Post on 20-Dec-2015
ALS
Kate Crain
Amyotrophic Lateral Sclerosis
Lou Gehrig Disease
Charcot’s Syndrome
Amyotrophic Lateral Sclerosis
Lou Gehrig Disease
Charcot’s Syndrome
A brief historyA 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?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 ClassificationsThree 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 researchPresent 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?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?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?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 ExpectancyLife 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 ALSDiagnosing 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 SymptomsSigns 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 SymptomsSigns 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.
TreatmentTreatment
Physical Therapy Occupational therapy Help with eating and drink may
become necessary Respiratory support may become
necessary BiPAP
Better living through Chemistry?
Better living through Chemistry?
Baclofen may help spasticity Quinine or Phentyloin may help
cramps Anticholenergics may help saliva
production Pain management
RiluzoleRiluzole
Neuroprotective Still not fully understood. Inhibits Glutamate release, inactivation of
voltage-dependant Sodium Channels. Not a cure, but helps in Bulbar-varient ALS.
MyotropinMyotropin
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 GehrigLou Gehrig
June 19, 1903 - June 2, 1941
“Iron Horse” 1939 was his last
season with the Yankees
Morrie SchwartzMorrie Schwartz
Philosophy professor at Brandeis University
Interviewed by Ted Koppel in a series on “Nightline”
Subject of Tuesdays With Morrie
Stephen HawkingStephen 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 contagiousALS is not contagious
Thank you!