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.