Winner of Essey Award Reviews Findings at
April 12, 2005
Contrary to prior concepts, amyotrophic lateral sclerosis may not just kill the motor neurons that contract muscles, according to recent findings by Canadian researcher Michael J. Strong, M.D., FRCPC, winner of this year’s The Sheila Essey Award for ALS Research presented by The ALS Association (ALSA) and the American Academy of Neurology (AAN).
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| Dr. Michael Strong, chief of neurology at London Health Sciences Centre , and investigator at the John P. Robarts Research Institute in London, Ontario, Canada, winner of ALSA's 2005 Essey Award. |
These new findings are beginning to influence how clinicians view the nerve wasting disease, also known as ALS or Lou Gehrig’s disease. Many ALS patients also show subtle changes in cognition. Those changes can be associated with microscopic damage to the responsible lobes of the brain.
Strong will review his findings as he accepts the award during the AAN’s annual meeting in Miami, Florida on April 12.
The Sheila Essey Award for ALS Research was inaugurated in 1996 by ALSA in partnership with AAN to acknowledge and honor an individual actively engaged in ALS scientific research who has made significant contribution to the search for the cause, prevention or cure for the disease. Named for Sheila Essey who succumbed to ALS in 2004, this important award includes a $25,000 prize to be used toward continuing ALS research. Three of the past winners have established Sheila Essey fellowships at their institutions to help fund junior researchers to induce them to work in the field of ALS. Today the Sheila Essey Award has been characterized as the most prestigious award in the world in the field of ALS research.
“The work of Dr. Strong and other researchers are bringing us closer to understanding Lou Gehrig’s disease,” said Richard P. Essey, ALSA national trustee. “On behalf of The Essey Family Fund, I am proud to add Dr. Strong’s name to a list of pioneer researchers who are bringing us closer to finding a solution to this devastating disease.”
“It is wonderful to acknowledge the important contributions that Michael Strong has made to both clinical and basic research into ALS,” said ALSA Science Director Lucie Bruijn, Ph.D. Strong has served on ALSA’s scientific review committees.
The award goes to Strong for his work to advance the understanding of this devastating disease that produces progressive weakness ending in paralysis and respiratory failure. Strong is director of the multidisciplinary ALS clinic at the University of Western Ontario, chief of neurology at London Health Sciences Centre, and an investigator at the John P. Robarts Research Institute in London, Ontario, Canada.
Strong is recognized for his translational approach to the puzzle of ALS. Not only is Strong respected for expertise in the basic biology of the motor nerve cells affected by ALS, but also for his clinical investigations into the possible link of this motor neuron disease to frontotemporal dementia (FTD). This link may provide new knowledge of the disease process that could lead to innovative treatments.
"This award is a tremendous recognition of our work, and that of my colleagues in trying to understand the nature of this devastating illness,” Strong said. “It will allow us to move forward in determining whether our current findings are unique and potentially diagnostic of FTD in ALS."
FTD is a shift in mental processes that appears to accompany ALS in a number of patients, and may even precede motor symptoms in some cases. Frontotemporal refers to the forward part of the brain that sits above the eyes and behind the temples. Lowering of the function of this region can lead to impulsive, compulsive, and emotional behavior. These changes can hamper a person’s ability to cope with the demanding nature of an illness such as ALS.
Strong and his colleagues have pointed to changes in brain tissue from ALS patients that may correspond to the clinical signs of FTD. His basic studies on the inner dynamics of motor neurons meanwhile show that ALS may affect the structural proteins that allow neurons to maintain their lengthy connections to muscle.
Because there is still so much to learn about the association between frontotemporal dementia and ALS, ALSA is co-sponsoring a workshop on this topic in London, Ontario, Canada this May. Strong will chair the meeting.
His current project, "A Molecular And Neuropathological Characterization Of The Cognitive Impairment Of Sporadic Amyotrophic Lateral Sclerosis," funded by ALSA, focuses on abnormal deposits within cells of a protein called tau. The aim is now to define the extent of abnormal cellular handling of tau in ALS and determine if there is a unique molecular signature of cognitive impairment in ALS.
Prior winners of the Essey prize, including Nigel Leigh, Stanley Appel, and Don Cleveland, are making a significant impact on ALS research, opening doors to other research paths in understanding and solving the ALS puzzle.
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Note: The nomination process and selection of the award winner is under the direction of the American Academy of Neurology as part of its annual Scientific Awards Program. The American Academy of Neurology, an association of more than 18,000 neurologists and neuroscience professionals, is dedicated to improving patient care through education and research. A neurologist is a doctor with specialized training in diagnosing, treating and managing disorders of the brain and nervous system such as stroke, Alzheimer’s disease, epilepsy, Parkinson’s disease, autism and multiple sclerosis. For more information about the American Academy of Neurology, visit its website at www.aan.com.
