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Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic Lateral Sclerosis (ALS). Patrick Gilbert SPT Northeastern University. ALS Facts. ALS is not contagious About 5,600 people diagnosed per year in the United States Incidence – 2 in 100,000 As many as 30,000 Americans living with disease. What is ALS?.

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Amyotrophic Lateral Sclerosis (ALS)

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  1. Amyotrophic Lateral Sclerosis (ALS) Patrick Gilbert SPT Northeastern University

  2. ALS Facts • ALS is not contagious • About 5,600 people diagnosed per year in the United States • Incidence – 2 in 100,000 • As many as 30,000 Americans living with disease

  3. What is ALS? • ALS is a neurodegenerative disease that affects upper and lower motor neurons in the brain and spinal cord • As ALS progresses and more nerves become unable to supply the muscles they innervate, voluntary movement becomes increasingly difficult, then impossible • Ultimately, the majority of people with ALS pass away d/t respiratory complications

  4. How Does ALS Occur? • There is no single reason people develop ALS • It can happen to anyone • 90-95% of cases are random (sporadic) • Strangely, military veterans deployed during the Gulf war are approximately 2x as likely to develop ALS • Generally develops between the age of 40 and 70, but can occur sooner • The cause is unknown, though some studies suggest a genetic link between cases

  5. Familial ALS • Constitutes 5-10% of cases • Found to have a link to a defect in the gene that produces the copper-zinc superoxide dismutase 1 (SOD1) enzyme • Also found to have a defect in the gene “chromosome 0 open reading frame” (C9orf72) • Function of this gene is currently unknown

  6. Symptoms • Symptoms can vary greatly between pts • Muscle weakness generally occurs peripherally • Impairment of UE and LE • Fasciculations and/or cramping of muscles, especially distally • Thick or slurred speech • Difficulty projecting voice


  7. Diagnosis • No single test to diagnose ALS exists • Mainly based on observance of symptoms • Neurologic exams performed at regular intervals to detect worsening of symptoms • Muscle weakness/atrophy, hyperreflexia, spasticity • Electromyography (EMG) detects electrical activity in muscles • Nerve Conduction Study (NCS) detects nerves ability to send information/signal • Decrease in activity of both EMG and NCS suggests possibility of ALS

  8. PT Intervention • PTs are an important part of a multidisciplinary team approach to treating patients with ALS • Team approach is crucial to maintaining QoL in pts with ALS • PTs may expect to work with: • OTs, Speech Pathologists • Nurses • MDs • Pharmacists • Social Workers • And many more!

  9. PT Intervention cont. • Can increase independence through many types of exercise and stretching • Low-impact aerobic exercise (while still able) • Stationary bike • Walking • Swimming • ROM and stretching exercises can prevent: • Px • Spasticity • Contractures

  10. Medication • There is no cure or permanent treatment available for ALS • Riluzole (Rilutek) is the sole FDA approved drug used to combat the effects of ALS • May increase survival and delay tracheostomy by a few months • Perform regular LFTs as riluzole can cause liver damage

  11. Riluzole and Glutamate • Excess release of glutamate can damage motor neurons • Riluzole is an antiglutamate drug that has been clinically shown to slow the progression of ALS • Decreases the release of glutamate, which reduces damage to motor neurons • Cannot reverse damage already done • Pts remain able to function at a higher level for longer before ALS robs them of their function

  12. Research • Much research is being done to aid in multiple facets of how health care providers work with and understand ALS • Diagnosis • Mechanisms of the disease • Progression of the disease • Rx options and routes

  13. Pluripotent Stem Cell Research • Researchers have developed a method to force skin cells to become pluripotent stem cells • These stem cells are then forced to become motor neurons, among other cells involved with ALS • Creating more, healthy motor neurons may be able to improve function in pts with ALS

  14. References • ALS Association. What is ALS? ALS Association. http://www.alsa.org/about-als/. Updated 2010. Accessed October 11, 2013. • Muscular Dystrophy Association. Amyotrophic Lateral Sclerosis (ALS, Lou Gehrig’s Disease). Muscular Dystrophy Association. http://mda.org/disease/amyotrophic-lateral-sclerosis. Updated 2013. Accessed October 11, 2013. • National Institute of Health. Amyotrophic Lateral Sclerosis (ALS) Fact Sheet. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_ALS.htm#240454842. Updated July 8, 2013. Accessed October 13, 2013.

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