What is ALS?

ALS stands for Amyotrophic Lateral Sclerosis, also called Lou Gehrig's disease in North America and motor neuron disease in the UK. It is a rapidly progressive and fatal neuromuscular disease that is characterized by degeneration of a select group of nerve cells and pathways (motor neurons) in the brain and spinal cord. This loss of motor neurons leads to progressive paralysis of the voluntary muscles. The heart is not a voluntary muscle, and therefore remains unaffected by the disease. However, since breathing is controlled voluntarily by the chest muscles, death usually occurs when the chest muscles are no longer able to help the lungs adequately expel carbon dioxide.

"Amyotrophic" means:

A = absence of
myo = muscle
trophic = nourishment
lateral = side (of spine)
sclerosis = hardening or scarring

ALS is not contagious, but it is fatal. For the most part, the battle is short, with 80 per cent losing their lives within two to five years of diagnosis. Between 10 and 20 per cent live 10 years or more after diagnosis, while others live only a few months. The cause is unknown, but research is being conducted in areas relating to genetic predispositions, viral or infectious agents, environmental toxins and immunological changes.

For some people, the muscles for speaking, swallowing or breathing are the first to be affected. This is known as bulbar ALS. The term "bulbar" refers to the motor neurons located in the brain stem that control the muscles used for chewing, swallowing and speaking. ALS symptoms, and the order in which they occur, vary from one person to another.

In the majority of cases, ALS affects the lower portion of the spinal cord first. This is known as limb onset ALS. In these cases, the first signs of ALS are usually muscle weakness, cramps and weakened reflexes in the arms and legs. The rate of muscle loss can vary significantly from person to person with some patients having long periods with very slow degeneration, and others experiencing rapid degeneration.

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Signs and symptoms

Upper Motor Neuron Degeneration

muscle stiffness or rigidity
emotional lability (decreased ability to control emotions)
excessive fatigue
dysphagia (difficulty swallowing)
dyspnea (shortness of breath)
dysarthria (a speech disorder caused by impairment of the muscles used for speaking. Speech is slurred, slow, and difficult to produce)
hyperreflexia (increased or 'brisk' reflexes)
gait spasticity (stiffened legs with toes that drag and catch when walking)

Lower Motor Neuron Degeneration

muscle weakness and atrophy
involuntary contraction of muscle fibres
muscle cramps
weakened reflexes
flaccidity (decreased muscle tone)
difficulty swallowing
disordered articulation
shortness of breath at rest

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Is ALS a rare disease?

ALS is not considered a rare disease. Approximately 2,500 to 3,000 Canadians currently live with ALS. Two or three Canadians are diagnosed with ALS daily, while another two to three lose their battle with ALS every day. In Ontario, more than 1,000 people have ALS at any one time. While these numbers may seem insignificant, ALS is five times more prevalent than Huntington’s disease, four times more prevalent than muscular dystrophy, and has a similar incidence rate as multiple sclerosis. The number of people with ALS is smaller than people with these other neurological diseases because people with ALS often die so rapidly.

According to Dr. Michael Strong, chief of neurology at the University Health Sciences Centre and research scientist at the Robarts Research Institute in London, Ontario, "ALS is clearly the most common cause of neurological death on an annual basis." Furthermore, the World Health Organization (WHO) estimates that neurological diseases such as ALS will surpass cancer to become the second-leading cause of death in Canada by 2040.

What causes ALS?

The cause of ALS is unknown, but we do know that it can strike any adult at any time. While the usual age at onset is between 45 and 65, people as young as 16 have been diagnosed. Males and females are affected almost equally, with a slightly higher percentage of males diagnosed with ALS. People of all ethnicities and social strata may be affected. Between five and 10 per cent of ALS cases are inherited, meaning that they are "familial" and are definitely linked genetically. In most cases, an ALS diagnosis is sporadic and we don't know how it is caused. Many researchers believe ALS is the product of multiple factors.

What are the early symptoms?

ALS usually becomes apparent either in the legs, the arms, the throat or the upper chest area. Some people begin to trip and fall, some may notice muscle loss in their hands and arms and some find it hard to swallow and slur their speech.

ALS is difficult to diagnose. There is no specific test available that will either rule out or confirm the presence of ALS. Diagnosis is usually made through a 'diagnosis of exclusions'. Neurologists conduct a number of tests, thereby ruling out other disorders or illnesses that may cause similar symptoms, such as stroke, multiple sclerosis or lyme disease. If a person tests negative for all these tests but the symptoms continue to worsen, ALS is often the reason.

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What are the effects of ALS?

Because ALS frequently takes its toll before being positively diagnosed, many patients are debilitated before learning they have ALS. The disease usually does not affect the senses of taste, touch, sight, smell, and hearing. Eye and bladder muscles, along with sexual function and drive, are typically unaffected. In some cases, people with ALS can present with personality changes known as cognitive impairments. The exact percentage of people with ALS who experience cognitive impairment is unknown, but is estimated at 15 to 50 per cent.

ALS has a devastating effect on patients and their families. As families cope with the prospect of advancing disability and eventual death, their financial and emotional reserves are consumed. ALS is a costly disease, demanding both extensive nursing care and expensive equipment.

What can be done about ALS?

There is no known cure at this time and little in the way of treatment that will have an effect on the disease itself. The only drug approved for people with ALS is Rilutek (riluzole). Researchers don’t fully understand how Rilutek works, but think it may protect nerve cells from overexposure to glutamate, a naturally occurring substance that carries signals to the motor neurons. Too much glutamate may cause motor neuron dysfunction and death, preventing the neurons from relaying the brain’s messages to the muscles. Rilutek has been found to extend the life of some people with ALS by a few months.

Is there hope for people with ALS?

Researchers are looking to find not only the cause of the disease so that a cure can be developed, but also other medications or treatments that can help until a cure is found. With improved knowledge about ALS, healthcare providers and families can help people living with ALS live life more fully. The services offered by the ALS Society of Ontario help improve the quality of life for those who live with ALS and their families.

If you have been diagnosed with ALS and would like more information on our services or would like to download a registration form please visit Our Services page.

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