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!