1. ALS Kate Crain

2. Amyotrophic Lateral Sclerosis Lou Gehrig Disease Charcot’s Syndrome

3. A brief history  Discovered in 1874 by French physician J.M. Charcot.  Was thought to be caused by the same virus as Polio.  Maybe its an environmental toxin causing it?  An extremely high incidence observed in Guam and trust territories of the Pacific in the 1950’s.

4. What is ALS? Classified as a Motor Neuron Disorder. Progressive neurodegenerative disease that effects nerve cells in the brain and spinal cord. A-myo-trophic means no muscle nourishment. Lateral identifies areas of spinal cord where nerve cells that control muscles are located. Sclerosis is hardening or scarring.

6.  Neuron is damaged and the signal to the muscle is cut off causing muscular atrophy.  Effects muscles innervated by the cranial nerves and corticobulbar tracts.  Involuntary muscles not effected.

7.  Does not effect Heart, Brain, and sexual organ function.

8. Three Classifications  Sporadic - Most common, occurring in 90 - 95% of cases.  Familial - When more than one case occurs in a family, (Possibly genetic dominant inheritance) 50% chance offspring will inherit it in these families.  Guamanian - Isolated to those in Guam and Japan.

9. Present research  Exact cause is still unknown.  Presently thought to be an autoimmune disease, however…  Also thought to be the possible result of excessive levels of Glutamate in the brain.

10. What is Glutamate?  Glutamic Acid creates the taste umami.  Found in muscles, brain, kidneys, and liver for metabolism.  Non-essential Amino Acid.  Breaks down food and builds up tissue.  Too much is found to be toxic.

11. Glutamate and ALS?  Elevated levels have been found in the ICF of some ALS patients.  Experiments have found The Glutamate Regulating Protein missing or ineffective in Some patients.  Exact link is still unknown.

12. Who is at risk?  Exact risk factors are still a mystery.  5,600 people are diagnosed each year.  As many as 30,000 Americans have ALS at any given time.  60% of ALS patients are men.  93% of patients are white.  Most patients are between 40 - 70 years old at diagnosis, average age is 55.

13. Life Expectancy  Most live 3 years after diagnosis.  Up to 10% live longer than 10 years.  In rare cases, the disorder will halt or remit symptoms.

14. Diagnosing ALS  Hard to diagnose.  No exact test, must rule out everything else.  Testing includes MRI, EMG, blood and urine studies, spinal tap, muscle and/or nerve biopsy.  Many diseases mimic ALS.

15. Signs and Symptoms  Weakness in hands and feet  Fasciculations, spastic, hyperactive deep tendon reflexes  Clumsiness  Slurred speech  Dysphagia  Fatigue  Difficult controlling facial expressions and tongue  Stiffness in movements  Uncontrolled laughter or crying  Cramps

16. Signs and Symptoms  Onset is so slight it may be overlooked.  Muscle weakness is initial symptom in 60% of patients.  Early Dysphagia is poor prognosis due to complications with airway and aspiration.

17. Treatment  Physical Therapy  Occupational therapy  Help with eating and drink may become necessary  Respiratory support may become necessary  BiPAP

18. Better living through Chemistry?  Baclofen may help spasticity  Quinine or Phentyloin may help cramps  Anticholenergics may help saliva production  Pain management

19. Riluzole  Neuroprotective  Still not fully understood.  Inhibits Glutamate release, inactivation of voltage-dependant Sodium Channels.  Not a cure, but helps in Bulbar-varient ALS.

20. Myotropin  In “Investigational New Drug” status with FDA.  Recombinant Insulin-like growth factor that seems to prevent neuron loss and promote neuron regeneration in animal studies.

21. Lou Gehrig  June 19, 1903 - June 2, 1941  “Iron Horse”  1939 was his last season with the Yankees

22. Morrie Schwartz  Philosophy professor at Brandeis University  Interviewed by Ted Koppel in a series on “Nightline”  Subject of Tuesdays With Morrie

23. Stephen Hawking  A Brief History Of Time.  Black Holes and Baby Universes.  The Universe in a Nutshell.  Diagnosed at 21  Father of three after diagnosis

24. ALS is not contagious  Thank you!