Transcript Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis
PATRICK CASEY
FALL 2007 PARAMEDIC CLASS
Common Names
 ALS
 Lou Gerig’s Disease
 NMD (Europe)
What is ALS
• A - MYO - TROPHIC
with out - muscle - nutrition
LATERAL SCLEROSIS
ALS is “a rapidly progressive, invariably fatal,
neurological disease that attacks the nerve cells
responsible for controlling voluntary muscles”
The A and P
 Voluntary (motor) nerve fibers in:
 The brain (Upper Motor Neurons)
 Spinal cord (Lower Motor Neurons)
and in the periphery die for an
unknown reason. All of the involuntary
nerves are spared. Patients can see,
smell, think and most do not loose
bowel control.
The A and P
ALS
“just the facts maam”
• 20,000 cases in the U.S.
• 5000 new cases per year
• 90% die with in the first 3-5 years
• Onset most commonly 40-60 y/o
• More common in men
• 90-95% have no clear cause
• 5-10% inherited
• 20%, or 10% depending on who you ask, of
inherited cases have been traced back to one gene
mutation. (superoxide dismutase 1)
Signs and Symptoms
 Mild at first:
 Twitching
 Cramping
 Muscle stiffness
 Localized weakness
 Changes in speech
Signs and Symptoms
 Then progressively worsening to:
 Obvious weakness
 Awkwardness when walking
 Tripping and falling increasingly often
 Greater speech problems
 Loss of abilities to perform daily tasks such as:
Buttoning shirts
 Writing
 Turning a key in a lock

Signs and Symptoms
 Patients Eventually have:
 Loss of coordinated movement
 Difficulty swallowing
 Difficulty speaking
 Tight, stiff muscles
Signs and Symptoms
 Finally the patient will loose control of their
diaphragm and suffocate if not placed on a
ventilator.
 Most patients die from respiratory complications.
 In late ALS muscle fasciculations and postitive
Babinski’s sign may be present.
Diagnosis
 No test for disease
 Tests are used to rule out other diseases
 To be diagnosed patient must present with:
 Upper motor damage (Positive Babinski’s sign)
 Lower motor damage (muscle weakness, cramps)
Diagnosis
 Diseases that commonly must be ruled out:
 HIV
 T-cell Leukemia
 Lyme Disease
 Multiple Sclerosis
 Post Polio Syndrome
 Multifocal Muscle Neuropathy
 Spinal Muscle Atrophy
Treatment and Research
 There is no cure
 One drug on the market and others in research that
slow the disease.
 Tx is comfort care and treat symptoms

Ventilators, electric wheel chairs, computers that speak,
feeding tubes…
 Not reversible.
 Research is being done but little is known about
the disease.
What should EMS do with this patient?
 Treat symptoms
 Listen to caregiver
 Look for Hospice paperwork
Works cited
Wikipedia. amyotrophic lateral sclerosis, 18 Nov. 2007
<http://en.wikipedia.org/wiki/Amyotrophic_lateral_
sclerosis>
Amyotrophic Lateral Sclerosis Fact Sheet, National
Institute of Neurological Disorders and Stroke, 2
November 2007
http://www.ninds.nih.gov/disorders/amyotrophiclat
eralsclerosis/detail_amyotrophiclateralsclerosis.ht
Works cited
 ALS Informational Movie, Youtube, 2 November
2007 <http://www.youtube.com/watch?v=HX75nURDfM>
 ALS Hug, Youtube, 2 November 2007
<http://www.youtube.com/watch?v=77HO49diVX
M>
 Institut Des Neurosciences, Ecole polytechnique
Federal De Lausanne, 19 November 2007
<http://len.epfl.ch/page46598.html>