A L S

1. A L S By: Frank Marinaro

2. Ride For Life • http://www.youtube.com/watch?v=DeW1vnH_wis

3. What is it? • Amyotrophic lateral sclerosis • Generally, patients loose strength in muscles throughout the body. Eventually when the diaphragm and muscles in the chest wall fail, breathing subsides.

4. History • 1850 - English scientist Augustus Waller describes the appearance of shriveled nerve fibers • 1869 - French doctor Jean-Marie Charcot first describes ALS in scientific literature • 1881 - "On Amyotrophic Lateral Sclerosis" gets translated into English and published in a three-volume edition of Lectures on the Diseases of the Nervous System • 1939 - ALS becomes a cause célèbre in the United States when baseball legend Lou Gehrig's career—and, two years later, his life—is ended by the disease. • 1950s - ALS epidemic occurs among the Chamorro people on Guam • 1991 - Researchers link chromosome 21 to FALS • 1993 - SOD1 gene on chromosome 21 found to play a role in some cases of FALS • 1996 - Rilutek becomes the first FDA-approved drug for ALS • 1998 - El Escorial is developed as the standard for confirming ALS • 2001 - Alsin gene on chromosome 2 found to cause ALS2

5. Who it Affects • Men and Woman (men more frequently) • All races • Primarily between the ages of 40 and 60 • Roughly 20,000 people in the US

6. Interesting Diagnosis • Regions in the Pacific have seen abnormally high instances of ALS. • The dietary habits are believed to have played a role in the high occurrence of the disease.

7. CYCAD • Cycads are a group of seed plants characterized by a large crown of compound leaves and a stout trunk. • They are found in South and Central America, Mexico, the Antilles, south-eastern United States of America, Australia, Melanesia, Micronesia, Japan, China, Southeast Asia, India, Sri Lanka, Madagascar, and southern and tropical Africa

8. Symptoms • The earliest symptoms may include: • Twitching • Cramping or stiffness of muscles • Weakness of arm or leg muscles • Slurred and nasal speech • Difficulty chewing or swallowing

9. Causes • No definitive cause is known • However, an inherited genetic defect on chromosome 21 is believed to cause approximately 40% of familial cases of ALS • Some other possible causes: • Triggered by a virus • Exposure to neurotoxins • DNA defects • Immune system abnormalities

10. Internal

11. Diagnosis • There is no one test to diagnose ALS • EMG • NCV • MRI

12. SOD1 • 1993- Scientists discovered that mutations in the gene that produces the superoxide dismutase (SOD1) enzyme were associated with some cases (approximately 20%) of familial ALS

13. Glutamate • Studies have shown that glutamate plays a role in motor neuron degeneration. • Scientists have found that, compared to healthy people, ALS patients have higher levels of glutamate in the serum and spinal fluid. Laboratory studies have demonstrated that neurons begin to die off when they are exposed over long periods to excessive amounts of glutamate

14. Riluzole/Rilutek • Only FDA approved drug, targets glutamate transporters • Has shown only modest improvements, proving that glutamate may not be a cause of the disease.

15. Survival • ALS will eventually kill you • From diagnosis the average lifespan is 18-48 months • 10% of patients live more than 10 years • Most patients do not actually die from the disease. Respiratory failure, or pneumonia are often the cause of death.

16. Works Cited • http://en.wikipedia.org/wiki/Amyotrophic_lateral_sclerosis • http://www.rideforlife.com/ • http://www.alsa.org/