PATRICK CASEY FALL 2007 PARAMEDIC CLASS Amyotrophic Lateral Sclerosis

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 new cases per year 90% die with in the first 3-5 years Onset most commonly 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 sclerosis Amyotrophic Lateral Sclerosis Fact Sheet, National Institute of Neurological Disorders and Stroke, 2 November eralsclerosis/detail_amyotrophiclateralsclerosis.ht eralsclerosis/detail_amyotrophiclateralsclerosis.ht

Works cited ALS Informational Movie, Youtube, 2 November X75nURDfM ALS Hug, Youtube, 2 November M Institut Des Neurosciences, Ecole polytechnique Federal De Lausanne, 19 November