Northwestern University and Evanston's Only Daily News Source Since 1881

The Daily Northwestern

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Northwestern University and Evanston's Only Daily News Source Since 1881

The Daily Northwestern

Northwestern University and Evanston's Only Daily News Source Since 1881

The Daily Northwestern

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NU researchers discover common cause of all forms of ALS

On the night 62-year-old attorney Daniel Weiler was diagnosed amyotrophic lateral sclerosis in April 2009, commonly known as Lou Gehrig’s disease, he and his wife went out for dinner at a restaurant. During dinner, they cried. He later asked her to tell their children about his disease.

“We were pretty devastated,” said Weiler, an Elgin resident. “We had our moments and our sorrow.”

But Weiler and the roughly 35,000 Americans diagnosed with Lou Gehrig’s may find solace in the latest ALS research.

This summer, Northwestern University Feinberg School of Medicine researchers discovered a common cause of all forms of ALS.

In their research, scientists found that the root of the disorder lies in the malfunction of a protein recycling system in the neurons of the spinal cord and the brain. Once this system malfunctions, cells can no longer repair themselves and suffer considerable damage. This breakdown occurs in all three types of ALS and may play a role in other neurodegenerative diseases such as Alzheimer’s disease, dementia and Parkinson’s disease.

Dr. Teepu Siddique, the senior author of the study and a neurologist at Northwestern Memorial Hospital, said this is the first time the cause of ALS has been found to be linked to a specific biological mechanism.

“Once you have a mechanism, then you have a target with the disease,” said Siddique, a Les Turner ALS Foundation/Herbert C. Wenske professor at Feinberg.

ALS is the slow degeneration of motor nerves, according to Wendy Abrams, the executive director of the Les Turner ALS Foundation. Among those diagnosed with the disease, 10 percent of cases are familial or inherited and 90 percent are sporadic or random. Most people afflicted are typically in their 50s, but Abrams has seen patients even in their 20s and 30s with young families.

“It’s a neurological degeneration of motor nerves so most people experience a wasting away of the muscle,” she said. “So little by little, they’re paralyzed.”

Weiler, a patient of Siddique, can attest to this slow degeneration. After his diagnosis, it became gradually more difficult to walk. He soon progressed from cane to walker to power wheelchair. Though his family modified their car to allow him to transfer between driver’s seat and wheelchair, he decided to stop driving because of the lack of muscle control.

“The driving experience was too dangerous and anxiety-producing,” Weiler said. “I decided it would be much safer not to do it.”

Weiler’s disease also prevents him from participating in other activities, such as the golf outings and candy fundraisers sponsored by the Elgin Lions Club and social events hosted by the bar association. He said tasks like eating, bathing and dressing take more time than before.

“It takes away all of a person’s independence,” Abrams said. “You become completely dependent on other people for your well-being.”

Although Weiler has become dependent on his family and his disease saddens them, he said their support is helpful. He said he feels he is luckier than younger ALS patients.

“In some ways, it’s fortunate I have this at this age,” he said. “I have at least seen my grandchildren, both children married. I kind of know that their life is gonna be good. It’s not as cruel as somebody in their 40s who still has a family to raise and has to worry about that. I think I’m blessed to have this happen in a time in my life when I don’t feel unfulfilled.”

But there is progress being made ­- Weiler, Abrams and Siddique said the NU discovery provides hope for the future.

“I think it really offers some possibility that they’ll be able to find some type of medication that can stop this process,” Weiler said.

The next steps for Siddique, who has been studying ALS since 1985, is validating the current testing model of the disease and finding the vulnerable points of the discovered pathway so as to be able to test drugs.

Even though Weiler knows what may lie ahead, he still tries to stay positive. Ongoing ALS research will continue to benefit others, he said.

“Your life has not ended just because you got that diagnosis,” he said. “You just have to keep yourself going as much as you can.”

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Northwestern University and Evanston's Only Daily News Source Since 1881
NU researchers discover common cause of all forms of ALS