosen if patient is more active, have stairs/ramp to negotiate, but still requires ankle control

Question 12

ALS Spasticity

Answer 12

• Avoid contractures
• Can do splint, PNF, Botox, orthotics, or serial casting
• Positioning out of synergy (3 mins to 3 hours)
• Gentle prolonged strengthening and PROM would not be wrong but probably not the best choice because those are very therapist intensive