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A picture of a microscope

Mouse Model Sets Trap for ALS Dementia

Mouse Model Leads Way to Testing for Treatment

ALS overtook our social media streams in the summer of 2014 with the Ice Bucket Challenge, and the attention was more than well needed. The neurological disorder, also called Lou Gehrig’s disease, affects nearly 350,000 people worldwide, with no known cure and a survival rate of only three to five years. But there may be hope at the end of the viral social media campaign: A breakthrough from a group of Northwestern scientists will lead to the rapid testing of drug treatments for the dementia form of the disease.

With unprecedented interest and attention, expanded research capabilities and funding has allowed scientists like those led by Teepu Siddique, MD, the Les Turner ALS Foundation/Herbert C. Wenske Professor of Neurology at Northwestern University Feinberg School of Medicine, and Han-Xiang Deng, MD, PhD, research professor in the Ken and Ruth Davee Department of Neurology, both of Northwestern Medicine, to pursue advances in the field. The Northwestern scientists and authors of a new study in the Proceedings of the National Academy of Sciences successfully reproduced the symptoms of amyotrophic lateral sclerosis (ALS) dementia in mice.

Breaking barriers to drug testing

An animal model of ALS dementia, the first of its kind, will allow the scientists to observe directly how test drugs affect the brains of mice under anesthesia at a microscopic level. Up until this point, difficulty recreating the genetic mutations of ALS and ALS dementia has significantly slowed the process of drug therapy testing for the disease and a successful model represents a significant accomplishment.

ALS leads to muscle weakness, impaired speaking, and difficulty swallowing and breathing, eventually causing paralysis and death. ALS with dementia, representing five percent or more of ALS cases, is considered an even more vicious disease, attacking the brain, causing changes in behavior and language, and affecting judgment, insight and decision-making.

By mimicking the behavioral, neurophysiological and pathological changes of ALS dementia seen in humans, scientists will be able to rapidly test and directly monitor new drug therapies in real time, accelerating the development of treatment.