Risk factors that may contribute to the onset of Amyotrophic Lateral Sclerosis

Researchers in Rensselaer Polytechnic Institute's Social and Behavioral Research Lab (SBRL) are leading a study to investigate potential environmental, lifestyle, and medical variables that may contribute to the onset of Amyotrophic Lateral Sclerosis (ALS).

Through a national survey constructed with the help of an advisory panel consisting of leading public health officials, biologists, and neuroscientists, among others, the SBRL hopes to contribute to an emerging body of research into potential causes of ALS.

Called the Patricia Wieler Memorial ALS Project, the study is being primarily funded by Eric “Rip” Wieler '54 in memory of his late wife who suffered from ALS, and for whom the research project is named.

Commonly referred to as Lou Gehrig's Disease, ALS is a progressive neurodegenerative disease that attacks nerve cells in the brain and spinal cord. Motor neurons in the human brain send signals through the spinal cord to our muscles in order to initiate or control our voluntary movements such as kicking a ball, rubbing an eye, or picking up a telephone. Individuals who suffer from ALS experience motor neuron degeneration, and as the neurons die, signals are no longer sent to the body's muscle fibers. Lack of stimulation causes muscle weakening and shrinking – called atrophy – and eventual paralysis.

The life expectancy of an ALS patient averages about two to five years from the time of diagnosis, according to the ALS Association, and it is estimated that as many as 30,000 Americans may have the disease at any given time. Several studies have investigated possible environmental causes of ALS, but none have conclusively identified links between external factors and disease onset.

SBRL researchers are currently working with advisory board members to devise the most effective and methodologically rigorous questions possible for a survey that will be distributed nationally. They expect to receive half of their responses from ALS patients or a designated family member, friend, or caretaker who can respond on the patient's behalf. The second half of the responses will come from individuals who have a similar background to the patients, but who do not have ALS. They will serve as a statistical comparison group, according to James Watt, director of the SBRL and project administrator.

“The existing body of ALS research has revealed possible connections between the disease's onset and a single or small set of variables such as military service; exposure to lead, pesticides, and herbicides; and behavioral variables such as diet and smoking,” said Watt. “Most of these studies have focused on a single factor. Our project will involve asking questions about a wider range of such variables, and investigating complex interactions between environmental factors and possible genetic factors. We hope to provide insight into potential causal mechanisms that might warrant further experimental or epidemiological research.”

Wieler serves as a member of the project's advisory board along with Wilfredo Colon, associate professor of chemistry and a nationally known researcher in molecular processes such as protein “misfolding” and degradation, which may be involved with the onset of ALS; Russ Ferland, assistant professor of biology, whose research in the field of neuroscience has revealed important insights into the genetic determinants of various neurological conditions; William “Al” Wallace, professor of decision sciences and engineering systems, who is an expert in computationally intensive data mining techniques; Paula Loring Simon, Rensselaer trustee; and Dhelia “Dee” Williamson, an epidemiologist at the Centers for Disease Control and Prevention in Atlanta, who has extensive experience researching various motor neuron diseases including ALS.

“This project is the culmination of a dream I have had since my wife died 19 years ago,” said Wieler. “Until now, many physicians have searched for possible cures for ALS without even knowing what causes the disease. I have long sought the proper environment to sponsor a project that uses advanced analysis techniques to properly search for the most likely causes of ALS, and I am grateful to all those at RPI who are making this possible. I am very optimistic about our potential for success.”

The current research is planned for one year. If the initial survey successfully produces environmental clues related to the disease's onset, the researchers will continue the research for a second year and undertake a much more extensive survey, according to Watt.

“By combining the survey research expertise of the SBRL with the biological science, computational, and data mining skills of Rensselaer researchers, we hope to bring a new approach that will uncover paths to more effective treatments for this disease,” said Watt.

Wieler's support of this study supports the $1.4 billion Renaissance at Rensselaer capital campaign.