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What is Amyotrophic Lateral Sclerosis (ALS)?

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What is Amyotrophic Lateral Sclerosis (ALS)?

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Amyotrophic Lateral Sclerosis is a type of motor neuron disease that is progressive and fatal. Motor neurons in the brain, brainstem and spinal cord degenerate which control voluntary muscle movement and breathing. This leads to progressive muscle weakness, difficulty speaking and breathing. There is no cure for the disease. (National Institute of Neurological Disorders and Stroke, n.d)

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Two groups of motor neurons: the upper motor neurons and lower motor neurons

Upper motor neurons: causes spasticity, slow movements, poor balance and incoordination

Lower motor neurons: causes muscle weakness, atrophy, and twitching

Eventually, motor neurons deteriorate, resulting in their death. The brain can't send signals to muscles --> muscles won't be able to contract. As a result, muscles will shrink and weaken.

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Early Symptoms:

Muscle weakness in distal upper and lower extremities

Spasticity

Slurred speech

Difficulty swallowing

Fatigue

Dropping things

Tripping and falling

Uncontrollable periods of laughing or crying

Advanced Symptoms: (Kiernan, M. C. et al., 2011)

Inability to walk

Muscle atrophy spread to other body parts

Dysphagia

Drooling

Dysarthria

Dyspnea

Death

*ALS does NOT affect intelligence (University of Rochester Medical Center, n.d.)

*Respiratory muscle dysfunction = MAIN CAUSE OF DEATH

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Age (55-75)

Biological sex (men)

Race and ethnicity

Environment & Lifestyle factors:

Toxic chemicals

Military personnel

Physical trauma

Poor diet

History of electric shock

Smoking

Excessive exercise

2 types of ALS:

1. Sporadic ALS:

~90% or more are sporadic

Occurs randomly

No associated risk factors

No genetic component

2. Familial ALS:

5-10% of cases are familial

Inherited from parents

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There is NO specific single test to diagnose ALS

Diagnosis is mainly based on:

History and presence of motor neuron S&S

Along with other tests to rule out other neurological diseases:

Nerve Conduction Study (NCS)

Magnetic Resonance Imaging (MRI)

Lab tests

Electromyogram (EMG)

Muscle or nerve biopsy

Spinal tap

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Treatments focus on:

Managing symptoms

Preventing complications

Maintaining QOL

Treatments:

Heat or whirlpool therapy

Exercise (in moderation)

Medications

Eg. Riluzole --> ONLY disease-modifying drug

Physical Therapy:

Low-impact aerobic exercise

ROM & stretching

Nutritional Support:

Smaller meals + easier to swallow

Feeding tube

Respiratory Support:

Noninvasive ventilation (NIV) (Kiernan, M. C. et al., 2011)

Mechanical cough assist devices

Tracheostomy (aka breathing tube)

Prognosis:

Most people live ~3-5 years after onset of symptoms

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Implications for the Interdisciplinary Team

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Implications for the Interdisciplinary Team

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PT: (Lewis & Rushanan, 2007)

Focus - on maintaining physical fitness, mobility and independence

Create exercise programs

Patient and family education

Electrical stimulation, thermals, ultrasound

PTA:

Show and explain exercise programs for patient

Patient and family education

Support and motivation

Thermals and ultrasound

OT: (Lewis & Rushanan, 2007)

Focus - maintain independence and quality of life

Teach compensatory techniques (i.e. adaptive equipment)

Patient and family education

Energy conservation techniques

OTA:

Teaching adaptive device use

Patient and family education

Provide motivation and encouragement

Energy conservation

Dietician: (Parrish, 2022)

Focus - preventing malnutrition

SLP:

Focus - speech and swallowing issues

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1. What are some early and subtle symptoms individuals with ALS might experience?

2. Which of the following is NOT a risk factor for developing ALS?

a) Physical trauma

b) Lack of exercise

c) Military veterans

d) Exposure to toxic chemicals

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1. Amyotrophic lateral sclerosis (ALS). Johns Hopkins Medicine. (2024, February 23). https://www.hopkinsmedicine.org/health/conditions-and-diseases/amyotrophic-lateral-sclerosis-als

2. Amyotrophic Lateral Sclerosis (ALS). University of Rochester Medical Center. (n.d.). https://www.urmc.rochester.edu/encyclopedia/content.aspx?ContentTypeID=85&ContentID=P00771

3. Amyotrophic lateral sclerosis. Physiopedia. (n.d.). https://www.physio-pedia.com/Amyotrophic_Lateral_Sclerosis

4. Brickman, S. (2016, April 17). The role of occupational therapy with the ALS patient. https://louisville.edu/medicine/cme/documents/5-burgener-occupational

5. Goetz C. G. (2000). Amyotrophic lateral sclerosis: early contributions of Jean-Martin Charcot. Muscle & nerve, 23(3), 336–343. https://doi.org/10.1002/(sici)1097-4598(200003)23:3<336::aid-mus4>3.0.co;2-l

6. Irwin, D., Lippa, C. F., & Swearer, J. M. (2007). Cognition and amyotrophic lateral sclerosis (ALS). American journal of Alzheimer's disease and other dementias, 22(4), 300–312. https://doi.org/10.1177/1533317507301613

7. Kiernan, M. C. et al. (2011). Amyotrophic lateral sclerosis. Lancet (London, England), 377(9769), 942–955. https://doi.org/10.1016/S0140-6736(10)61156-7

8. Lewis, M., & Rushanan, S. (2007). The role of physical therapy and occupational therapy in the treatment of amyotrophic lateral sclerosis. NeuroRehabilitation, 22(6), 451–461.

9. National Institutes of Neurological Disorders and Stroke. (2017, January). Amyotrophic Lateral Sclerosis (ALS). https://www.ninds.nih.gov/health-information/disorders/amyotrophic-lateral-sclerosis-als

10. Parrish, C. R. (2022, April). Nutritional Care of the Patient with Amyotrophic Lateral Sclerosis. https://med.virginia.edu/ginutrition/wp-content/uploads/sites/199/2022/04/April-2022-ALS.pdf

11. Walling A. D. (1999). Amyotrophic lateral sclerosis: Lou Gehrig's disease. American family physician, 59(6), 1489–1496.