Amyotrophic lateral sclerosis (ALS), or Lou Gehrig’s disease, is a rapidly progressive, ultimately fatal neurological disease that attacks the nerve cells responsible for controlling voluntary muscles. Individuals with ALS lose their strength, their ability to move their arms, legs, and body, and, ultimately, their ability to breathe without ventilator support. Although not associated with impaired thinking or other cognitive abilities, recent studies suggest some ALS patients may develop a form of dementia, including trouble with memory and decision-making. Some treatments appear to slow ALS or relieve symptoms, and new treatment trials are ongoing. There are currently no cures. Other disorders involve the peripheral nerves, neuromuscular junction, and muscle include: peripheral neuropathy (diabetic but also chemotherapy-induced and hereditary), Guillain-Barre & CIDP, myasthenia gravis, muscular dystrophy (Duchenne & Becker’s) and myopathies (hereditary/congenital or acquired).
What We Know:
It is known from pre-clinical studies that inflammatory cells called “glia” are activated in brains and spinalcords of people with Amyotrophic Lateral Sclerosis (ALS) and may play a role in disease progression.
Our Plan to Help:
Dr. Babu’s goal is to understand the inflammatory and neurodegenerative changes in the spinal cords of people living with ALS. Dr. Babu will enroll ALS participants who have weakness and atrophy of their arm muscles and healthy controls. Eligible participants will undergo a single unique scan of their cervical spine that acquires both magnetic resonance imaging (MRI) and Positron Emission Tomography (PET) images in one scan session. She will quantify (a) inflammation using a PET dye called “PBR28” that binds to activated glia in the spinal cord and (b) spinal cord atrophy at each vertebral level of the cervical spine. These imaging findings will be correlated with corresponding measures of strength and function of arm and respiratory muscles receiving their nerve supply from motor nerve cells residing at these spinal cord levels.
Dr. Babu and her team’s study addresses important, under-studied, disease mechanism-related research questions with potential implications for improving clinical trial designs in ALS.
How You Can Help:
Donate to the American Brain Foundation to support Dr. Babu’s research that will help inform the design of future longitudinal studies of the spinal cord and subsequently clinical drug trials targeting neuroinflammation in ALS patients with weakness and atrophy of limb muscles.
Dr. Babu is the recipient of the 2019 Richard Olney Clinician Scientist Development Award in ALS funded by the American Brain Foundation and The ALS Association in collaboration with the American Academy of Neurology.