1. AMYOTROPHIC LATERAL SCLEROSIS Presentation by: Laura Bigelow EXS 486

2. It is a progressive neurodegenerative disease that affects nerve cells in the brain and spinal cord. Motor neurons are neurons located within the Central Nervous System that directly and indirectly control the muscles. In ALS, a person's motor neurons will degenerate and eventually die When the neurons die, the brain is no longer able to initiate and control movement of the muscles What is Amyotrophic Lateral Sclerosis?

3. Motor Neurons Upper Motor Neurons are located within the brain. These neurons communicate directly with the Lower Motor Neurons, located in the Spinal Cord. The muscles of the body are innervated stemming from the Lower Motor Neurons. In ALS, both the upper and lower motor neurons degenerate or die, and are not longer able to send messages to the muscles. Without communication from the motor neurons, muscles begin to gradually weaken and atrophy. Eventually, the ability of the brain to start and control voluntary movement is lost.

4. Causes of Amyotrophic Lateral Sclerosis In 90% of cases of ALS the cause is completely unknown. 5% of cases show genetic link through mutations on several different genes A mutation in the gene that produces enzyme superoxide dismutase I (SOD I) can be seen in 15-20% of familial cases of ALS. Enzyme that helps control free radicals. As defect on the C9orf72 gene has also been seen in a large amount of ALS patients. Defects in the structure and shape of motor neurons has been seen to cause ALS or make neurons more susceptible to potential environmental toxins. Higher glutamate levels have been seen in cases of ALS, which can be toxic to cells of the body

5. Muscles may be weak, stiff, tight, spastic or soft Muscles may be cramping, twitching( fasciculations), or decrease in muscle size Symptoms may only affect one region or mildly affect more than one area of the body Person may have poor balance, slurred words, weak grip, or increased fatigue A Person may be in this stage or experience these symptoms before a diagnosis is made Person during this stage may benefit from use of cane, leg brace, or other simple support devices. Progression of ALS More regions of muscles are affected and some muscles are paralyzed. Fasciculations continue. Unused muscles may form contractures causing the joints to be rigid,painful, and sometimes deformed Difficulty eating and breathing may increase due to decrease functioning of muscles Pseudobulbar Affect may occur Pseudobulbar Affect In this stage, a patient may use more adaptive equipment such as a wheelchair or assistive talking devices ROM exercises are especially important during this stage for shoulder and knee joints Most voluntary muscles are paralyzed Ability is breathe on the patient's own becomes compromised Unable to care for personal needs Extremely limited mobility Poor respiration may cause fatigue, fuzzy thinking, headaches, and makes a patient more susceptible to pneumonia Patient will most likely be on an invasive or non invasive ventilator Early Stages Middle Stages Late Stages

6. Symptoms of ALS The earliest symptoms include fasciculations, muscle tightness, cramping, muscle weakness affecting an arm or leg,slurred speech, difficulty chewing or swallowing Symptoms of upper motor neuron involvement include spasticity and exaggerated reflexes including an overactive gag reflex or seen through Babinski’s sign Babinski’s sign Large toe extends upward as the sole of the foot is stimulated in a certain way Symptoms of lower motor neuron degeneration include muscle weakness and atrophy, muscle cramps, and fasciculations. To be diagnosed with ALS, people must have signs and symptoms of both upper and lower motor neuron damage that cannot be attributed to other causes.

7. How To Diagnose ALS Step 1: Neurological Exam A neurologist will exam a patient for muscle weakness and changes in the person's voice to determine strength of muscles involved in chewing and swallowing. Lower motor neuron symptoms such as decrease in muscle size and muscle twitching (fasciculations) Upper motor neuron symptoms such as hyperactive reflexes or muscle spasticity Neurologist will also look for signs of pain, loss of sensation, or extra- pyramidal rigidity to rule out Parkinson's type disorders

8. How To Diagnose ALS Step 2: Diagnostic Testing Electromyogram (EMG): tiny electrical shocks are sent through the body to see how fast the nerves conduct electricity, rule out possible nerve blocks, and test to see if nerves that communicate sensation are affected Magnetic Resonance Imaging (MRI): is used to take a detailed picture of the spinal cord, nerves coming from the spinal cord, and bones/ connective tissue surrounding the spinal cord to rule out compression of the spinal cord or major nerves. To rule out other similar disorders a blood test, urine test, or lumbar puncture may be preformed From both these neurological and diagnostic testing, neurologist will be able to accurately diagnose a patent with ALS

9. The Effect of ALS on the Exercise Response Oxygen cost of exercise was increased on leg cycle ergometer potentially caused by increased spasticity of muscles or increased muscle weakness of leg muscles On cycle ergometer,in early stages heart rate and ventilation rates of patients with ALS were within normal limits Patients with later stage ALS may have limited ability to exercise because of respiratory insufficiency and may experience increased fatigue Muscle weakness throughout legs may alter gait which will increase oxygen cost on treadmill

