Amyotrophic Lateral Sclerosis (ALS)

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