What is the AGS Scale?
The AGS Scale is a way for doctors to measure how severe the outcomes are for people with Aicardi Goutières Syndrome (AGS). The team at the Children's Hospital of Philadelphia developed a short, simple questionnaire that scores the severity of a person's overall global neurologic dysfunction. The scale is easy to apply and remains relevant to AGS' unique challenges. It was developed to better understand how individuals affected by AGS change over time.
Why is a neurologic severity scale important for AGS?
Doctors use the AGS Scale to track how Aicardi Goutières Syndrome (AGS) is affecting a patient over time. This helps them determine whether the patient's condition is improving or worsening. This helps doctors make better decisions about how to treat the patient and can help families better understand the course of the disease. The scale also helps researchers study the disease and measure the effect of new treatments.
How was the neurologic severity scale developed?
A team of AGS experts from the U.S. and Italy worked together to create the AGS Scale. They reviewed patient medical records and chose features that could help determine the severity of the disease, including motor, cognitive, and communication abilities. To make sure the results were consistent, multiple doctors examined many patient records and applied the AGS Scale. They then compared the scores from the AGS Scale with results from other scales to ensure that the results were meaningful.
How do I apply the scale?
For each item in a list, a patient can receive one point, and the maximum score is 11.
Normocephaly (absence of microcephaly)
Social Smile
Vocalizations (cooing or babbling)
Single, meaningful words
Minimum of three-word phrases
Head Control (>60 seconds)
Pincer grasp or self-feeding
Independent Sitting (>2 minutes)
Rolling or crawling to goal
Ambulation with assistance (devices or two-hand assist)
Independent Ambulation
How do scores compare?
Scores can be compared by AGS type (or gene) and with how doctors considered patients to have "mild", "moderate", or "severe" AGS.
Adang, Laura A et al. “Development of a neurologic severity scale for Aicardi Goutières Syndrome.” Molecular genetics and metabolism vol. 130,2 (2020): 153-160. doi:10.1016/j.ymgme.2020.03.008
How do scores change over time?
For most patients studied, the AGS Scale scores changed the most in the first six months after they were diagnosed with AGS. After that, the scores generally stayed the same. While this stability can be seen as a good thing, it can also mean that patients are less likely to develop new abilities over time. However, in a clinical trial for baricitinib, most children treated with JAK inhibitors saw improving scores on the AGS Scale. This shows that the treatment had a positive effect on their symptoms, and the AGS Scale was important in demonstrating this effect.
AGS Scale & JAK Inhibition
The AGS scale demonstrated that baricitinib (a JAK inhibitor) helped patients with AGS gain new abilities and reach new milestones. For more information, visit agsaa.org/jaki
Vanderver, Adeline et al. “Janus Kinase Inhibition in the Aicardi-Goutières Syndrome.” The New England journal of medicine vol. 383,10 (2020): 986-989. doi:10.1056/NEJMc2001362
What are the AGS Scale's limitations?
While the AGS Scale is helpful in measuring how a person's abilities change over time, it only looks at their abilities in broad terms. People with AGS have a wide range of abilities and intelligence, and this scale cannot fully capture all of their potential. We should not let the AGS Scale limit the opportunities available to people with AGS. Instead, we should focus on helping them to develop their strengths and abilities to the fullest extent possible.