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ALS
Kate Crain
Amyotrophic Lateral Sclerosis
Lou Gehrig Disease
Charcot’s Syndrome
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.
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.
 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.
 Does not effect Heart, Brain, and
sexual organ function.
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.
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.
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.
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.
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.
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.
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.
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
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.
Treatment
 Physical Therapy
 Occupational therapy
 Help with eating and drink may
become necessary
 Respiratory support may become
necessary
 BiPAP
Better living through
Chemistry?
 Baclofen may help spasticity
 Quinine or Phentyloin may help
cramps
 Anticholenergics may help saliva
production
 Pain management
Riluzole
 Neuroprotective
 Still not fully understood.
 Inhibits Glutamate release, inactivation of
voltage-dependant Sodium Channels.
 Not a cure, but helps in Bulbar-varient ALS.
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.
Lou Gehrig
 June 19, 1903 June 2, 1941
 “Iron Horse”
 1939 was his last
season with the
Yankees
Morrie Schwartz
 Philosophy professor
at Brandeis
University
 Interviewed by Ted
Koppel in a series on
“Nightline”
 Subject of Tuesdays
With Morrie
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
ALS is not contagious
 Thank you!