Patrick Casey

1
Amyotrophic Lateral Sclerosis

• Patrick Casey
• Fall 2007 Paramedic Class

2
Common Names

• ALS
• Lou Gerigs Disease
• NMD (Europe)

3
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

4
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.

5
The A and P
6
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)

7
Signs and Symptoms

• Mild at first
• Twitching
• Cramping
• Muscle stiffness
• Localized weakness
• Changes in speech

8
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

9
Signs and Symptoms

• Patients Eventually have
• Loss of coordinated movement
• Difficulty swallowing
• Difficulty speaking
• Tight, stiff muscles

10
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
  Babinskis sign may be present.

11
Diagnosis

• No test for disease
• Tests are used to rule out other diseases
• To be diagnosed patient must present with
• Upper motor damage (Positive Babinskis sign)
• Lower motor damage (muscle weakness, cramps)

12
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

13
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.

14
What should EMS do with this patient?

• Treat symptoms
• Listen to caregiver
• Look for Hospice paperwork

15
Works cited

• Wikipedia. amyotrophic lateral sclerosis, 18
  Nov. 2007 lthttp//en.wikipedia.org/wiki/Amyotroph
  ic_lateral_sclerosisgt
• Amyotrophic Lateral Sclerosis Fact Sheet,
  National Institute of Neurological Disorders and
  Stroke, 2 November 2007 http//www.ninds.nih.gov/d
  isorders/amyotrophiclateralsclerosis/detail_amyotr
  ophiclateralsclerosis.ht

16
Works cited

• ALS Informational Movie, Youtube, 2 November 2007
  lthttp//www.youtube.com/watch?vH-X75nURDfMgt
• ALS Hug, Youtube, 2 November 2007
  lthttp//www.youtube.com/watch?v77HO49diVXM gt
• Institut Des Neurosciences, Ecole polytechnique
  Federal De Lausanne, 19 November 2007
  lthttp//len.epfl.ch/page46598.htmlgt