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What is ALS?

Amyotrophic Lateral Sclerosis (ALS), commonly known as Lou Gehrig’s disease, is a deadly neurodegenerative disease.Ìý Approximately 3,000 Canadian adults have ALS. ÌýMost patients have no family history of the disease, in fact less than 10 % have a familial form of ALS.Ìý A large majority of Ìýpatients die within two to five years of their diagnosis. ÌýThe main symptom is muscle weakness that progressively reaches the point of paralysis and prevents a person from breathing. ÌýALS is a result of the death of motor neurons---nerve cells in the brain and spinal cord that control voluntary muscle movement.

ALS research and treatment initiatives at The Neuro

The Montreal Neurological Institute and Hospital – The Neuro, Â鶹AV is committed to providing the highest level of clinical care for ALS patients and their families, as well as state-of-the-art facilities for basic and clinical research.Ìý

A multidisciplinary ALS clinic was established at The Neuro in 1998.Ìý ÌýToday the clinic is recognized as a leader in caring for this special patient population, and its program is considered a model for multidisciplinary clinics.ÌýÌý As a neurologist and Director of The Neuro's Clinical Research Unit, Dr. Angela Genge leads an ALS clinical research program that develops and clinically tests new ALS therapies in collaboration with researchers in Quebec and abroad.

In 2011, two leading researchers joined The Neuro’s ALS team.Ìý Dr. Heidi McBride, a Â鶹AV graduate, is an internationally recognized expert on mitochondrial biology, the study of how mitochondria provide energy to cells, particularly to muscle cells.Ìý The second new recruit, Dr. Hiroshi Tsuda, studies experimental models of the disease.Ìý These models help to understand what makes nerve cells susceptible to damage in ALS and in other motor neuron diseases, as well as to allow researchers to test experimental therapies.

The Unit's clinical research includes a multicentre clinical trial of the experimental drug, ceftriaxone.ÌýÌý Other clinical trials are being developed in collaboration with the Canadian ALS Clinical Trial Consortium (CALS).Ìý In addition, a symptom management trial is being considered for control of pain and spasticity.ÌýÌýÌý

The Neuro's scientists are constantly forging local, national and international partnerships as the best way to advance research into the causes of ALS and to develop effective treatments.

For more than 20 years, Dr. Heather Durham has devoted her research to ALS.ÌýÌý She specializes in developing tissue culture models of the disease. ÌýÌýDr. Durham is particularly interested in developing treatments that activate cellular stress responses that fight the disease.Ìý She's collaborating with investigators led by Dr. Guy Rouleau at the Centre Hospitalier de l'Université de Montréal (CHUM) to establish models of genetic motor neuron disease.Ìý In conjunction with pharmaceutical and biotech companies, she is testing two putative therapies for motor neuron diseases in culture and animal models. ÌýDr. Durham also works to promote ALS research initiatives across Canada.Ìý She serves as a Board member of the ALS Society of Canada and as Chair of the Research Policy Committee. ÌýOn the ALS Society's behalf, she accepted the Partnership Award of the Year in 2008 bestowed by the CIHR Institute of Neuroscience, Mental Health and Addiction.Ìý

Dr. Peter McPherson’s laboratory uses biochemical, molecular, structural and cellular approaches to discover and understand the function of proteins in nerve cells.Ìý His laboratory has identified proteins that could play a fundamental role in neurodegenerative and motor neuron diseases.Ìý ÌýDr. McPherson is also working on the basic mechanisms underlying hereditary spastic paraplegias (HSPs), a genetically diverse group of motor neuron diseases related to ALS.Ìý One of Dr. McPherson’s lab members, Jason Hamelin, received an ALS Society/CIHR Doctoral Award for his work on a mouse model of the disease.Ìý

Dr. Stefano Stifani’s research focuses on understanding the generation, muscle connectivity, and regeneration of nerve cells that are important for mastication, swallowing, and motor activities. His laboratory has made important strides in elucidating the intricate mechanisms underlying the development of those particular types of neurons. ÌýThe 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 adult nervous system's ability to repair itself in response to trauma or disease.Ìý

Dr. Eric Shoubridge is identifying genes that cause mitochondrial dysfunction.Ìý Mitochondrial dysfunction is thought to contribute to many neurodegenerative diseases, including ALS.ÌýÌý His study of the mechanisms of molecular pathology could enrich our understanding of motor neuron disease.

ALS and Community Support

The Tony Proudfoot Fund was established by the former quarterback of the Montreal Alouettes of the Canadian Football League, the late Tony Proudfoot, after he was diagnosed with ALS in 2007.Ìý The fund raises awareness about this devastating disease.Ìý It also provides important support for ALS research at The Neuro and for patient and family services at the ALS Society of Quebec. ÌýIn 2009, Dr. Miranda Tradewell held the Tony Proudfoot postdoctoral fellowship while working in Dr. Durham's laboratory.

The ALS Society of Quebec was established in 1983 to provide support for people with ALS and their families, to create public awareness, and to raise funds for patient services and research. ÌýThe ALS Society of Quebec works in partnership with other provincial ALS agencies and with the ALS Society of Canada.

About the Montreal Neurological Institute and Hospital

The Montreal Neurological Institute and Hospital is a world-renowned academic medical centre dedicated to neuroscience. ÌýThe Neuro is a research and teaching institute of Â鶹AV University, and forms the basis for the Neuroscience Mission of the Â鶹AV Health Centre. ÌýFounded in 1934 by Dr. Wilder Penfield, the Neuro is known for integrating research, compassionate patient care and advanced medical training. ÌýThe Neuro's 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. ÌýFor more information, please visit .