1. Amyotrophic Lateral Sclerosis (ALS)
Winter 2009

2. Also know as Lou Gehrig's Disease
Named after the New York Yankees baseball star who played first base and was diagnosed with the disease in 1939 and died in 1941 but due to popular belief this disease had been discovered almost 100 years earlier

3. History
Was discovered in 1850 by English scientist Augustus Waller who didn’t know he had discovered ALS but saw nerve fibers that had extreme atrophy
But 19 years later a French neurologist Jean-Martin Charcot took credit for it in publishing the disease in a scientific journal

4. Objectives What is this disease? Signs and Symptoms
Probable causes of ALS
Forms of ALS
Diagnosis of ALS
Medications you will see for ALS patients
Prognosis (outcome)
Stats for ALS

5. What is this disease?
a progressive neurodegenerative disease “is a condition in which cells of the brain and spinal cord are lost.”
This affects mostly motor neurons” the cells that control needed voluntary muscle activity such as speaking, skeletal muscle movement, breathing, and swallowing.”

6. What is ALS cont…
When the motor neurons eventually die, the ability of the brain to control muscle movement is lost. Causing paralysis of essential body systems.
When muscles no longer receive the messages from the motor neurons that they require to function, the muscles begin to atrophy (become smaller).

7. Signs and Symptoms Early symptoms
Increasing muscle weakness “especially in the arms and legs”
Difficulty speaking
Trouble swallowing
Problems with breathing
Twitching
Cramping of muscles (mostly hands and feet)

8. Signs and symptoms cont…
Late Symptoms
Extreme muscle atrophy
Reflexes that are slow to non-existent
Excessive drooling
Babinski's sign: “the big toe dorsiflexes and the other toes fan out “
Increased spasticity (muscle rigidity)
Weight loss
Choking
Cardiac arrest due to respiratory arrest usually resulting in death

9. Probable Causes of ALS
Free radicals. The inherited form of ALS often involves a mutation in a gene responsible for producing a strong antioxidant enzyme that protects your cells from damage caused by free radicals — the byproducts of oxygen metabolism.
Glutamate. People who have ALS typically have higher than normal levels of glutamate, a chemical messenger in the brain, in their spinal fluid. Too much glutamate is known to be toxic to some nerve cells.

10. Probable Causes of ALS cont…
Autoimmune responses. Sometimes, a person's immune system begins attacking some of his or her body's own normal cells, and scientists have speculated that such antibodies may trigger the process that results in ALS.
Thus ALS is not contagious.

11. Forms of ALS
Sporadic - the most common form in the United States - 90 to 95% of all cases.
Familial - genetic “accounts for a small amount of cases in the United States” - 5 to 10% of all cases.
Guamanian - an extremely high incidence of ALS was observed in Guam 1950's.Due to toxins in the water and food

12. How to diagnose x-rays MRI Blood and urine studies
muscle and nerve biopsy
electomyography (EMG)
nerve conduction velocity (NCV)
spinal tap
Even with all this technology ALS is extremely difficult to diagnose. This is because many diseases mimic signs of ALS

13. Common MEDS
Most commonly seen is riluzole Rilutek© is aimed at symptomatic relief basically maintaining optimal quality of life.
Side note : there is no drug interactions to any of the drugs we use as paramedics.
Medical marijuana
Baclofen or diazepam may be used to control spasticity
Trihexyphenidyl or amitriptyline may be prescribed for people with problems swallowing their own saliva.

14. Meds Cont….
O2 and gastric tubes maybe prescribed as well due to trouble breathing and choking
Coming Soon due to Stem cell research maybe a cure ?

15. Prognosis Usually end up needing to go to a care home or have hospice
Still after all these years patients after diagnosis only usually live 3 years

16. STATS
Based on U.S. population studies, a little over 5,600 people in the U.S. are diagnosed with ALS each year (approximately 15 new cases a day.)
Most people who develop ALS are between the ages of 40 and 70
ALS is 20% more common in men than in women.
93% of patients are Caucasian.

17. MORE STATS
Half of all people affected with ALS live at least three or more years after diagnosis.
All stats are courtesy of Studies done by University of Maryland

18. References
"Recent advances in amyotrophic lateral sclerosis". Current Opinion in Neurology 13/4: 397–405
"Playing defense against Lou Gehrig's Disease“ Verlagsgruppe Georg von Holtzbrinck Scientific American, (November 2007)., pp. 86–93.
“Amyotrophic lateral sclerosis: theories and therapies”. Rowland L Journal of Neurological Sciences. 1994;31(169):126–127

19. Ref cont… www.alsa.org als.mdausa.org www.alsinfo.org