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ALS and The Montreal Neurological Institute and Hospital

Published: 21 June 2010

What is ALS?

Amyotrophic Lateral Sclerosis (ALS), commonly known as Lou Gehrig’s disease, is a deadly neurodegenerative disease.  Approximately 3,000 Canadian adults have ALS.  A large majority of them will die within two to five years of their diagnosis.  The main symptom is muscle weakness that progressively reaches the point of paralysis and prevents a person from breathing.  ALS is a result of the death of motor neurons---nerve cells in the brain and spinal cord that control voluntary muscle movement. (Source:  www.als.ca)

ALS research and treatment initiatives at The Neuro

The Montreal Neurological Institute and Hospital is committed to the highest level of care for ALS patients and their families.  The Neuro's dynamic program provides both exceptional clinical care and state-of-the-art facilities for basic and clinical research. 

A multidisciplinary ALS clinic was established at The Neuro in 1998.   Today the clinic is recognized as a leader in caring for this special patient population, and its program is considered a model for multidisciplinary clinics.   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 and therapy combinations in collaboration with other researchers in Quebec and abroad.

The Unit's clinical research includes a multicentre clinical trial in ceftriaxone.   Two other clinical trials are being developed in collaboration with the Canadian ALS Clinical Trial Consortium (CALS).  In addition, a symptom management trial is being considered for control of pain and spasticity.   The Neuro is recruiting new faculty to conduct research in motor neuron diseases, and to provide neuropalliative care.

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.

Dr. Heather Durham, for example, specializes in developing tissue culture models of ALS.  These models help to understand what makes nerve cells susceptible to damage in ALS and in other motor neuron diseases.  Models also allow researchers to test experimental therapies.  Dr. Durham is particularly interested in developing treatments that activate cellular stress responses that fight the disease.  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.  In conjunction with pharmaceutical and biotech companies, she tests putative therapies for motor neuron diseases in culture and animal models; and works with Dr. David Burns in McGill University's Department of Chemistry to identify biomarkers of ALS in blood samples.  Dr. Durham is working at the national level to promote ALS research initiatives.  She serves as a member of the Board of the ALS Society of Canada and as Chair of the Research Policy Committee.  On 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. 

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.  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 adult nervous system's ability to repair itself 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 laboratory has identified a number of proteins that could play a fundamental role in neurodegenerative and motor neuron diseases.  These proteins could further 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. McPherson’s student, PhD candidate Jason Hamlin was the 2009 recipient of the ALS Society/CIHR Doctoral Award. His work is profiled in the recent issue of the Northern Neuron, the newsletter of the ALS Society.

Dr. Eric Shoubridge’s laboratory investigates the molecular genetics of disease.  His 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 that have improved diagnoses and treatment.

ALS and Community Support

The Tony Proudfoot Fund was established by the former quarterback of the Montreal Alouettes of the Canadian Football League, Tony Proudfoot, after he was diagnosed with ALS in 2007.  The fund raises awareness about this devastating disease.  It also provides important support for ALS research at The Neuro and for patient and family services at the ALS Society of Quebec.  

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.  The 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, The Neuro, is a world-class Canadian academic medical centre, that combines an internationally leading research centre at McGill University, dedicated to unlocking the secrets of the brain and neurological diseases, with specialized hospital services as part of the McGill University Health Centre (MUHC), that ensure the highest quality of advanced and compassionate care for patients with neurological diseases. This unique structure has became a model for institutions around the world, giving unprecedented hope for some of our most debilitating conditions, from epilepsy to stroke to Parkinson’s disease. The Neuro has become one of the largest neuroscience institutes in the world, attracting some of the best minds from around the globe.

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