In a study using a non-toxic substance on mice, Northwestern University researchers have identified the first compound that eliminates the ongoing degeneration of brain neurons in the paralyzing disease known as ALS. Amyotrophic lateral sclerosis is a neurodegenerative disease in which upper motor neurons degrade in victims, producing a swift and fatal demise. In addition to ALS, other motor neuron diseases, such as hereditary spastic paraplegia (HSP) and primary lateral sclerosis (PLS) progress in a similar fashion. In ALS, movement-initiating nerve cells in the brain (upper motor neurons) and muscle-controlling nerve cells in the spinal cord (lower motor neurons) die—and, so far, there has been no drug or treatment for the brain component of ALS, and no drug for HSP and PLS patients. “Even though the upper motor neurons are responsible for the initiation and modulation of movement, and their degeneration is an early event in ALS, so far there has been no treatment option to improve their health,” said senior author Hande Ozdinler, associate professor of neurology at Northwestern University Feinberg School of Medicine.