Amyotrophic Lateral Sclerosis (ALS)

Winter 2009

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

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

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

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.”

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).

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)

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

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.

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.

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

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

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.

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 ?

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

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.

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

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

Ref cont…

• www.alsa.org • als.mdausa.org • www.alsinfo.org • www.mayoclinic.com• www.lougehrig.com/about/als • www.pbs.org