10. There is no cure for ALS Focus of treatment is to strengthen unaffected muscles (early) and maintain functioning The only medication approved by FDA is Riluzole, and is able to Slow progression of disease by reducing chemical messenger in the brain that is higher in people with ALS Slows decrease is muscle strength/functioning involved in swallowing and respiration Cannot reverse damage May cause side effects such as liver damage, dizziness,and gastrointestinal conditions Can increase life expectancy by 60-90 days Treatment http://dailymed.nlm.nih.gov

11. Case Study - Patient Information Mary is a 50 year old female, mother of 3 children who has recently been diagnosed with Amyotrophic Lateral Sclerosis. She has only recently started to notice weakness in her one arm, and still able to walk without the use of assistive devices. She is 5' 4" and weighs 145 lbs. For exercise, she walks most days for 30 minutes. Her mother had a heart attack at age 52, and passed away,but her father is still living. Her blood glucose level is 95 mg/dL. Her cholesterol levels total is 186 mg/dL. Her blood pressure is 146/90 mmHg. She has never smoked.

12. Age: (-) Client in under the age of 55 years old. Not a risk factor Height and Weight: (-) BMI is 24.9 which is in the normal range, not a risk factor Exercise History: (-) Active at least 4 days per week for 45 minutes, not a risk factor. Family History: (+) Client's mother had a heart attack at age 52 and therefore this is a risk factor. Blood Glucose: (-) Blood glucose level is 95 mg/dL, not a risk factor Cholesterol: (-) Total cholesterol is 186 mg/dL, not a risk factor Smoking: (-) Client has never smoked, not a risk factor Hypertension: (+) Blood pressure is 146/90 mmHg therefore this is a risk factor Risk Group: Moderate Risk- she has two risk factors,but is still asymptotic for any cardiovascular,pulmonary, or metabolic disorder Risk Stratification

13. Goals and Special Considerations for Exercise Goals Maximize aerobic capacity, muscular endurance, and functional level for as long as possible Prevent limitations in range of motion Special Considerations Do not over fatigue Balance may become progressively worse Respiratory capacity may decrease over time

14. Assess gait, balance, and range of motion Preform exercise test to judge level of fitness & fatiguability To asses aerobic capacity of this client: 6 Minute Walk Test Submaximal Cycle Ergometer test (Astrand) To asses functional level: Timed up and Go Test Exercise Testing

15. Exercise Prescription: Aerobic Activity Frequency: 3-4 days per week Intensity: 50-60% of VO2r, 5 to 6 on 0 to 10 RPE scale Time: Begin with 15 minutes per day for the first two weeks, increase to 20- 30 minutes per day at week 3. Type: Walking, if balance becomes safety concern switch to stationary/recumbent bicycle

16. Exercise Prescription: Resistance Training Frequency: 2-3 days per week Intensity: 60-70% 1 RM, RPE 5 to 6 Time: 1 set of 10 repetitions, 8-12 exercises to hit all major muscle groups Type: Light machine exercises, if strength decreases use resistance bands. * Free weights should not be used for safety concerns

17. Exercise Prescription: Flexibility Training Frequency: 7-14 times per week, 1-2 times per day Intensity: Hold static stretches at level of 5 to 6 on RPE scale Time: 30 minutes per day, preform 2- 4 receptions of all exercises holding each stretch for 15-30 seconds Type: Static stretching, dynamic stretching, Range of Motion exercises * Focus should be on shoulder, knee, and hip joints

18. Conclusion Clients with ALS cannot be pushed to fatigue or it will interfere with activities of daily living This disease is a progressive disorder meaning exercise prescription will have to be adapted and changed often Exercise can help to slow the progression of ALS and allow person to maintain function for longer

19. References Amyotrophic Lateral Sclerosis (ALS) Fact Sheet. (2014, September 19). Retrieved January 4, 2015, from http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_ALS.htm#26770484 2 http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_ALS.htm#26770484 2 ALS Association. (n.d.). Retrieved January 4, 2015, from http://www.alsa.org Assistance in Stages of ALS Amyotrophic Lateral Sclerosis. (n.d.). Retrieved January 4, 2015, from http://mda.org/disease/amyotrophic-lateral-sclerosis/medical- management/assistance-stages-of-alshttp://mda.org/disease/amyotrophic-lateral-sclerosis/medical- management/assistance-stages-of-als Chapter 8: Exercise Everyday Life with ALS: A Practical Guide. (n.d.). Retrieved January 4, 2015, from http://mda.org/publications/everyday-life-als/chapter-8http://mda.org/publications/everyday-life-als/chapter-8 Riluzole Tablets. (2013, May 1). Retrieved December 28, 2014, from http://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=ace12152-117c-455b- 8e68-e6325657569e&type=display http://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=ace12152-117c-455b- 8e68-e6325657569e&type=display