ALS

About ALSAmyotrophic Lateral Sclerosis (ALS) – also known as Charcot's Disease, Lou Gehrig's disease and Motor Neuron Disease – is a rapidly progressive, and fatal, neuromuscular disease.  It is the most common cause of neurological death on an annual basis in Canada. According to the ALS Society of Canada, between 1,500 and 2,000 people in Canada currently have ALS. Although it most commonly occurs between the ages of 40 and 70, it can also occur in older people, and, although rarely, in teenagers. Approximately 80 per cent of people who have ALS die within two to five years of their diagnosis.ALS destroys motor neurons, which are an important link in the nervous system, and through which the brain controls the voluntary muscles throughout the body. Leg and foot muscles are controlled by motor neurons in the lower spinal cord. Arm, hand and finger muscles are controlled by motor neurons in the upper spinal cord. Speaking, swallowing and chewing are controlled by motor neurons in the brain stem. ALS symptoms include muscle weakness in the arms and legs, slurred speech, twitching and cramping of the muscles, and eventually paralysis.  As the disease progresses and the nerve cells become more damaged, the muscles that control breathing and swallowing become impaired.  Because ALS only destroys the neurons that control muscle movement, the five senses (hearing, sight, smell, touch, and taste) are not normally affected, and people with ALS usually remain alert and their mental processes continue to function.Robarts Scientists Involved in ALS ResearchRobarts scientist and neurologist Dr. Michael Strong is a world leader in the search for a cure for ALS. In addition to being a research scientist, Dr. Strong is a practicing neurologist with one of the largest research-based ALS clinics in Canada. He is acknowledged as one of the Canada’s leading ALS researchers, a role he balances with frontline clinical care of patients affected by the disease.Specifically, Dr. Strong is an expert in understanding the metabolism of proteins – called neurofilaments – that maintain the shape of nerve cells, but clump together and lead to the death of motor neurons in ALS patients. He has found abnormalities in the quantity and organization of these neurofilaments in the motor neurons of people with ALS, and is currently investigating why these masses form and the role they play in the progression of the disease. Although it is not known what 'triggers' these abnormalities, Dr. Strong and his research team have discovered the messages the cell uses to control the production of these proteins (RNA) is fundamentally disrupted in ALS. He has also found, once these masses of neurofilaments are formed, they are dynamic structures that actively disturb the function of the motor neuron and trigger a cascade of events that lead to its death. These discoveries have opened up an entirely new area of investigation into the cause of ALS. Dr. Strong has also discovered a relationship between the injured neurons in ALS and the immune system of the nervous system. His research team has observed that as soon as a neuron is injured, it sends out a signal to the inflammatory cell. Dr. Strong has proven one can transfer the disease to a perfectly healthy motor neuron via the inflammatory cell. This breakthrough opens up the exciting possibility that newly-developed drugs may be able to stop the transmission of the disease to healthy cells.Finally, Dr. Strong, along with his colleagues at Robarts, has pioneered the concept that ALS can be associated with a form of a dementia. In early research with Robarts imaging scientist Dr. Ravi Menon, Dr. Strong has demonstrated that neurons can be lost in regions of the brain in ALS and this loss is associated with dementia. Previously, it was thought this region of the brain wasn’t affected by ALS.  In subsequent studies with Robarts imaging scientist Dr. Ting-Yim Lee, Dr. Strong utilized computed tomography (CT) scanning to assess the blood flow in ALS patients’ brains – a technique developed at Robarts – to determine the amount and speed of the blood flowing through the brain. Using this system, Dr. Strong looks for signs of impaired brain function early in the progression of ALS, so doctors might be able to fight the disease on a new and different front.  For this research, Dr Strong was awarded the Shiela Essey award for ALS research by the American Academy of Neurology in 2005. The Sheila Essey award is the pre-eminent international award for ALS research, with Dr Strong being only the second Canadian to be awarded it.Impact of Research Today, a diagnosis of ALS is equal to a death sentence within two to five years. Dr. Strong is at the forefront of research on this fatal disease and is leading the way to find a cure. Robarts'  commitment and dedication to furthering ALS research offers extraordinary hope for every new patient diagnosed with the disease.