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Amyotrophic Lateral Sclerosis (ALS) Fact Sheet

Published: 9 June 2009

ALS and The Montreal Neurological Institute (MNI)

About ALS:

Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s disease, is a devastating neurodegenerative disease affecting between two and five people per 100,000. Approximately 2,500 - 3,000 Canadian adults currently live with ALS. 80% per cent of people with ALS die within two to five years of diagnosis. The major symptoms of ALS are muscle weakness and wasting, progressively worsening to the point of paralysis and inability to breathe. These symptoms result from the death of motor neurons, nerve cells in the brain and spinal cord that control movement of voluntary muscles. (source www.als.ca)

MNI and ALS research and treatment initiatives:

The Montreal Neurological Institute and Hospital (The Neuro) is committed to the highest level of care for patients and families with ALS through a dynamic program that encompasses exceptional clinical care as well as basic and clinical research. 

The Neuro's multidisciplinary ALS clinic was established in 1998 and is recognized as a leader within Québec, Canada, and the world, in the delivery of care to this very special patient population.  It is considered a model program for multidisciplinary clinics. As Director of the Clinical Research Unit, Dr. Angela Genge leads an important ALS clinical research program, developing and participating in clinical trials with new ALS therapies and combination therapies along with collaborators in Quebec and internationally.

Scientists at The Neuro are forging local, national and international partnerships in order to advance research into the potential causes of ALS and development of effective treatments.

Dr. Heather Durham specializes in developing tissue culture models of ALS to understand what makes nerve cells susceptible to damage in motor neuron diseases such as ALS, and to test candidate therapies. Her particular interest is in developing treatments that activate cellular stress responses to fight disease.  Dr. Durham collaborates with investigators at the CHUM, led by Dr. Guy Rouleau, to establish models of genetic motor neuron disease; with pharmaceutical and biotech companies to test putative therapies in culture and animal models of motor neuron diseases, and with Dr. David Burns in the Department of Chemistry at McGill to identify biomarkers of ALS in blood samples. Dr. Durham also is working at the national level to further leverage ALS research initiatives, as a member of the Board of the ALS Society of Canada and Chair of the Research Policy Committee. On behalf of the ALS Society, she accepted the Partnership Award of the Year in 2008 awarded by the CIHR Institute of Neuroscience, Mental Health and Addiction.  

Dr. Stefano Stifani’s research focuses on understanding the generation, muscle connectivity, and regeneration of nerve cells important for mastication, swallowing, and motor activities. His lab has made important strides in elucidating the intricate mechanisms of how those particular types of neurons develop. The ultimate goal of Dr. Stifani’s program is to provide strategies to manipulate neural stem cells and facilitate the design of approaches that may promote the repair of the adult nervous system in response to trauma or disease. 

Dr. Peter McPherson’s laboratory uses biochemical, molecular, structural and cellular approaches to discover and understand the function of proteins in nerve cells.  His lab has identified a number of proteins that could play a fundamental role in neurodegenerative and motor neuron diseases, furthering our understanding of the basic mechanisms of disease. Dr. McPherson is also working on the basic mechanisms underlying hereditary spastic paraplegias (HSPs), a genetically diverse group of motor neuron diseases related to ALS.

Dr. Eric Shoubridge’s laboratory investigates the molecular genetics of disease. Their work has led to major advances in understanding the mechanisms and pathways underlying numerous metabolic disorders.  This work has formed the basis for genetic tests which have improved diagnoses and treatment.

ALS and community support:

Former quarterback for the Aloulettes team in the CFL, and guest coach for the Alouettes training camp for a second year in a row, Tony Proudfoot established the Tony Proudfoot Fund after being diagnosed with ALS in 2007.  The fund raises awareness of this devastating disease and provides vital support for ALS research at The Neuro and patient and family services at The ALS Society of Quebec.  Dr. Miranda Tradewell, in Heather Durham’s lab, holds the Tony Proudfoot postdoctoral fellowship.

The ALS Society of Quebec, established in 1983, provides support for individuals living with ALS and their families, creates awareness, and raises funds for patient services and research. The ALS Society of Quebec works in partnership with the other provincial agencies and the ALS Society of Canada.

The Fondation André-Delambre was created in October 2003 when André Delambre, a well-known businessman and associate of Réné Angelil and Céline Dion, was diagnosed with ALS. The foundation was set-up to assist patients and their families and to support research, including a clinical fellowship at the MNI. Danielle Lavoie, a medical doctor and current André-Delambre fellow is designing protocols for clinical trials and working on treatments for ALS.

About the Montreal Neurological Institute and Hospital:

The Montreal Neurological Institute and Hospital (The Neuro) is a unique academic medical centre dedicated to neuroscience. The Neuro is a research and teaching institute of McGill University and forms the basis for the Neuroscience Mission of the McGill University Health Centre. Founded in 1934 by the renowned Dr. Wilder Penfield, The Neuro is recognized internationally for integrating research, compassionate patient care and advanced training, all key to advances in science and medicine. Neuro researchers are world leaders in cellular and molecular neuroscience, brain imaging, cognitive neuroscience and the study and treatment of epilepsy, multiple sclerosis and neuromuscular disorders. For more information, please visit www.mni.mcgill.ca.

 

 

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