Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, is a progressive and fatal neurodegenerative disease that initially weakens, and eventually destroys motor neurons.
Motor neurons are essential for the transmission of signals between the brain and skeletal muscles. As these pathways deteriorate so does a patients ability to control muscles.
In most cases, ALS does not affect cognitive abilities - the mind is often unaffected. New research shows that approximately one third of ALS patients will have some form of cognitive impairment, but this impairment does not affect survival.
Regardless of what part of the body is first affected by Amyotrophic Lateral Sclerosis (ALS), muscle weakness and atrophy always progresses. The speed of progression and severity of intial affects differs from individual to indvidual, but the affects are fatal (90% of patients die within 3-5 years of symptoms).
Individuals affected by ALS have increased problems with mobility, swallowing, speaking, and/or forming words.
As the disease progresses to the later stages individuals will have difficulty breathing (due to a weakened respiratory system). Ventilation support can reduce problems associated with breathing, and has in some cases prolonged survival, however, ventililators do not affect the progression of ALS.
ALS is difficult to diagnose because there is not one test which provides a definitive diagnosis. While the presence of upper and lower motor neuron degeneration is suggestive of ALS, a diagnosis cannot be based on this evidence alone. Instead, a doctor (neurologist) upon presentation of ALS symptoms must run a series of tests to eliminate other diseases with similar symptoms.
90% of people with ALS die from respiratory failure within three to five years from the onset of symptoms. Approximately 5% percent of individuals with ALS will survive for more than 10 years.
At the moment, there is no cure for ALS, diagnosis is difficult, and the causes of ALS are not completely understood.
Amyotrophic Lateral Sclerosis (ALS) Facts
- Quebec and France, referred to as “Amytrophic la Sclérose Latérale”
International ALS Facts
- ALS was first recognized in 1869 by French neurologist Jean-Martin Charcot.
- Between five and ten of every 100,000 people in the world will develop ALS.
- 90% of ALS patients will die within three to five years of diagnosis.
- Half of all people diagnosed with ALS die within 14 months.
- 5-10% of ALS cases are familial (an inherited genetic defect).
- 90-95% of ALS cases are sporadic (studies show cases of sporadic ALS are increasing internationally).
- Every 90 seconds someone in the world is diagnosed with ALS.
- Approximately 80% of reported cases of ALS/MND are individuals between the ages of 40 and 70.
- The gene defect responsible for approximately 40% of familial forms of ALS has been identified (located on chromosome 21, the gene codes the enzyme named superoxide dismutase [SOD])
United Kingdom ALS Facts
- Approximtely 3 people are diagnosed with ALS every day.
- Approximtely 3 people a day die of ALS in the United Kingdom.
- Approximately 5,000 individuals in the UK live with motor neurone disease (MND).
American ALS Facts
- As many as 5,600 Americans are diagnosed with ALS every year.
- As many as 30,000 Americans live with ALS.
- More than 120,000 cases of ALS are diagnosed worldwide annually.
Canadian ALS Facts
- Eighty percent of people with ALS die within two to five years of diagnosis
- Approximately 2,500 - 3,000 Canadians currently live with ALS.
Motor Neuron Disease (MND) Facts
- United States and Canada, referred to as “Motor Neuron Disease”
- United Kingdom, referred to as “Motor Neurone Disease”
- Quebec and France, referred to as “Maladie Neurodégénérative”