amyotrophic lateral sclerosis (als)
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Amyotrophic Lateral Sclerosis (ALS). Patrick Gilbert SPT Northeastern University. ALS Facts. ALS is not contagious About 5,600 people diagnosed per year in the United States Incidence – 2 in 100,000 As many as 30,000 Americans living with disease. What is ALS?. - PowerPoint PPT PresentationTRANSCRIPT
AMYOTROPHIC LATERAL SCLEROSIS (ALS)
Patrick Gilbert SPT
Northeastern University
ALS Facts
ALS is not contagious About 5,600 people diagnosed per year
in the United States Incidence – 2 in 100,000 As many as 30,000 Americans living with
disease
What is ALS?
ALS is a neurodegenerative disease that affects upper and lower motor neurons in the brain and spinal cord
As ALS progresses and more nerves become unable to supply the muscles they innervate, voluntary movement becomes increasingly difficult, then impossible
Ultimately, the majority of people with ALS pass away d/t respiratory complications
How Does ALS Occur?
There is no single reason people develop ALS It can happen to anyone
90-95% of cases are random (sporadic) Strangely, military veterans deployed during
the Gulf war are approximately 2x as likely to develop ALS
Generally develops between the age of 40 and 70, but can occur sooner
The cause is unknown, though some studies suggest a genetic link between cases
Familial ALS
Constitutes 5-10% of cases Found to have a link to a defect in the
gene that produces the copper-zinc superoxide dismutase 1 (SOD1) enzyme
Also found to have a defect in the gene “chromosome 0 open reading frame” (C9orf72) Function of this gene is currently unknown
Symptoms
Symptoms can vary greatly between pts
Muscle weakness generally occurs peripherally Impairment of UE and LE
Fasciculations and/or cramping of muscles, especially distally
Thick or slurred speech Difficulty projecting voice
Diagnosis
No single test to diagnose ALS exists Mainly based on observance of symptoms
Neurologic exams performed at regular intervals to detect worsening of symptoms Muscle weakness/atrophy, hyperreflexia, spasticity
Electromyography (EMG) detects electrical activity in muscles
Nerve Conduction Study (NCS) detects nerves ability to send information/signal Decrease in activity of both EMG and NCS
suggests possibility of ALS
PT Intervention
PTs are an important part of a multidisciplinary team approach to treating patients with ALS
Team approach is crucial to maintaining QoL in pts with ALS
PTs may expect to work with:
OTs, Speech Pathologists Nurses MDs Pharmacists Social Workers And many more!
PT Intervention cont.
Can increase independence through many types of exercise and stretching Low-impact aerobic exercise (while still
able) Stationary bike Walking Swimming
ROM and stretching exercises can prevent: Px Spasticity Contractures
Medication
There is no cure or permanent treatment available for ALS
Riluzole (Rilutek) is the sole FDA approved drug used to combat the effects of ALS
May increase survival and delay tracheostomy by a few months Perform regular LFTs as riluzole
can cause liver damage
Riluzole and Glutamate
Excess release of glutamate can damage motor neurons
Riluzole is an antiglutamate drug that has been clinically shown to slow the progression of ALS Decreases the release of glutamate, which
reduces damage to motor neurons Cannot reverse damage already done Pts remain able to function at a higher level
for longer before ALS robs them of their function
Research
Much research is being done to aid in multiple facets of how health care providers work with and understand ALS Diagnosis Mechanisms of the disease Progression of the disease Rx options and routes
Pluripotent Stem Cell Research
Researchers have developed a method to force skin cells to become pluripotent stem cells
These stem cells are then forced to become motor neurons, among other cells involved with ALS
Creating more, healthy motor neurons may be able to improve function in pts with ALS
References
ALS Association. What is ALS? ALS Association. http://www.alsa.org/about-als/. Updated 2010. Accessed October 11, 2013.
Muscular Dystrophy Association. Amyotrophic Lateral Sclerosis (ALS, Lou Gehrig’s Disease). Muscular Dystrophy Association. http://mda.org/disease/amyotrophic-lateral-sclerosis. Updated 2013. Accessed October 11, 2013.
National Institute of Health. Amyotrophic Lateral Sclerosis (ALS) Fact Sheet. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_ALS.htm#240454842. Updated July 8, 2013. Accessed October 13, 2013.