July 12, 2007 

Irish Researcher Funded to Follow Link to Cognitive Change in ALS

[Quick Summary: Irish ALS experts are funded to take advantage of a unique registry of patients to find and follow links with cognitive changes in the disease that could point the way to effective therapy.]

The ALS Association is funding an effort to understand in detail the evolution of cognitive changes that can accompany amyotrophic lateral sclerosis (ALS, also known as Lou Gehrig's disease), using a unique registry of Irish patients and following patients in person during their disease course to find out possible genetic factors that could give insight into the biology of the disease.

A better understanding of how the disease involves changes in brain and behavior could lead to therapeutic progress.

Orla Hardiman, M.D., of Beaumont Hospital in Dublin, will lead the investigation. A registry of Irish ALS patients has been maintained for a decade in that country and highlights why the U.S. needs a similar resource. Efforts to establish a U.S. ALS registry are underway.

A recent conference in London, Ontario, Canada, focused on the type of cognitive and behavioral change that can occur in some ALS patients. Researchers need to learn more about who will develop these changes and how to best accommodate them during the disease. A common process may underlie ALS and the disorder called frontotemporal dementia.

The project now funded and underway will follow all Irish ALS patients over time so that the investigators can determine how often cognitive changes, especially the early changes in behavior, occur within the population. This study of an entire population of people with ALS seeks to identify and characterize early signs of frontotemporal dementia and to monitor progression over time.  Collection of DNA from affected patients and their families will then permit detailed genetic studies that will help to identify possible causes of ALS and FTD.

“To date, there have been no population-based clinical studies of cognitive decline in ALS, and it is likely that the current estimates suggesting that more than half of ALS patients have cognitive impairment reflect selection bias,” Hardiman noted in her proposal, as the existing reports include patients that choose to come to tertiary medical centers with special ALS clinics.

The true incidence of cognitive impairment among the ALS population may be very different from existing reported incidence, she noted.  Furthermore, there have been few longitudinal studies aimed at characterizing the evolution of cognitive decline in ALS patients. Hardiman added, “This project will allow the investigators to define distinct subgroups of ALS patients with different profiles of cognitive impairment and to later correlate these subgroups with a corresponding genetic risk.”

A feature of this project is that a research Fellow and assistant (where appropriate) will travel to the homes of all living Irish ALS patients around the country to conduct a standardized evaluation including neuropsychological examination. This effort should provide a more reliable picture of the cognitive aspects of the disease compared to existing reports that have depended on patients maintaining visits to clinics.

This study will dovetail with ongoing investigations also funded by The Association that are seeking information about coincidence of ALS and other neurodegenerative diseases in families.

Please see The ALS Association’s web site under the research tab for further information about cognitive changes in ALS.

Also see the following relevant link for U.S. Registry efforts.