PATRICK CASEYFALL 2007 PARAMEDIC CLASS

Amyotrophic Lateral Sclerosis

Common Names

ALSLou Gerig’s DiseaseNMD (Europe)

What is ALS

• A - MYO - TROPHICwith 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 diseaseTests are used to rule out other diseasesTo 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 cureOne 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 symptomsListen to caregiverLook 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/amyotrophiclateralsclerosis/detail_amyotrophiclateralscl

erosis.ht

Works cited

ALS Informational Movie, Youtube, 2 November 2007 <http://www.youtube.com/watch?v=H-X75nURDfM>

ALS Hug, Youtube, 2 November 2007 <http://www.youtube.com/watch?v=77HO49diVXM >

Institut Des Neurosciences, Ecole polytechnique Federal De Lausanne, 19 November 2007 <http://len.epfl.ch/page46598.html>