The ALS Association

Ice Bucket Challenge Progress
Carrie Munk
The ALS Association
(571) 319-3047



Dr. Ammar Al-Chalabi Delivers the Sheila Essey Award Lecture

Washington, D.C. (May 3, 2016)— Ammar Al-Chalabi, Ph.D., FRCP, DipStat, Professor of Neurology and Complex Disease Genetics at King’s College London, received the Sheila Essey Award in a special session devoted to ALS research at the annual meeting of the American Academy of Neurology in Vancouver, B.C., in April. The $50,000 award, which honors the memory of Sheila Essey and was presented by her husband Dick. The award recognizes significant research contributions in the search for the cause, prevention of and cure for amyotrophic lateral sclerosis (ALS). Since 1996, The ALS Association and the American Academy of Neurology choose recipients jointly.

ALS is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Eventually, people with ALS lose the ability to initiate and control muscle movement, which often leads to total paralysis and death within two to five years of diagnosis. For unknown reasons, veterans are twice as likely to develop ALS as the general population. There is no cure, and only one drug.

“Dr. Al-Chalabi is a brilliant clinical scientist and a world leader in the complex genetic of ALS,” Essey said. He was co-discoverer of the genetic linkage between ALS and frontotemporal dementia that eventually led to the discovery of the C9orf72 gene, now the most common genetic cause of both diseases.

In beginning his acceptance lecture, Dr. Al-Chalabi thanked the Essey family, as well as his mentors in ALS research, Nigel Leigh, Robert Brown and Christopher Shaw.

“Much of what we thought we knew about ALS in 1994, when I began my career in ALS, we now know is wrong,” he said. In particular, ALS was then thought to be one disease, but it is now clear that it is actually multiple diseases with similar clinical features ultimately initiated by different causes.

The number of known ALS genes is doubling every four years, he pointed out, with seven to eight new genes found every year, as a result of better technology for gene discovery, as well as much more extensive collaborations among genetic researchers. That collaboration increases the sample size for study and allows discovery of less common genes.

With large-scale genomic sequencing projects underway, “the real problem,” Dr. Al-Chalabi said, “will be the analysis.” The volume of data “makes even the best information technology systems creak under the strain.”

Future gene discoveries in ALS are likely to be genes that individually have modest effects, but which combine with other genes or with environmental factors to cause disease. “Sporadic ALS may be caused by many different, rare gene variants,” he said. He also noted that “sporadic” can no longer be thought of as equivalent to “non-genetic,” as more and more genes are discovered in those without a decipherable family history of the disease.

Several difficult-to-explain features of ALS become more explainable if the disease is understood to result from the sequential occurrence of multiple “hits” or contributing events. These can include inheritance of a gene, exposure to an environmental toxin, increasing age and presence of one or more accessory genes that increase risk. Working with colleagues, Dr. Al-Chalabi has developed a mathematical model, based on population-wide incidence levels of ALS across Europe, suggesting that six hits are needed to cause ALS.

This model helps explain “incomplete penetrance,” in which not every person who inherits a gene develops the disease, as well as the difficulty of identifying clear environmental causes, and the variation in disease features between people with same gene mutation.

“ALS genetics is advancing rapidly,” he concluded, “and understanding this will allow us to develop new therapies tailored to the individual genes.”

The Sheila Essey Award is made possible through the generosity of the Essey Family Fund through The ALS Association Golden West Chapter. Initiated in 1996, the award was created in memory of Sheila Essey, who battled ALS for 10 years and died in 2004. Richard Essey, Sheila's husband, served as a National Trustee of The ALS Association and is one of the founders of the Greater Bay Area Chapter, now the Golden West Chapter. Past recipients have used funds to continue their ALS research or to support promising young scientists on their research teams.

About The ALS Association
The ALS Association is the only national non-profit organization fighting Lou Gehrig’s Disease on every front.  By leading the way in global research, providing assistance for people with ALS through a nationwide network of chapters, coordinating multidisciplinary care through certified clinical care centers, and fostering government partnerships, The Association builds hope and enhances quality of life while aggressively searching for new treatments and a cure.  For more information about The ALS Association, visit our website at

Powered by Blackbaud
nonprofit software