About ALS

Amyotrophic lateral sclerosis (also known as ALS, Lou Gehrig’s disease, or motor neuron disease) is a disease that progressively paralyzes people because the brain is no longer able to communicate with the muscles of the body that we are typically able to move at will. Over time, as the muscles of the body break down, someone living with ALS will lose the ability to walk, talk, eat, swallow, and eventually breathe.

ALS is not contagious. There is no cure for ALS and few treatment options for the majority of people living with the disease. Approximately 80 per cent of people with ALS die within two to five years of being diagnosed.

Our brain is connected to our muscles through millions of specialized nerve cells, called motor neurons, which serve as our bodies’ internal wiring and enable us to move our bodies as we choose. Motor neurons work in pairs: an upper motor neuron in the brain extends to the brainstem at the back of the neck or the spinal cord, and a lower motor neuron extends from the brainstem or spinal cord to the muscle. The brain sends a signal along these motor neurons telling a muscle to contract. This signal is an electrical impulse created by chemicals in our neurons.

In ALS, motor neurons gradually break down and die. This means that the brain can no longer communicate with the muscles of the body. As a result, the muscles become weak and eventually someone living with ALS will be unable to move them.

Limb Onset

The most common type of ALS is spinal or limb onset ALS. As the name indicates, limbs (arms and legs) are usually the first areas to be affected. Usually, symptoms begin in the arms or the legs, not both at once.

Symptoms of lower limb onset ALS include:

• Tripping
• Stumbling
• Difficulty running properly
• Foot drop (foot slapping down on the ground when walking rather than rolling smoothly)

Symptoms of upper limb onset ALS include:

• Less finger dexterity
• Cramping in the arm or hand
• Weaker hands
• Stiffness in the arms or hands

Both types of limb onset ALS have other symptoms that indicate whether upper or lower motor neurons are being affected first.

Bulbar Onset

In about 20–25% of ALS cases, the muscles for speaking, swallowing, and/or breathing are the first to be affected. This is known as bulbar onset ALS. The term “bulbar” refers to the motor neurons located in the corticobulbar area of the brainstem, which controls muscles of the face, head, and neck.

Bulbar ALS often progresses faster than limb onset ALS. In rare cases, the respiratory muscles are the first to be affected, and the disease may be classified separately as respiratory onset ALS. People with this type of ALS may be referred directly to acute respiratory care instead of to an ALS clinic or a neurologist.

Symptoms of bulbar ALS include the following:

• Changes in voice and speech
• Harsh, hoarse, or strained voice
• Whispery voice (as a result of the vocal cords not folding as they do normally allowing more air to escape during speech)
• Poor articulation
• Less vocal range
• Quieter voice
• Spasms in muscles of the jaw, face, voice box, throat, or tongue
• Involuntary excessive laughing and crying
• Brisk jaw jerks
• Involuntary twitching in muscles of the tongue
• Vocal cord spasms causing the sensation that air cannot be moved in or out.

For more information on symptoms of ALS, visit here.

Sporadic ALS and Familial ALS

The most common form of ALS is called sporadic ALS, meaning the disease can affect anyone regardless of gender, ethnicity or age—although it most often affects people between the ages of 40 and 60.

The other type of ALS is called familial ALS, which means it is capable of being passed from a parent to his or her child. Approximately 5 to 10% of ALS cases are familial.

Regardless of whether someone has sporadic or familial ALS, 30% of all people with the disease have a form referred to as Bulbar ALS. In the early stages of Bulbar ALS, the motor neurons in the corticobulbar area of the brainstem are the first to be affected. This means that the muscles of the head, face and neck become paralyzed before muscles in other parts of the body.

There is no one thing that causes ALS. Rather, ALS is recognized as having multiple interacting causes that are likely based on changes in people’s genes, and possibly contribution of environmental factors. A number of genes have been identified as playing a role in the development of ALS. Through research, we are learning more about ALS and the multiple factors that may trigger its development.

• More than 200,000 people around the world are living with ALS.
• An estimated 4,000 Canadians are currently living with ALS.
• Each year approximately 1,000 Canadians die from ALS. A similar number of Canadians are diagnosed with ALS each year.
• 80% of people with ALS die within two to five years of diagnosis.